Commentary

Total knee arthroplasty (TKA) is a good surgical option to relieve pain and improve function in patients with osteoarthritis. The goal of surgery is to achieve a well-aligned prosthesis with well-balanced ligaments in order to minimize wear and improve implant survival. Overall, 82% to 89% of patients are satisfied with their outcomes after TKA, with good 10- to 15-year implant survivorship; however, there is still a subset of patients that are unsatisfied. In many cases, patient dissatisfaction is attributed to improper component alignment.^1-3 Over the past decade, computer navigation and robotics have been introduced to control surgical variables so as to gain greater consistency in implant placement and postoperative component alignment.

Computer-assisted navigation tools were introduced not only to improve implant alignment but, more importantly, to optimize clinical outcomes. Most studies have demonstrated that the use of navigation is associated with fewer radiographic outliers after TKA.⁴ Various studies have compared radiographic results of navigated TKA with results of TKA using standard instrumentation.^4-7 While long-term studies are necessary, short-term follow-up has shown that computer-assisted TKA can improve alignment, especially in patients with severe deformity.^8-10 Currently, there is no definitive consensus that computer-assisted TKA leads to significantly better component alignment or postoperative outcomes due to the fact that many studies are limited by study design or small cohorts. However, the currently published articles support better component alignment and clinical outcomes with computer-assisted TKA. While some argue that the use of computer-assisted surgery is dependent on the user’s experience, computer-assisted surgery can assist less-experienced surgeons to reliably achieve good midterm outcomes with a low complication rate.^8,11 Various studies have looked at computer-assisted TKA at midterm follow-up, with no significant differences in clinical outcome between navigated and traditional techniques. However, long-term studies showing the benefits of computer navigation are beginning to emerge. For example, de Steiger and colleagues¹² recently found that computer-assisted TKA reduced the overall revision rate for loosening after TKA in patients less than 65 years of age.

While surgical navigation helps improve implant planning, robotic tools have emerged as a tool to help refine surgical execution. Coupled with surgical navigation tools, robotic control of surgical gestures may further enhance precision in implant placement and/or enable novel implant design features. At present, robotic techniques are increasingly used in unicompartmental knee arthroplasty (UKA) and TKA.¹³ Studies have demonstrated that the robotic tool is 3 times more accurate with 3 times less variability than conventional techniques in UKA.¹⁴ The utility of robotic tools for TKA remains unclear. Robotic-driven automatic cutting guides have been shown to reduce time and improve accuracy compared with navigation guides in femoral TKA cutting procedures in a cadaveric model.¹⁵ However, robotic-enabled TKA procedures are poorly described at present, and the clinical implications of their proposed improved precision remain unclear.

Computer navigation and robotic tools in TKA hold the promise of enhanced control of surgical variables that influence clinical outcome. The variables that may be impacted by these advanced tools include implant positioning, lower limb alignment, soft-tissue balance, and, potentially, implant design and fixation. At present, these tools have primarily been shown to improve lower limb alignment in TKA. The clinical impact of the enhanced control of this single surgical variable (lower limb alignment) has been muted in short-term and midterm studies. Future studies should be directed at understanding which surgical variable, or combination of variables, it is most essential to precisely control so as to positively impact clinical outcomes. ◾

Total knee arthroplasty (TKA) is a good surgical option to relieve pain and improve function in patients with osteoarthritis. The goal of surgery is to achieve a well-aligned prosthesis with well-balanced ligaments in order to minimize wear and improve implant survival. Overall, 82% to 89% of patients are satisfied with their outcomes after TKA, with good 10- to 15-year implant survivorship; however, there is still a subset of patients that are unsatisfied. In many cases, patient dissatisfaction is attributed to improper component alignment.^1-3 Over the past decade, computer navigation and robotics have been introduced to control surgical variables so as to gain greater consistency in implant placement and postoperative component alignment.

Computer-assisted navigation tools were introduced not only to improve implant alignment but, more importantly, to optimize clinical outcomes. Most studies have demonstrated that the use of navigation is associated with fewer radiographic outliers after TKA.⁴ Various studies have compared radiographic results of navigated TKA with results of TKA using standard instrumentation.^4-7 While long-term studies are necessary, short-term follow-up has shown that computer-assisted TKA can improve alignment, especially in patients with severe deformity.^8-10 Currently, there is no definitive consensus that computer-assisted TKA leads to significantly better component alignment or postoperative outcomes due to the fact that many studies are limited by study design or small cohorts. However, the currently published articles support better component alignment and clinical outcomes with computer-assisted TKA. While some argue that the use of computer-assisted surgery is dependent on the user’s experience, computer-assisted surgery can assist less-experienced surgeons to reliably achieve good midterm outcomes with a low complication rate.^8,11 Various studies have looked at computer-assisted TKA at midterm follow-up, with no significant differences in clinical outcome between navigated and traditional techniques. However, long-term studies showing the benefits of computer navigation are beginning to emerge. For example, de Steiger and colleagues¹² recently found that computer-assisted TKA reduced the overall revision rate for loosening after TKA in patients less than 65 years of age.

While surgical navigation helps improve implant planning, robotic tools have emerged as a tool to help refine surgical execution. Coupled with surgical navigation tools, robotic control of surgical gestures may further enhance precision in implant placement and/or enable novel implant design features. At present, robotic techniques are increasingly used in unicompartmental knee arthroplasty (UKA) and TKA.¹³ Studies have demonstrated that the robotic tool is 3 times more accurate with 3 times less variability than conventional techniques in UKA.¹⁴ The utility of robotic tools for TKA remains unclear. Robotic-driven automatic cutting guides have been shown to reduce time and improve accuracy compared with navigation guides in femoral TKA cutting procedures in a cadaveric model.¹⁵ However, robotic-enabled TKA procedures are poorly described at present, and the clinical implications of their proposed improved precision remain unclear.

Computer navigation and robotic tools in TKA hold the promise of enhanced control of surgical variables that influence clinical outcome. The variables that may be impacted by these advanced tools include implant positioning, lower limb alignment, soft-tissue balance, and, potentially, implant design and fixation. At present, these tools have primarily been shown to improve lower limb alignment in TKA. The clinical impact of the enhanced control of this single surgical variable (lower limb alignment) has been muted in short-term and midterm studies. Future studies should be directed at understanding which surgical variable, or combination of variables, it is most essential to precisely control so as to positively impact clinical outcomes. ◾

Total knee arthroplasty (TKA) is a good surgical option to relieve pain and improve function in patients with osteoarthritis. The goal of surgery is to achieve a well-aligned prosthesis with well-balanced ligaments in order to minimize wear and improve implant survival. Overall, 82% to 89% of patients are satisfied with their outcomes after TKA, with good 10- to 15-year implant survivorship; however, there is still a subset of patients that are unsatisfied. In many cases, patient dissatisfaction is attributed to improper component alignment.^1-3 Over the past decade, computer navigation and robotics have been introduced to control surgical variables so as to gain greater consistency in implant placement and postoperative component alignment.

Computer-assisted navigation tools were introduced not only to improve implant alignment but, more importantly, to optimize clinical outcomes. Most studies have demonstrated that the use of navigation is associated with fewer radiographic outliers after TKA.⁴ Various studies have compared radiographic results of navigated TKA with results of TKA using standard instrumentation.^4-7 While long-term studies are necessary, short-term follow-up has shown that computer-assisted TKA can improve alignment, especially in patients with severe deformity.^8-10 Currently, there is no definitive consensus that computer-assisted TKA leads to significantly better component alignment or postoperative outcomes due to the fact that many studies are limited by study design or small cohorts. However, the currently published articles support better component alignment and clinical outcomes with computer-assisted TKA. While some argue that the use of computer-assisted surgery is dependent on the user’s experience, computer-assisted surgery can assist less-experienced surgeons to reliably achieve good midterm outcomes with a low complication rate.^8,11 Various studies have looked at computer-assisted TKA at midterm follow-up, with no significant differences in clinical outcome between navigated and traditional techniques. However, long-term studies showing the benefits of computer navigation are beginning to emerge. For example, de Steiger and colleagues¹² recently found that computer-assisted TKA reduced the overall revision rate for loosening after TKA in patients less than 65 years of age.

While surgical navigation helps improve implant planning, robotic tools have emerged as a tool to help refine surgical execution. Coupled with surgical navigation tools, robotic control of surgical gestures may further enhance precision in implant placement and/or enable novel implant design features. At present, robotic techniques are increasingly used in unicompartmental knee arthroplasty (UKA) and TKA.¹³ Studies have demonstrated that the robotic tool is 3 times more accurate with 3 times less variability than conventional techniques in UKA.¹⁴ The utility of robotic tools for TKA remains unclear. Robotic-driven automatic cutting guides have been shown to reduce time and improve accuracy compared with navigation guides in femoral TKA cutting procedures in a cadaveric model.¹⁵ However, robotic-enabled TKA procedures are poorly described at present, and the clinical implications of their proposed improved precision remain unclear.

Computer navigation and robotic tools in TKA hold the promise of enhanced control of surgical variables that influence clinical outcome. The variables that may be impacted by these advanced tools include implant positioning, lower limb alignment, soft-tissue balance, and, potentially, implant design and fixation. At present, these tools have primarily been shown to improve lower limb alignment in TKA. The clinical impact of the enhanced control of this single surgical variable (lower limb alignment) has been muted in short-term and midterm studies. Future studies should be directed at understanding which surgical variable, or combination of variables, it is most essential to precisely control so as to positively impact clinical outcomes. ◾

Anaphylaxis is a relatively common occurrence for many adolescents. As primary care doctors, we normally see the patient after the acute phase, and then are required to do the detective work to figure out the causes of the episode. The cause may be obvious, but many times we have to hope for another occurrence with similar circumstances to identify it. Surprisingly, the cause may not be what you think. Factors that contribute to an anaphylaxis response may be related to activity, timing of food ingestion, an environmental factor, or medication.

Let’s look at just one type, exercise-induced anaphylaxis. It’s divided into two categories: food dependent and nonfood dependent. Both are described as an induction of itching, urticaria, and fatigue, with progression to angioedema and hypotension, associated with exercise (J. Allergy Clin. Immunol. 1980;66:106-11).

Food-dependent exercise-induced anaphylaxis occurs when exercise is started 30 minutes after ingesting food. This may be difficult to identify because patients react to the food only if they exercise, so food is usually eliminated as a cause. Wheat and wheat flour are common culprits for this type of reaction because of the omega-5 gliadin, which is the protein in gluten (J. Allergy Clin. Immunol. 1991;87:34-40). In one study, larger amounts of the suspected agent were given; hives and angioedema did start to occur in 20% of patients challenged, which suggested that there was likely a baseline allergy to the food, and exercise itself might be a cofactor in augmentation of the allergic reaction.

In nonfood-dependent exercise-induced anaphylaxis, symptoms of itching, urticaria, and fatigue can occur 5-30 minutes after the start of exercise. Although bronchospasm is rare, it can occur along with angioedema, nausea, vomiting, and hypotension, and can even be fatal if exercise continues. If exercise is stopped, it usually resolves. However, many people try to push through it, which only worsens the symptoms.

Cofactors associated with nonfood-dependent exercise-induced anaphylaxis are ingestion of alcohol and an NSAID several hours beforehand. These agents also might be overlooked if well tolerated independently (Br. J. Dermatol. 2001;145:336-9).

Timing of the episode also plays a role. Premenstrual syndrome can be a factor in augmentation of anaphylaxis, so it also should be considered. Knowing the date of the last menstrual cycle and identifying if the anaphylaxis is episodic will identify premenstrual syndrome as a cause.

The work-up should include standard allergy testing and determination of tryptase levels. Skin testing is essential to identify offending agents, and is rarely negative. If a food is suspected and skin testing is negative, repeat the skin testing in 6 months. In one study, wheat extract was found to be positive in only 29% of persons suspected of having a wheat allergy, but when the paste of wheat flour was tested, 80% were identified. The ImmunoCAP Test also was found to have a sensitivity of 80%, so it is a valuable test to try along with the skin prick.

Tryptase levels should be evaluated because in nonfood-dependent exercise-induced anaphylaxis, these levels are slightly elevated at the time of the anaphylaxis, but return to normal. A patient with mastocytosis, a group of disorders characterized by pathologic mast cells infiltrating the skin, will consistently have elevated tryptase levels. Seasonal allergies associated with pollen, and asthma bronchospasm also should be considered as causes.

Although these exercise-induced anaphylaxis episodes can occur at any age, they are most frequent in the adolescent age group, probably because that’s the time most of this population are involved in organized sports. Upon presentation, a careful detailed history will help to identify the cause of anaphylaxis and result in quicker resolution.

Treatment includes avoidance of the offending agent if identified and an antihistamine, and if symptoms do occur, ceasing exercise immediately to avoid a full-blown anaphylactic reaction.

Dr. Pearce is a pediatrician in Frankfort, Ill. E-mail her at [email protected].

Anaphylaxis is a relatively common occurrence for many adolescents. As primary care doctors, we normally see the patient after the acute phase, and then are required to do the detective work to figure out the causes of the episode. The cause may be obvious, but many times we have to hope for another occurrence with similar circumstances to identify it. Surprisingly, the cause may not be what you think. Factors that contribute to an anaphylaxis response may be related to activity, timing of food ingestion, an environmental factor, or medication.

Let’s look at just one type, exercise-induced anaphylaxis. It’s divided into two categories: food dependent and nonfood dependent. Both are described as an induction of itching, urticaria, and fatigue, with progression to angioedema and hypotension, associated with exercise (J. Allergy Clin. Immunol. 1980;66:106-11).

Food-dependent exercise-induced anaphylaxis occurs when exercise is started 30 minutes after ingesting food. This may be difficult to identify because patients react to the food only if they exercise, so food is usually eliminated as a cause. Wheat and wheat flour are common culprits for this type of reaction because of the omega-5 gliadin, which is the protein in gluten (J. Allergy Clin. Immunol. 1991;87:34-40). In one study, larger amounts of the suspected agent were given; hives and angioedema did start to occur in 20% of patients challenged, which suggested that there was likely a baseline allergy to the food, and exercise itself might be a cofactor in augmentation of the allergic reaction.

In nonfood-dependent exercise-induced anaphylaxis, symptoms of itching, urticaria, and fatigue can occur 5-30 minutes after the start of exercise. Although bronchospasm is rare, it can occur along with angioedema, nausea, vomiting, and hypotension, and can even be fatal if exercise continues. If exercise is stopped, it usually resolves. However, many people try to push through it, which only worsens the symptoms.

Cofactors associated with nonfood-dependent exercise-induced anaphylaxis are ingestion of alcohol and an NSAID several hours beforehand. These agents also might be overlooked if well tolerated independently (Br. J. Dermatol. 2001;145:336-9).

Timing of the episode also plays a role. Premenstrual syndrome can be a factor in augmentation of anaphylaxis, so it also should be considered. Knowing the date of the last menstrual cycle and identifying if the anaphylaxis is episodic will identify premenstrual syndrome as a cause.

The work-up should include standard allergy testing and determination of tryptase levels. Skin testing is essential to identify offending agents, and is rarely negative. If a food is suspected and skin testing is negative, repeat the skin testing in 6 months. In one study, wheat extract was found to be positive in only 29% of persons suspected of having a wheat allergy, but when the paste of wheat flour was tested, 80% were identified. The ImmunoCAP Test also was found to have a sensitivity of 80%, so it is a valuable test to try along with the skin prick.

Tryptase levels should be evaluated because in nonfood-dependent exercise-induced anaphylaxis, these levels are slightly elevated at the time of the anaphylaxis, but return to normal. A patient with mastocytosis, a group of disorders characterized by pathologic mast cells infiltrating the skin, will consistently have elevated tryptase levels. Seasonal allergies associated with pollen, and asthma bronchospasm also should be considered as causes.

Although these exercise-induced anaphylaxis episodes can occur at any age, they are most frequent in the adolescent age group, probably because that’s the time most of this population are involved in organized sports. Upon presentation, a careful detailed history will help to identify the cause of anaphylaxis and result in quicker resolution.

Treatment includes avoidance of the offending agent if identified and an antihistamine, and if symptoms do occur, ceasing exercise immediately to avoid a full-blown anaphylactic reaction.

Dr. Pearce is a pediatrician in Frankfort, Ill. E-mail her at [email protected].

Anaphylaxis is a relatively common occurrence for many adolescents. As primary care doctors, we normally see the patient after the acute phase, and then are required to do the detective work to figure out the causes of the episode. The cause may be obvious, but many times we have to hope for another occurrence with similar circumstances to identify it. Surprisingly, the cause may not be what you think. Factors that contribute to an anaphylaxis response may be related to activity, timing of food ingestion, an environmental factor, or medication.

Let’s look at just one type, exercise-induced anaphylaxis. It’s divided into two categories: food dependent and nonfood dependent. Both are described as an induction of itching, urticaria, and fatigue, with progression to angioedema and hypotension, associated with exercise (J. Allergy Clin. Immunol. 1980;66:106-11).

Food-dependent exercise-induced anaphylaxis occurs when exercise is started 30 minutes after ingesting food. This may be difficult to identify because patients react to the food only if they exercise, so food is usually eliminated as a cause. Wheat and wheat flour are common culprits for this type of reaction because of the omega-5 gliadin, which is the protein in gluten (J. Allergy Clin. Immunol. 1991;87:34-40). In one study, larger amounts of the suspected agent were given; hives and angioedema did start to occur in 20% of patients challenged, which suggested that there was likely a baseline allergy to the food, and exercise itself might be a cofactor in augmentation of the allergic reaction.

In nonfood-dependent exercise-induced anaphylaxis, symptoms of itching, urticaria, and fatigue can occur 5-30 minutes after the start of exercise. Although bronchospasm is rare, it can occur along with angioedema, nausea, vomiting, and hypotension, and can even be fatal if exercise continues. If exercise is stopped, it usually resolves. However, many people try to push through it, which only worsens the symptoms.

Cofactors associated with nonfood-dependent exercise-induced anaphylaxis are ingestion of alcohol and an NSAID several hours beforehand. These agents also might be overlooked if well tolerated independently (Br. J. Dermatol. 2001;145:336-9).

Timing of the episode also plays a role. Premenstrual syndrome can be a factor in augmentation of anaphylaxis, so it also should be considered. Knowing the date of the last menstrual cycle and identifying if the anaphylaxis is episodic will identify premenstrual syndrome as a cause.

The work-up should include standard allergy testing and determination of tryptase levels. Skin testing is essential to identify offending agents, and is rarely negative. If a food is suspected and skin testing is negative, repeat the skin testing in 6 months. In one study, wheat extract was found to be positive in only 29% of persons suspected of having a wheat allergy, but when the paste of wheat flour was tested, 80% were identified. The ImmunoCAP Test also was found to have a sensitivity of 80%, so it is a valuable test to try along with the skin prick.

Tryptase levels should be evaluated because in nonfood-dependent exercise-induced anaphylaxis, these levels are slightly elevated at the time of the anaphylaxis, but return to normal. A patient with mastocytosis, a group of disorders characterized by pathologic mast cells infiltrating the skin, will consistently have elevated tryptase levels. Seasonal allergies associated with pollen, and asthma bronchospasm also should be considered as causes.

Although these exercise-induced anaphylaxis episodes can occur at any age, they are most frequent in the adolescent age group, probably because that’s the time most of this population are involved in organized sports. Upon presentation, a careful detailed history will help to identify the cause of anaphylaxis and result in quicker resolution.

Treatment includes avoidance of the offending agent if identified and an antihistamine, and if symptoms do occur, ceasing exercise immediately to avoid a full-blown anaphylactic reaction.

Dr. Pearce is a pediatrician in Frankfort, Ill. E-mail her at [email protected].

Attention deficit/hyperactivity disorder (ADHD) has been identified in children, and appropriate treatments studied now for over half a century. The vast majority of cases that present for treatment do so after the child starts school and concerns are raised about ability to manage academics. Yet, when asked when the symptoms first began, many parents will describe onset prior to the school years – in the preschool period. But identification of ADHD in preschoolers can be difficult because of the developmental changes that are ongoing during the period from 3 to 5 years. Many of the symptoms that one would attribute to ADHD, such as increased motor activity, inattention, and distractibility are commonplace in this age group. Furthermore, some behaviors commonly associated with ADHD, such as emotional lability and obstinacy, are nearly synonymous with being a preschooler. So, how is the diagnosis made? When is it appropriate to treat? And what would that treatment look like? The following case, where symptoms of preschool ADHD go beyond typical development, provides some guides for treatment based on the evolving literature regarding preschool ADHD.

Case Summary

Johnny is a 4-year-old boy who was the product of a complicated pregnancy and delivery. Born at 35 weeks to a 17-year-old mother with a history of tobacco use disorder and depression, he spent several weeks in the special care nursery before leaving the hospital with his mother. His early temperament was described as being “difficult” with frequent episodes of colic and trouble establishing a sleep routine. His father had a history of conduct problems and school failure, and would come in and out of the family for the first 3 years. Lately, he had moved in with Johnny and his mother, and they were trying to “make a go of it.” Johnny had been slightly behind in his developmental milestones – particularly his language – but by 4 years he was able to speak in simple sentences, was able to name his colors, and had started copying circles and squares.

His parents bring Johnny in for an appointment that they made specifically to discuss his activity level and the question of ADHD, which has been brought up by multiple family members and his preschool teacher. They describe some behaviors that you have not heard about previously because they had assumed that “this is what boys did.” At age 3 years, he impulsively ran into the road after being told “no” and was nearly struck by a car. He continually tries to put things into the toaster, and they have had to get “industrial strength” plug covers because he tries to pry them off with a kitchen knife. On multiple occasions, his mother has locked herself in her bedroom because he wouldn’t stop talking to her and she couldn’t stand it anymore. When this happens, she checks often to make sure Johnny is safe, but then calls Johnny’s father home from his job as a delivery driver because she’s at her limit. In fact, Johnny’s father has been called to the preschool to bring Johnny home so many times that his father is in danger of losing his job. While Johnny appears to be a good athlete, he is often picked last for teams because he doesn’t pay attention in the game and likes to “play his own game” of tackling the other children. The stress of raising Johnny is weighing on the parents’ relationship, and Johnny’s father is considering moving out again. The parents ask for an assessment and treatment, preferably with medication.

Case Discussion

Johnny very likely has ADHD. However, to take appropriate caution in the diagnosis, one would consider that he needs to have six of nine criteria of inattention (being careless, difficulty sustaining attention, not listening, not following through, avoiding hard mental tasks, not organizing, losing important items, being easily distractible, and being forgetful) and/or six of nine criteria of hyperactivity/impulsivity (squirming/fidgeting, can’t stay seated, running or climbing excessively, can’t play quietly, “driven by a motor,” talking excessively, blurting out answers, not waiting his turn, and interrupting/intruding on others). As with school-aged ADHD, there need to be symptoms that are frequent (“often”) and that interfere with home, academic, or occupational function. One must take into account the base rate for these symptoms in preschoolers. For example, Willoughby and colleagues (J. Abnorm. Child Psychol. 2012;40:1301-12) demonstrated that at age 4 years, 26.3% of children fidget or squirm, 39.5% act as if “driven by a motor,” 46.3% talk excessively, 28.8% are easily distracted, and 25.4% have difficult waiting their turn. In fact, on average, a 4-year-old will have 1.3 inattentive items and 2.4 hyperactive-impulsive items. Still, Johnny seems to have more than his fair share. This can be validated by a) doing a careful evaluation over time using multiple informants, b) taking a family history, c) looking at developmental signs and ruling out other developmental disorders, d) making physical observations in the office (although these can be deceiving) and e) having the parents and others complete parent and caregiver checklists.

When asking parents and caregivers to complete checklists, it is crucial to make sure that these checklists look for symptoms other than just ADHD, because there are often co-occurring symptoms and disorders. These include oppositional defiant disorder, anxiety, obsessive compulsive disorder, depressive disorders, autism spectrum disorders, trauma, and learning/communication disorders. In fact, the Preschool ADHD Treatment Study (PATS) demonstrated that 71.5% of children with preschool ADHD had at least one other diagnosis and 29.7% had two or more (J. Child Adolesc. Psychopharmacol. 2007;17:563-80). Use of a broad-based instrument that captures all of these domains, in addition to attention, is warranted. In our clinic, we also assess the parents for psychopathology using the same instruments. The reason for this is, first, that family history increases the likelihood of an ADHD diagnosis and, perhaps more importantly, presence of family psychopathology makes treatment more difficult. This is because the treatment you will prescribe is going to actively involve the parents.

The treatment of choice for preschool ADHD, based on practice parameters and expert opinion, is to start with family-based behavioral treatments. There are now several empirically-based treatments that have shown efficacy for the symptoms of inattention and hyperactivity-impulsivity in preschoolers. These include Triple P (“Practitioner’s Manual for Enhanced Triple P” [Brisbane: Families International Publishing, 1998]), The Incredible Years (Webster-Stratton & Hancock, 1998), and the Revised New Forest Parent Program (Daley & Thompson, 2007), among others. If these are not available in your community, other options would be “Helping the noncompliant child: A clinician’s guide to effective parent training,” 2nd ed. (The Guilford Press: New York, 2003) or any other empirically-based parent training program. This is why it is critical to engage the parents in treatment and to refer them for treatment for their own psychopathology, if present. Furthermore, engaging the family in a program of wellness (freedom from substances, enhanced nutrition, avoidance of artificial food coloring, increased exercise), has less of a research base, but the available evidence is that it is helpful.

If medications become necessary because of safety concerns, there are few options that have a Food and Drug Administration indication. Those that do have an indication for disruptive behavior below the age of 5 years (haloperidol, dextroamphetamine, chlorpromazine, and risperidone) should not be considered as first line. The PATS study demonstrated the safety and efficacy of methylphenidate, but with optimal doses lower than those seen in school-aged children (0.7 mg/kg per day) and with increased numbers of adverse effects (11% discontinuing) (J. Am. Acad. Child Adolesc. Psychiatry 2006;45:1284-93; J. Am. Acad. Child Adolesc. Psychiatry 2006;45:1294-303).

Because of the increased amount of side effects, medication treatment cannot be considered as the first treatment. Treatment with nonstimulants is poorly studied. Any treatment with methylphenidate would be considered off-label prescribing, which must be done with great caution and, preferably, in consultation with a child and adolescent psychiatrist.

The diagnosis and management of ADHD in the very young is tricky, but possible. Doing a comprehensive evaluation with information from multiple informants, assessing and treating the parents for psychopathology, engaging the family in wellness, and starting with behavioral management is the way to go. If you feel that medication treatment is necessary for safety of the little ones, it’s best to consult, because none of the medications with FDA indication are likely to be the answer.

Dr. Althoff is associate professor of psychiatry, psychology, and pediatrics at the University of Vermont, Burlington. He is director of the division of behavioral genetics and conducts research on the development of self-regulation in children. Dr. Althoff receives no funding from pharmaceutical companies or industry. He has grant funding from the National Institute of General Medical Sciences and the Klingenstein Third Generation Foundation, and is employed, in part, by the nonprofit Research Center for Children, Youth, and Families that develops the Child Behavior Checklist and associated instruments. E-mail him at [email protected].

Attention deficit/hyperactivity disorder (ADHD) has been identified in children, and appropriate treatments studied now for over half a century. The vast majority of cases that present for treatment do so after the child starts school and concerns are raised about ability to manage academics. Yet, when asked when the symptoms first began, many parents will describe onset prior to the school years – in the preschool period. But identification of ADHD in preschoolers can be difficult because of the developmental changes that are ongoing during the period from 3 to 5 years. Many of the symptoms that one would attribute to ADHD, such as increased motor activity, inattention, and distractibility are commonplace in this age group. Furthermore, some behaviors commonly associated with ADHD, such as emotional lability and obstinacy, are nearly synonymous with being a preschooler. So, how is the diagnosis made? When is it appropriate to treat? And what would that treatment look like? The following case, where symptoms of preschool ADHD go beyond typical development, provides some guides for treatment based on the evolving literature regarding preschool ADHD.

Case Summary

Johnny is a 4-year-old boy who was the product of a complicated pregnancy and delivery. Born at 35 weeks to a 17-year-old mother with a history of tobacco use disorder and depression, he spent several weeks in the special care nursery before leaving the hospital with his mother. His early temperament was described as being “difficult” with frequent episodes of colic and trouble establishing a sleep routine. His father had a history of conduct problems and school failure, and would come in and out of the family for the first 3 years. Lately, he had moved in with Johnny and his mother, and they were trying to “make a go of it.” Johnny had been slightly behind in his developmental milestones – particularly his language – but by 4 years he was able to speak in simple sentences, was able to name his colors, and had started copying circles and squares.

His parents bring Johnny in for an appointment that they made specifically to discuss his activity level and the question of ADHD, which has been brought up by multiple family members and his preschool teacher. They describe some behaviors that you have not heard about previously because they had assumed that “this is what boys did.” At age 3 years, he impulsively ran into the road after being told “no” and was nearly struck by a car. He continually tries to put things into the toaster, and they have had to get “industrial strength” plug covers because he tries to pry them off with a kitchen knife. On multiple occasions, his mother has locked herself in her bedroom because he wouldn’t stop talking to her and she couldn’t stand it anymore. When this happens, she checks often to make sure Johnny is safe, but then calls Johnny’s father home from his job as a delivery driver because she’s at her limit. In fact, Johnny’s father has been called to the preschool to bring Johnny home so many times that his father is in danger of losing his job. While Johnny appears to be a good athlete, he is often picked last for teams because he doesn’t pay attention in the game and likes to “play his own game” of tackling the other children. The stress of raising Johnny is weighing on the parents’ relationship, and Johnny’s father is considering moving out again. The parents ask for an assessment and treatment, preferably with medication.

Case Discussion

Johnny very likely has ADHD. However, to take appropriate caution in the diagnosis, one would consider that he needs to have six of nine criteria of inattention (being careless, difficulty sustaining attention, not listening, not following through, avoiding hard mental tasks, not organizing, losing important items, being easily distractible, and being forgetful) and/or six of nine criteria of hyperactivity/impulsivity (squirming/fidgeting, can’t stay seated, running or climbing excessively, can’t play quietly, “driven by a motor,” talking excessively, blurting out answers, not waiting his turn, and interrupting/intruding on others). As with school-aged ADHD, there need to be symptoms that are frequent (“often”) and that interfere with home, academic, or occupational function. One must take into account the base rate for these symptoms in preschoolers. For example, Willoughby and colleagues (J. Abnorm. Child Psychol. 2012;40:1301-12) demonstrated that at age 4 years, 26.3% of children fidget or squirm, 39.5% act as if “driven by a motor,” 46.3% talk excessively, 28.8% are easily distracted, and 25.4% have difficult waiting their turn. In fact, on average, a 4-year-old will have 1.3 inattentive items and 2.4 hyperactive-impulsive items. Still, Johnny seems to have more than his fair share. This can be validated by a) doing a careful evaluation over time using multiple informants, b) taking a family history, c) looking at developmental signs and ruling out other developmental disorders, d) making physical observations in the office (although these can be deceiving) and e) having the parents and others complete parent and caregiver checklists.

When asking parents and caregivers to complete checklists, it is crucial to make sure that these checklists look for symptoms other than just ADHD, because there are often co-occurring symptoms and disorders. These include oppositional defiant disorder, anxiety, obsessive compulsive disorder, depressive disorders, autism spectrum disorders, trauma, and learning/communication disorders. In fact, the Preschool ADHD Treatment Study (PATS) demonstrated that 71.5% of children with preschool ADHD had at least one other diagnosis and 29.7% had two or more (J. Child Adolesc. Psychopharmacol. 2007;17:563-80). Use of a broad-based instrument that captures all of these domains, in addition to attention, is warranted. In our clinic, we also assess the parents for psychopathology using the same instruments. The reason for this is, first, that family history increases the likelihood of an ADHD diagnosis and, perhaps more importantly, presence of family psychopathology makes treatment more difficult. This is because the treatment you will prescribe is going to actively involve the parents.

The treatment of choice for preschool ADHD, based on practice parameters and expert opinion, is to start with family-based behavioral treatments. There are now several empirically-based treatments that have shown efficacy for the symptoms of inattention and hyperactivity-impulsivity in preschoolers. These include Triple P (“Practitioner’s Manual for Enhanced Triple P” [Brisbane: Families International Publishing, 1998]), The Incredible Years (Webster-Stratton & Hancock, 1998), and the Revised New Forest Parent Program (Daley & Thompson, 2007), among others. If these are not available in your community, other options would be “Helping the noncompliant child: A clinician’s guide to effective parent training,” 2nd ed. (The Guilford Press: New York, 2003) or any other empirically-based parent training program. This is why it is critical to engage the parents in treatment and to refer them for treatment for their own psychopathology, if present. Furthermore, engaging the family in a program of wellness (freedom from substances, enhanced nutrition, avoidance of artificial food coloring, increased exercise), has less of a research base, but the available evidence is that it is helpful.

If medications become necessary because of safety concerns, there are few options that have a Food and Drug Administration indication. Those that do have an indication for disruptive behavior below the age of 5 years (haloperidol, dextroamphetamine, chlorpromazine, and risperidone) should not be considered as first line. The PATS study demonstrated the safety and efficacy of methylphenidate, but with optimal doses lower than those seen in school-aged children (0.7 mg/kg per day) and with increased numbers of adverse effects (11% discontinuing) (J. Am. Acad. Child Adolesc. Psychiatry 2006;45:1284-93; J. Am. Acad. Child Adolesc. Psychiatry 2006;45:1294-303).

Because of the increased amount of side effects, medication treatment cannot be considered as the first treatment. Treatment with nonstimulants is poorly studied. Any treatment with methylphenidate would be considered off-label prescribing, which must be done with great caution and, preferably, in consultation with a child and adolescent psychiatrist.

The diagnosis and management of ADHD in the very young is tricky, but possible. Doing a comprehensive evaluation with information from multiple informants, assessing and treating the parents for psychopathology, engaging the family in wellness, and starting with behavioral management is the way to go. If you feel that medication treatment is necessary for safety of the little ones, it’s best to consult, because none of the medications with FDA indication are likely to be the answer.

Dr. Althoff is associate professor of psychiatry, psychology, and pediatrics at the University of Vermont, Burlington. He is director of the division of behavioral genetics and conducts research on the development of self-regulation in children. Dr. Althoff receives no funding from pharmaceutical companies or industry. He has grant funding from the National Institute of General Medical Sciences and the Klingenstein Third Generation Foundation, and is employed, in part, by the nonprofit Research Center for Children, Youth, and Families that develops the Child Behavior Checklist and associated instruments. E-mail him at [email protected].

Attention deficit/hyperactivity disorder (ADHD) has been identified in children, and appropriate treatments studied now for over half a century. The vast majority of cases that present for treatment do so after the child starts school and concerns are raised about ability to manage academics. Yet, when asked when the symptoms first began, many parents will describe onset prior to the school years – in the preschool period. But identification of ADHD in preschoolers can be difficult because of the developmental changes that are ongoing during the period from 3 to 5 years. Many of the symptoms that one would attribute to ADHD, such as increased motor activity, inattention, and distractibility are commonplace in this age group. Furthermore, some behaviors commonly associated with ADHD, such as emotional lability and obstinacy, are nearly synonymous with being a preschooler. So, how is the diagnosis made? When is it appropriate to treat? And what would that treatment look like? The following case, where symptoms of preschool ADHD go beyond typical development, provides some guides for treatment based on the evolving literature regarding preschool ADHD.

Case Summary

Johnny is a 4-year-old boy who was the product of a complicated pregnancy and delivery. Born at 35 weeks to a 17-year-old mother with a history of tobacco use disorder and depression, he spent several weeks in the special care nursery before leaving the hospital with his mother. His early temperament was described as being “difficult” with frequent episodes of colic and trouble establishing a sleep routine. His father had a history of conduct problems and school failure, and would come in and out of the family for the first 3 years. Lately, he had moved in with Johnny and his mother, and they were trying to “make a go of it.” Johnny had been slightly behind in his developmental milestones – particularly his language – but by 4 years he was able to speak in simple sentences, was able to name his colors, and had started copying circles and squares.

His parents bring Johnny in for an appointment that they made specifically to discuss his activity level and the question of ADHD, which has been brought up by multiple family members and his preschool teacher. They describe some behaviors that you have not heard about previously because they had assumed that “this is what boys did.” At age 3 years, he impulsively ran into the road after being told “no” and was nearly struck by a car. He continually tries to put things into the toaster, and they have had to get “industrial strength” plug covers because he tries to pry them off with a kitchen knife. On multiple occasions, his mother has locked herself in her bedroom because he wouldn’t stop talking to her and she couldn’t stand it anymore. When this happens, she checks often to make sure Johnny is safe, but then calls Johnny’s father home from his job as a delivery driver because she’s at her limit. In fact, Johnny’s father has been called to the preschool to bring Johnny home so many times that his father is in danger of losing his job. While Johnny appears to be a good athlete, he is often picked last for teams because he doesn’t pay attention in the game and likes to “play his own game” of tackling the other children. The stress of raising Johnny is weighing on the parents’ relationship, and Johnny’s father is considering moving out again. The parents ask for an assessment and treatment, preferably with medication.

Case Discussion

Johnny very likely has ADHD. However, to take appropriate caution in the diagnosis, one would consider that he needs to have six of nine criteria of inattention (being careless, difficulty sustaining attention, not listening, not following through, avoiding hard mental tasks, not organizing, losing important items, being easily distractible, and being forgetful) and/or six of nine criteria of hyperactivity/impulsivity (squirming/fidgeting, can’t stay seated, running or climbing excessively, can’t play quietly, “driven by a motor,” talking excessively, blurting out answers, not waiting his turn, and interrupting/intruding on others). As with school-aged ADHD, there need to be symptoms that are frequent (“often”) and that interfere with home, academic, or occupational function. One must take into account the base rate for these symptoms in preschoolers. For example, Willoughby and colleagues (J. Abnorm. Child Psychol. 2012;40:1301-12) demonstrated that at age 4 years, 26.3% of children fidget or squirm, 39.5% act as if “driven by a motor,” 46.3% talk excessively, 28.8% are easily distracted, and 25.4% have difficult waiting their turn. In fact, on average, a 4-year-old will have 1.3 inattentive items and 2.4 hyperactive-impulsive items. Still, Johnny seems to have more than his fair share. This can be validated by a) doing a careful evaluation over time using multiple informants, b) taking a family history, c) looking at developmental signs and ruling out other developmental disorders, d) making physical observations in the office (although these can be deceiving) and e) having the parents and others complete parent and caregiver checklists.

When asking parents and caregivers to complete checklists, it is crucial to make sure that these checklists look for symptoms other than just ADHD, because there are often co-occurring symptoms and disorders. These include oppositional defiant disorder, anxiety, obsessive compulsive disorder, depressive disorders, autism spectrum disorders, trauma, and learning/communication disorders. In fact, the Preschool ADHD Treatment Study (PATS) demonstrated that 71.5% of children with preschool ADHD had at least one other diagnosis and 29.7% had two or more (J. Child Adolesc. Psychopharmacol. 2007;17:563-80). Use of a broad-based instrument that captures all of these domains, in addition to attention, is warranted. In our clinic, we also assess the parents for psychopathology using the same instruments. The reason for this is, first, that family history increases the likelihood of an ADHD diagnosis and, perhaps more importantly, presence of family psychopathology makes treatment more difficult. This is because the treatment you will prescribe is going to actively involve the parents.

The treatment of choice for preschool ADHD, based on practice parameters and expert opinion, is to start with family-based behavioral treatments. There are now several empirically-based treatments that have shown efficacy for the symptoms of inattention and hyperactivity-impulsivity in preschoolers. These include Triple P (“Practitioner’s Manual for Enhanced Triple P” [Brisbane: Families International Publishing, 1998]), The Incredible Years (Webster-Stratton & Hancock, 1998), and the Revised New Forest Parent Program (Daley & Thompson, 2007), among others. If these are not available in your community, other options would be “Helping the noncompliant child: A clinician’s guide to effective parent training,” 2nd ed. (The Guilford Press: New York, 2003) or any other empirically-based parent training program. This is why it is critical to engage the parents in treatment and to refer them for treatment for their own psychopathology, if present. Furthermore, engaging the family in a program of wellness (freedom from substances, enhanced nutrition, avoidance of artificial food coloring, increased exercise), has less of a research base, but the available evidence is that it is helpful.

If medications become necessary because of safety concerns, there are few options that have a Food and Drug Administration indication. Those that do have an indication for disruptive behavior below the age of 5 years (haloperidol, dextroamphetamine, chlorpromazine, and risperidone) should not be considered as first line. The PATS study demonstrated the safety and efficacy of methylphenidate, but with optimal doses lower than those seen in school-aged children (0.7 mg/kg per day) and with increased numbers of adverse effects (11% discontinuing) (J. Am. Acad. Child Adolesc. Psychiatry 2006;45:1284-93; J. Am. Acad. Child Adolesc. Psychiatry 2006;45:1294-303).

Because of the increased amount of side effects, medication treatment cannot be considered as the first treatment. Treatment with nonstimulants is poorly studied. Any treatment with methylphenidate would be considered off-label prescribing, which must be done with great caution and, preferably, in consultation with a child and adolescent psychiatrist.

The diagnosis and management of ADHD in the very young is tricky, but possible. Doing a comprehensive evaluation with information from multiple informants, assessing and treating the parents for psychopathology, engaging the family in wellness, and starting with behavioral management is the way to go. If you feel that medication treatment is necessary for safety of the little ones, it’s best to consult, because none of the medications with FDA indication are likely to be the answer.

Dr. Althoff is associate professor of psychiatry, psychology, and pediatrics at the University of Vermont, Burlington. He is director of the division of behavioral genetics and conducts research on the development of self-regulation in children. Dr. Althoff receives no funding from pharmaceutical companies or industry. He has grant funding from the National Institute of General Medical Sciences and the Klingenstein Third Generation Foundation, and is employed, in part, by the nonprofit Research Center for Children, Youth, and Families that develops the Child Behavior Checklist and associated instruments. E-mail him at [email protected].

What happens when you give children everything they ask for? They get spoiled, of course. Any parent can tell you that.

The problem is that you’re trying to raise children to (eventually) be responsible adults. Part of this is teaching them that you can’t always win, you should always share, and you can’t always get what you want.

Most kids don’t like it. (I know I didn’t.) They only see that the candy or toy they want is being refused and don’t grasp the long-term plan of growing up to be a decent person. Across a thousand human cultures, any parent would agree.

But the same principle doesn’t seem to apply in modern health care. What would you think is more important in a hospital: competent staff or having a beverage offered to you after being checked into the emergency department?

Sadly, things like the latter seem to be winning because of the recent emphasis on patient satisfaction scores. In today’s world, 30% of a hospital’s Medicare reimbursement is based on these scores. That’s a lot of money.

Unfortunately, quality of care doesn’t necessarily have the same meaning between doctors and patients. The former will say it means you left the hospital with a good outcome. The latter will agree but also will throw in things like whether they got enough pain meds or their call light answered fast enough. If you’re having chest pain or severe dyspnea, getting that call light answered quickly is pretty important. But if all you want is a soda or for someone to hand you the TV remote … not so much.

The problem is that the patient satisfaction surveys (and yes, speed of call-light response is on there) don’t take that key point into account. What might make some patients happy isn’t necessarily in their best interest. The post-CABG patient who wants a double cheeseburger won’t be thrilled if he gets a salad instead. Another patient in for detox won’t be pleased if she doesn’t get Dilaudid on demand. A third will be angry that he’s not allowed to smoke. Those refusals are an integral part of their successful treatment and recovery plan, but they may not see it that way. And they’ll be sure to mark it on the survey.

As a result, the hospital gets penalized in spite of the fact that they’re doing their best to provide quality care. And the business-minded CEOs, who generally have no medical background, only care about this part of it.

Measuring what counts is important. But the idea that hospital care should be held to the same standards as Burger King and Walmart is fundamentally flawed. The things that are done in hospitals – cut people open, draw blood, biopsy bone marrow, put in endotracheal and feeding tubes – aren’t intended as recreational experiences. We try to make them as painless as possible, but in health care “do no harm” often means doing some harm in order to prevent a catastrophe.

The side effects of chemotherapy are (hopefully) offset by the successful treatment of cancer. But that doesn’t mean hair loss, nausea, vomiting, diarrhea, and other toxic symptoms are part of “customer satisfaction.” One study even found that the most satisfied patients had the highest mortality.

We owe patients the very best care we can give them, but they also need to understand that “best care” doesn’t always mean what they want in the short term. We’re focused on a goal that’s beyond the immediate horizon.

Dr. Block has a solo neurology practice in Scottsdale, Ariz.

What happens when you give children everything they ask for? They get spoiled, of course. Any parent can tell you that.

The problem is that you’re trying to raise children to (eventually) be responsible adults. Part of this is teaching them that you can’t always win, you should always share, and you can’t always get what you want.

Most kids don’t like it. (I know I didn’t.) They only see that the candy or toy they want is being refused and don’t grasp the long-term plan of growing up to be a decent person. Across a thousand human cultures, any parent would agree.

But the same principle doesn’t seem to apply in modern health care. What would you think is more important in a hospital: competent staff or having a beverage offered to you after being checked into the emergency department?

Sadly, things like the latter seem to be winning because of the recent emphasis on patient satisfaction scores. In today’s world, 30% of a hospital’s Medicare reimbursement is based on these scores. That’s a lot of money.

Unfortunately, quality of care doesn’t necessarily have the same meaning between doctors and patients. The former will say it means you left the hospital with a good outcome. The latter will agree but also will throw in things like whether they got enough pain meds or their call light answered fast enough. If you’re having chest pain or severe dyspnea, getting that call light answered quickly is pretty important. But if all you want is a soda or for someone to hand you the TV remote … not so much.

The problem is that the patient satisfaction surveys (and yes, speed of call-light response is on there) don’t take that key point into account. What might make some patients happy isn’t necessarily in their best interest. The post-CABG patient who wants a double cheeseburger won’t be thrilled if he gets a salad instead. Another patient in for detox won’t be pleased if she doesn’t get Dilaudid on demand. A third will be angry that he’s not allowed to smoke. Those refusals are an integral part of their successful treatment and recovery plan, but they may not see it that way. And they’ll be sure to mark it on the survey.

As a result, the hospital gets penalized in spite of the fact that they’re doing their best to provide quality care. And the business-minded CEOs, who generally have no medical background, only care about this part of it.

Measuring what counts is important. But the idea that hospital care should be held to the same standards as Burger King and Walmart is fundamentally flawed. The things that are done in hospitals – cut people open, draw blood, biopsy bone marrow, put in endotracheal and feeding tubes – aren’t intended as recreational experiences. We try to make them as painless as possible, but in health care “do no harm” often means doing some harm in order to prevent a catastrophe.

The side effects of chemotherapy are (hopefully) offset by the successful treatment of cancer. But that doesn’t mean hair loss, nausea, vomiting, diarrhea, and other toxic symptoms are part of “customer satisfaction.” One study even found that the most satisfied patients had the highest mortality.

We owe patients the very best care we can give them, but they also need to understand that “best care” doesn’t always mean what they want in the short term. We’re focused on a goal that’s beyond the immediate horizon.

Dr. Block has a solo neurology practice in Scottsdale, Ariz.

What happens when you give children everything they ask for? They get spoiled, of course. Any parent can tell you that.

The problem is that you’re trying to raise children to (eventually) be responsible adults. Part of this is teaching them that you can’t always win, you should always share, and you can’t always get what you want.

Most kids don’t like it. (I know I didn’t.) They only see that the candy or toy they want is being refused and don’t grasp the long-term plan of growing up to be a decent person. Across a thousand human cultures, any parent would agree.

But the same principle doesn’t seem to apply in modern health care. What would you think is more important in a hospital: competent staff or having a beverage offered to you after being checked into the emergency department?

Sadly, things like the latter seem to be winning because of the recent emphasis on patient satisfaction scores. In today’s world, 30% of a hospital’s Medicare reimbursement is based on these scores. That’s a lot of money.

Unfortunately, quality of care doesn’t necessarily have the same meaning between doctors and patients. The former will say it means you left the hospital with a good outcome. The latter will agree but also will throw in things like whether they got enough pain meds or their call light answered fast enough. If you’re having chest pain or severe dyspnea, getting that call light answered quickly is pretty important. But if all you want is a soda or for someone to hand you the TV remote … not so much.

The problem is that the patient satisfaction surveys (and yes, speed of call-light response is on there) don’t take that key point into account. What might make some patients happy isn’t necessarily in their best interest. The post-CABG patient who wants a double cheeseburger won’t be thrilled if he gets a salad instead. Another patient in for detox won’t be pleased if she doesn’t get Dilaudid on demand. A third will be angry that he’s not allowed to smoke. Those refusals are an integral part of their successful treatment and recovery plan, but they may not see it that way. And they’ll be sure to mark it on the survey.

As a result, the hospital gets penalized in spite of the fact that they’re doing their best to provide quality care. And the business-minded CEOs, who generally have no medical background, only care about this part of it.

Measuring what counts is important. But the idea that hospital care should be held to the same standards as Burger King and Walmart is fundamentally flawed. The things that are done in hospitals – cut people open, draw blood, biopsy bone marrow, put in endotracheal and feeding tubes – aren’t intended as recreational experiences. We try to make them as painless as possible, but in health care “do no harm” often means doing some harm in order to prevent a catastrophe.

The side effects of chemotherapy are (hopefully) offset by the successful treatment of cancer. But that doesn’t mean hair loss, nausea, vomiting, diarrhea, and other toxic symptoms are part of “customer satisfaction.” One study even found that the most satisfied patients had the highest mortality.

We owe patients the very best care we can give them, but they also need to understand that “best care” doesn’t always mean what they want in the short term. We’re focused on a goal that’s beyond the immediate horizon.

Dr. Block has a solo neurology practice in Scottsdale, Ariz.

With the increase in cesarean sections worldwide, it is imperative that physicians properly inform their patients as to potential procedure risks. One potential postcesarean section problem that is receiving increasing attention is the isthmocele or niche.

Defined as an anechoic area in the cesarean section scar, it has been noted to occur in 24%-69% of women undergoing transvaginal sonography, and 56%-78% of women evaluated with transvaginal saline infused sonogram. While most cesarean section defects are asymptomatic, the isthmocele has been noted to be associated with abnormal uterine bleeding, including prolonged menstruation or postmenopausal spotting, and fertility concerns (BJOG. 2014;121:145-56).

Interestingly, it has been 40 years since Stewart, et al. first reported the relationship of abnormal uterine bleeding and cesarean section (Br. J. Gynaecol. 1975;82:682-6). Bloody fluid can be generated at the isthmocele site, which travels up the endometrial canal, thus impacting implantation. The niche can also be the site of ectopic pregnancy implantation.

In this edition of Master Class in gynecologic surgery, I have asked my newest partner, Dr. Kirsten Sasaki, to share our views on this increasingly important subject. Dr. Sasaki completed her internship and residency at Tufts Medical Center, Boston, where she was awarded the Outstanding Chief Resident Clinician Award. Dr. Sasaki then went on to become our second fellow in the Fellowship in Minimally Invasive Gynecologic Surgery in affiliation with AAGL and SRS at Advocate Lutheran General Hospital, Park Ridge, Ill. Once again, Dr. Sasaki was singled out for her excellent teaching and research capabilities. Ultimately however, it was her tremendous surgical skills and surgical sense that led Dr. Aarathi Cholkeri-Singh and I to invite her into our practice.

Dr. Miller is clinical associate professor at the University of Illinois at Chicago, immediate past president of the International Society for Gynecologic Endoscopy (ISGE), and a past president of the AAGL. He is a reproductive endocrinologist and minimally invasive gynecologic surgeon in private practice in Naperville, Ill., and Schaumburg, Ill.; the director of minimally invasive gynecologic surgery and the director of the AAGL/SRS fellowship in minimally invasive gynecologic surgery at Advocate Lutheran General Hospital, Park Ridge, Ill.; and the medical editor of this column, Master Class. Dr. Miller is a consultant and on the speakers bureau for Ethicon. He is also a consultant, on the speakers bureau, and has received grant and research support from Intuitive Surgical.

With the increase in cesarean sections worldwide, it is imperative that physicians properly inform their patients as to potential procedure risks. One potential postcesarean section problem that is receiving increasing attention is the isthmocele or niche.

Defined as an anechoic area in the cesarean section scar, it has been noted to occur in 24%-69% of women undergoing transvaginal sonography, and 56%-78% of women evaluated with transvaginal saline infused sonogram. While most cesarean section defects are asymptomatic, the isthmocele has been noted to be associated with abnormal uterine bleeding, including prolonged menstruation or postmenopausal spotting, and fertility concerns (BJOG. 2014;121:145-56).

Interestingly, it has been 40 years since Stewart, et al. first reported the relationship of abnormal uterine bleeding and cesarean section (Br. J. Gynaecol. 1975;82:682-6). Bloody fluid can be generated at the isthmocele site, which travels up the endometrial canal, thus impacting implantation. The niche can also be the site of ectopic pregnancy implantation.

In this edition of Master Class in gynecologic surgery, I have asked my newest partner, Dr. Kirsten Sasaki, to share our views on this increasingly important subject. Dr. Sasaki completed her internship and residency at Tufts Medical Center, Boston, where she was awarded the Outstanding Chief Resident Clinician Award. Dr. Sasaki then went on to become our second fellow in the Fellowship in Minimally Invasive Gynecologic Surgery in affiliation with AAGL and SRS at Advocate Lutheran General Hospital, Park Ridge, Ill. Once again, Dr. Sasaki was singled out for her excellent teaching and research capabilities. Ultimately however, it was her tremendous surgical skills and surgical sense that led Dr. Aarathi Cholkeri-Singh and I to invite her into our practice.

Dr. Miller is clinical associate professor at the University of Illinois at Chicago, immediate past president of the International Society for Gynecologic Endoscopy (ISGE), and a past president of the AAGL. He is a reproductive endocrinologist and minimally invasive gynecologic surgeon in private practice in Naperville, Ill., and Schaumburg, Ill.; the director of minimally invasive gynecologic surgery and the director of the AAGL/SRS fellowship in minimally invasive gynecologic surgery at Advocate Lutheran General Hospital, Park Ridge, Ill.; and the medical editor of this column, Master Class. Dr. Miller is a consultant and on the speakers bureau for Ethicon. He is also a consultant, on the speakers bureau, and has received grant and research support from Intuitive Surgical.

With the increase in cesarean sections worldwide, it is imperative that physicians properly inform their patients as to potential procedure risks. One potential postcesarean section problem that is receiving increasing attention is the isthmocele or niche.

Defined as an anechoic area in the cesarean section scar, it has been noted to occur in 24%-69% of women undergoing transvaginal sonography, and 56%-78% of women evaluated with transvaginal saline infused sonogram. While most cesarean section defects are asymptomatic, the isthmocele has been noted to be associated with abnormal uterine bleeding, including prolonged menstruation or postmenopausal spotting, and fertility concerns (BJOG. 2014;121:145-56).

Interestingly, it has been 40 years since Stewart, et al. first reported the relationship of abnormal uterine bleeding and cesarean section (Br. J. Gynaecol. 1975;82:682-6). Bloody fluid can be generated at the isthmocele site, which travels up the endometrial canal, thus impacting implantation. The niche can also be the site of ectopic pregnancy implantation.

In this edition of Master Class in gynecologic surgery, I have asked my newest partner, Dr. Kirsten Sasaki, to share our views on this increasingly important subject. Dr. Sasaki completed her internship and residency at Tufts Medical Center, Boston, where she was awarded the Outstanding Chief Resident Clinician Award. Dr. Sasaki then went on to become our second fellow in the Fellowship in Minimally Invasive Gynecologic Surgery in affiliation with AAGL and SRS at Advocate Lutheran General Hospital, Park Ridge, Ill. Once again, Dr. Sasaki was singled out for her excellent teaching and research capabilities. Ultimately however, it was her tremendous surgical skills and surgical sense that led Dr. Aarathi Cholkeri-Singh and I to invite her into our practice.

Dr. Miller is clinical associate professor at the University of Illinois at Chicago, immediate past president of the International Society for Gynecologic Endoscopy (ISGE), and a past president of the AAGL. He is a reproductive endocrinologist and minimally invasive gynecologic surgeon in private practice in Naperville, Ill., and Schaumburg, Ill.; the director of minimally invasive gynecologic surgery and the director of the AAGL/SRS fellowship in minimally invasive gynecologic surgery at Advocate Lutheran General Hospital, Park Ridge, Ill.; and the medical editor of this column, Master Class. Dr. Miller is a consultant and on the speakers bureau for Ethicon. He is also a consultant, on the speakers bureau, and has received grant and research support from Intuitive Surgical.

In recent years, uterine isthmocele has increasingly been included as part of the differential in women with a history of a cesarean section who present with postmenstrual bleeding, pelvic pain, or secondary infertility.

The defect appears as a fluid-filled, pouch-like abnormality in the anterior uterine wall at the site of a prior cesarean section. The best method for diagnosis is usually a saline-infused sonogram. It can be treated in various ways, depending on the patient’s symptoms and desire for future fertility. Although we have treated isthmoceles with hysteroscopic desiccation, or resection, our best success has occurred with laparoscopic resection and reapproximation of normal tissue in a small series of patients.

There is no standard definition of the defect that fully describes its size, depth, and other characteristics. Many words and phrases have been used to describe the defect: It is commonly referred to as an isthmocele, because of its usual location at the uterine isthmus, but others have referred to it as a cesarean scar defect or niche, as the defect may be found at the endocervical canal or in the lower uterine segment. In any case, while diagnoses appear to be increasing, the incidence of the defect is unknown.

More research on risk factors and treatment is needed, but the literature, as well as our own experience, has demonstrated that this treatable defect should be considered in the differential diagnosis for women who have undergone cesarean section and subsequently have abnormal bleeding or staining, pelvic pain, or secondary infertility, especially when fluid is clearly visible in the cesarean section defect.

Diagnosis, symptoms

An isthmocele forms in the first place, it is thought, after an incision scar forms and causes retraction and dilation in the thinner, lower segment of the anterior wall and a thickening in the upper portion. There is a deficient scar, in other words, with disparate wound healing on the sides of the incision site.

The defect and its consequences were described in 1995 by Dr. Hugh Morris, who studied hysterectomy specimens in 51 women with a history of cesarean section (in most cases, more than one). Dr. Morris concluded that scar tissue in these patients contributed to significant pathological changes and anatomical abnormalities that, in turn, gave rise to a variety of clinical symptoms including menorrhagia, dysmenorrhea, dyspareunia, and lower abdominal pain refractory to medical management.

Distortion and widening of the lower uterine segment and “free” red blood cells in endometrial stroma of the scar were the most frequently identified pathological changes, followed by fragmentation and breakdown of the endometrium of the scar, and iatrogenic adenomyosis (Int. J. Gynecol. Pathol.1995;14:16-20).

Several small reports and case series published in the late 1990s offered additional support for a cause-and-effect correlation between cesarean scar defects and abnormal vaginal bleeding. Several years later, the link was strengthened as more investigators reported connections between the defects and various symptoms. These reports were followed by published comparisons of imaging techniques for the diagnosis of isthmoceles.

Diagnosis of the defects can be made with transvaginal ultrasound (TVUS), saline infused sonohysterogram (SIS), hysterosalpingogram, hysteroscopy, and magnetic resonance imaging (MRI). With any modality, imaging is best performed in the early proliferative phase, right after the menstrual cycle has ended.

Courtesy of Dr. Kirsten Sasaki

Comparisons of unenhanced TVUS and SIS – both of which may be easily performed in the office and at a much lower cost than MRI – have shown the latter technique to be superior for evaluating isthmoceles. Distension of the endometrial cavity makes the borders of the defects easier to delineate, which enables detection of more subtle defects and improves our ability to measure the size of defects.

This advantage was described by in 2010 by Dr. O. Vikhareva Osser and colleagues, who performed both TVUS and SIS in 108 women with a history of one or more cesarean sections. They identified more scar defects with SIS than with TVUS (Ultrasound Obstet. Gynecol. 2010;35:75-83).

Another benefit of SIS over TVUS and hysterosalpingogram is that one can measure the thickness of the remaining myometrium overlying the isthmocele, which is especially important knowledge for patients considering another pregnancy. As a result, we have relied on this technique to diagnose every case within our practice. I will perform SIS in a patient who has a history of one or multiple cesarean sections and symptoms of abnormal bleeding, pelvic pain, or secondary infertility as part of the basic work-up.

Similarly, an observational prospective cohort study of 225 women who had undergone a cesarean section 6-12 months prior compared TVUS and gel-infused sonohysterogram (GIS), and found that the prevalence of a niche – defined as an anechoic area at the site of the cesarean scar, with a depth of at least 1 mm on GIS – was 24% with TVUS and 56% with GIS (Ultrasound Obstet. Gynecol. 2011;37:93-9).

The abnormal bleeding is often described by patients as spotting or bleeding that continues for days or weeks after menstrual flow has ended; it is believed to result from an accumulation of blood in the defect and a lack of coordinated muscle contractions, which leads to continued accumulation of blood and menstrual debris. Dysmenorrhea and chronic pelvic pain are thought to be associated with iatrogenic adenomyosis and/or a chronic inflammatory state created when accumulated blood and mucus are intermittently expelled. Secondary infertility can occur, it is believed, as accumulated fluid and blood interfere with the endocervical and even the endometrial environment and disrupt sperm transport, sperm quality, and embryo implantation. Difficulty in embryo transfer may also occur because of the distortion caused to the endometrial cavity. Many of the isthmoceles that we and others have diagnosed have been in patients undergoing invitro fertilization. The patients are often found to have an accumulation of fluid in the endometrial canal and isthmocele during stimulation for either a fresh or frozen embryo transfer, thus necessitating the cancellation of their cycle.

Treatment

The choice of treatment depends upon the patient’s symptoms and desire for future fertility, but it can include hormonal treatment, hysteroscopic resection, transvaginal repair, a laparoscopic or robot-assisted approach, and hysterectomy.

Little has been published on nonsurgical treatment, but this may be considered for patients whose primary symptoms are bleeding or pain and who desire the least invasive option. In a small observational study of women with an isthmocele and bleeding, symptoms were eliminated with several cycles of oral contraceptive pills (Fertil. Steril. 2006;86: 477-9).

Courtesy of Dr. Kirsten Sasaki

Hysteroscopic isthmocele correction or resection are the surgical techniques most frequently described in the literature, but, as with other surgical approaches, studies are small. Hysteroscopic repair has typically involved the use of electrical energy to desiccate or cauterize abnormal tissue and eliminate the outpouching in which blood and fluid accumulate. Hysteroscopic resection is another technique that has also been championed.

However, for patients who desire future pregnancy, we do not recommend a hysteroscopic approach because it does not reinforce the often-thinning myometrium covering the defect. We are concerned that if this area is simply desiccated or resected, and not reapproximated, the patient will be at greater risk of pregnancy-related complications, including cesarean scar ectopic pregnancy with potential uterine dehiscence.

Laparoscopic repair was first described by Dr. Olivier Donnez, who rightly pointed out that the laparoscopic approach offers an optimal view from above during dissection of the vesico-vaginal space. Dr. Donnez used a CO₂ laser to excise fibrotic tissue, followed by laparoscopic closure (Fertil. Steril. 2008;89:974-80).

We have had success with a laparoscopic approach that uses concomitant hysteroscopy. The vesico-uterine peritoneum is incised over the anterior uterine wall, and the bladder is backfilled so that its boundaries may be identified prior to further dissection. With the area exposed, we perform a hysteroscopy to determine the exact location of the isthmocele. As the hysteroscope enters the thinned out isthmocele, the light will be more visible via laparoscopic visualization.

When performing conventional laparoscopy, the isthmocele is excised with an ultrasonic curved blade. We use this instrument because it has no opposing arm and because it enables precise tissue dissection in multiple planes. With harmonic energy, we can limit tissue dessication and destruction, lowering the risk of future pregnancy-related complications. Monopolar scissors are best when a robotic approach is used.

Once the isthmocele is resected, the clean edges are sutured together in two layers. The first layer is sutured in an interrupted mattress-style fashion, to prevent tissue strangulation and necrosis. We use a monofilament nonbarbed delayed-absorbable 3-0 PDS suture on a CT-1 needle – a choice that limits tissue trauma and postoperative inflammation.

Courtesy of Dr. Kirsten Sasaki

Sutures are initially placed at each angle with one or two sutures placed between. These sutures must be placed deep to close the bottom of the defect. A second layer of suture is then placed to imbricate over the initial layer of closure. We utilize 3-0 PDS in a running or mattress style, or a running 3-0 V-Loc suture. Our patients return after 1-3 months for a postoperative image, and are instructed to wait at least 3 months after surgery before attempting conception.

In our experience, of more than 10 patients, symptoms ceased in all patients whose surgery was performed for the indication of abnormal uterine bleeding. The follow-up on our series of patients who underwent the procedure for secondary infertility is ongoing, but the preliminary results are very positive, with resolution of intrauterine fluid in all of the patients, as well as several successful pregnancy outcomes.

A recent systematic review of minimally invasive therapy for symptoms related to an isthmocele shows good outcomes across the 12 included studies but does not offer evidence to favor one treatment over another. The studies show significant reductions in abnormal uterine bleeding and pain, as well as a high rate of satisfaction in most patients after hysteroscopic niche resection or vaginal or laparoscopic niche repair, with a low complication rate (BJOG 2014;121:145-6).

Pregnancies were reported after treatment, but sample sizes and follow-up were insufficient to draw conclusions on pregnancy and delivery outcomes, according to the review. As the reviewers wrote, following patients through their next delivery in larger, higher-quality studies will help provide more guidance for selecting the best isthmocele treatments and implementing these treatments into practice.

Dr. Sasaki reported having no financial disclosures relevant to this Master Class.

In recent years, uterine isthmocele has increasingly been included as part of the differential in women with a history of a cesarean section who present with postmenstrual bleeding, pelvic pain, or secondary infertility.

The defect appears as a fluid-filled, pouch-like abnormality in the anterior uterine wall at the site of a prior cesarean section. The best method for diagnosis is usually a saline-infused sonogram. It can be treated in various ways, depending on the patient’s symptoms and desire for future fertility. Although we have treated isthmoceles with hysteroscopic desiccation, or resection, our best success has occurred with laparoscopic resection and reapproximation of normal tissue in a small series of patients.

There is no standard definition of the defect that fully describes its size, depth, and other characteristics. Many words and phrases have been used to describe the defect: It is commonly referred to as an isthmocele, because of its usual location at the uterine isthmus, but others have referred to it as a cesarean scar defect or niche, as the defect may be found at the endocervical canal or in the lower uterine segment. In any case, while diagnoses appear to be increasing, the incidence of the defect is unknown.

More research on risk factors and treatment is needed, but the literature, as well as our own experience, has demonstrated that this treatable defect should be considered in the differential diagnosis for women who have undergone cesarean section and subsequently have abnormal bleeding or staining, pelvic pain, or secondary infertility, especially when fluid is clearly visible in the cesarean section defect.

Diagnosis, symptoms

An isthmocele forms in the first place, it is thought, after an incision scar forms and causes retraction and dilation in the thinner, lower segment of the anterior wall and a thickening in the upper portion. There is a deficient scar, in other words, with disparate wound healing on the sides of the incision site.

The defect and its consequences were described in 1995 by Dr. Hugh Morris, who studied hysterectomy specimens in 51 women with a history of cesarean section (in most cases, more than one). Dr. Morris concluded that scar tissue in these patients contributed to significant pathological changes and anatomical abnormalities that, in turn, gave rise to a variety of clinical symptoms including menorrhagia, dysmenorrhea, dyspareunia, and lower abdominal pain refractory to medical management.

Distortion and widening of the lower uterine segment and “free” red blood cells in endometrial stroma of the scar were the most frequently identified pathological changes, followed by fragmentation and breakdown of the endometrium of the scar, and iatrogenic adenomyosis (Int. J. Gynecol. Pathol.1995;14:16-20).

Several small reports and case series published in the late 1990s offered additional support for a cause-and-effect correlation between cesarean scar defects and abnormal vaginal bleeding. Several years later, the link was strengthened as more investigators reported connections between the defects and various symptoms. These reports were followed by published comparisons of imaging techniques for the diagnosis of isthmoceles.

Diagnosis of the defects can be made with transvaginal ultrasound (TVUS), saline infused sonohysterogram (SIS), hysterosalpingogram, hysteroscopy, and magnetic resonance imaging (MRI). With any modality, imaging is best performed in the early proliferative phase, right after the menstrual cycle has ended.

Courtesy of Dr. Kirsten Sasaki

Comparisons of unenhanced TVUS and SIS – both of which may be easily performed in the office and at a much lower cost than MRI – have shown the latter technique to be superior for evaluating isthmoceles. Distension of the endometrial cavity makes the borders of the defects easier to delineate, which enables detection of more subtle defects and improves our ability to measure the size of defects.

This advantage was described by in 2010 by Dr. O. Vikhareva Osser and colleagues, who performed both TVUS and SIS in 108 women with a history of one or more cesarean sections. They identified more scar defects with SIS than with TVUS (Ultrasound Obstet. Gynecol. 2010;35:75-83).

Another benefit of SIS over TVUS and hysterosalpingogram is that one can measure the thickness of the remaining myometrium overlying the isthmocele, which is especially important knowledge for patients considering another pregnancy. As a result, we have relied on this technique to diagnose every case within our practice. I will perform SIS in a patient who has a history of one or multiple cesarean sections and symptoms of abnormal bleeding, pelvic pain, or secondary infertility as part of the basic work-up.

Similarly, an observational prospective cohort study of 225 women who had undergone a cesarean section 6-12 months prior compared TVUS and gel-infused sonohysterogram (GIS), and found that the prevalence of a niche – defined as an anechoic area at the site of the cesarean scar, with a depth of at least 1 mm on GIS – was 24% with TVUS and 56% with GIS (Ultrasound Obstet. Gynecol. 2011;37:93-9).

The abnormal bleeding is often described by patients as spotting or bleeding that continues for days or weeks after menstrual flow has ended; it is believed to result from an accumulation of blood in the defect and a lack of coordinated muscle contractions, which leads to continued accumulation of blood and menstrual debris. Dysmenorrhea and chronic pelvic pain are thought to be associated with iatrogenic adenomyosis and/or a chronic inflammatory state created when accumulated blood and mucus are intermittently expelled. Secondary infertility can occur, it is believed, as accumulated fluid and blood interfere with the endocervical and even the endometrial environment and disrupt sperm transport, sperm quality, and embryo implantation. Difficulty in embryo transfer may also occur because of the distortion caused to the endometrial cavity. Many of the isthmoceles that we and others have diagnosed have been in patients undergoing invitro fertilization. The patients are often found to have an accumulation of fluid in the endometrial canal and isthmocele during stimulation for either a fresh or frozen embryo transfer, thus necessitating the cancellation of their cycle.

Treatment

The choice of treatment depends upon the patient’s symptoms and desire for future fertility, but it can include hormonal treatment, hysteroscopic resection, transvaginal repair, a laparoscopic or robot-assisted approach, and hysterectomy.

Little has been published on nonsurgical treatment, but this may be considered for patients whose primary symptoms are bleeding or pain and who desire the least invasive option. In a small observational study of women with an isthmocele and bleeding, symptoms were eliminated with several cycles of oral contraceptive pills (Fertil. Steril. 2006;86: 477-9).

Courtesy of Dr. Kirsten Sasaki

Hysteroscopic isthmocele correction or resection are the surgical techniques most frequently described in the literature, but, as with other surgical approaches, studies are small. Hysteroscopic repair has typically involved the use of electrical energy to desiccate or cauterize abnormal tissue and eliminate the outpouching in which blood and fluid accumulate. Hysteroscopic resection is another technique that has also been championed.

However, for patients who desire future pregnancy, we do not recommend a hysteroscopic approach because it does not reinforce the often-thinning myometrium covering the defect. We are concerned that if this area is simply desiccated or resected, and not reapproximated, the patient will be at greater risk of pregnancy-related complications, including cesarean scar ectopic pregnancy with potential uterine dehiscence.

Laparoscopic repair was first described by Dr. Olivier Donnez, who rightly pointed out that the laparoscopic approach offers an optimal view from above during dissection of the vesico-vaginal space. Dr. Donnez used a CO₂ laser to excise fibrotic tissue, followed by laparoscopic closure (Fertil. Steril. 2008;89:974-80).

We have had success with a laparoscopic approach that uses concomitant hysteroscopy. The vesico-uterine peritoneum is incised over the anterior uterine wall, and the bladder is backfilled so that its boundaries may be identified prior to further dissection. With the area exposed, we perform a hysteroscopy to determine the exact location of the isthmocele. As the hysteroscope enters the thinned out isthmocele, the light will be more visible via laparoscopic visualization.

When performing conventional laparoscopy, the isthmocele is excised with an ultrasonic curved blade. We use this instrument because it has no opposing arm and because it enables precise tissue dissection in multiple planes. With harmonic energy, we can limit tissue dessication and destruction, lowering the risk of future pregnancy-related complications. Monopolar scissors are best when a robotic approach is used.

Once the isthmocele is resected, the clean edges are sutured together in two layers. The first layer is sutured in an interrupted mattress-style fashion, to prevent tissue strangulation and necrosis. We use a monofilament nonbarbed delayed-absorbable 3-0 PDS suture on a CT-1 needle – a choice that limits tissue trauma and postoperative inflammation.

Courtesy of Dr. Kirsten Sasaki

Sutures are initially placed at each angle with one or two sutures placed between. These sutures must be placed deep to close the bottom of the defect. A second layer of suture is then placed to imbricate over the initial layer of closure. We utilize 3-0 PDS in a running or mattress style, or a running 3-0 V-Loc suture. Our patients return after 1-3 months for a postoperative image, and are instructed to wait at least 3 months after surgery before attempting conception.

In our experience, of more than 10 patients, symptoms ceased in all patients whose surgery was performed for the indication of abnormal uterine bleeding. The follow-up on our series of patients who underwent the procedure for secondary infertility is ongoing, but the preliminary results are very positive, with resolution of intrauterine fluid in all of the patients, as well as several successful pregnancy outcomes.

A recent systematic review of minimally invasive therapy for symptoms related to an isthmocele shows good outcomes across the 12 included studies but does not offer evidence to favor one treatment over another. The studies show significant reductions in abnormal uterine bleeding and pain, as well as a high rate of satisfaction in most patients after hysteroscopic niche resection or vaginal or laparoscopic niche repair, with a low complication rate (BJOG 2014;121:145-6).

Pregnancies were reported after treatment, but sample sizes and follow-up were insufficient to draw conclusions on pregnancy and delivery outcomes, according to the review. As the reviewers wrote, following patients through their next delivery in larger, higher-quality studies will help provide more guidance for selecting the best isthmocele treatments and implementing these treatments into practice.

Dr. Sasaki reported having no financial disclosures relevant to this Master Class.

In recent years, uterine isthmocele has increasingly been included as part of the differential in women with a history of a cesarean section who present with postmenstrual bleeding, pelvic pain, or secondary infertility.

The defect appears as a fluid-filled, pouch-like abnormality in the anterior uterine wall at the site of a prior cesarean section. The best method for diagnosis is usually a saline-infused sonogram. It can be treated in various ways, depending on the patient’s symptoms and desire for future fertility. Although we have treated isthmoceles with hysteroscopic desiccation, or resection, our best success has occurred with laparoscopic resection and reapproximation of normal tissue in a small series of patients.

There is no standard definition of the defect that fully describes its size, depth, and other characteristics. Many words and phrases have been used to describe the defect: It is commonly referred to as an isthmocele, because of its usual location at the uterine isthmus, but others have referred to it as a cesarean scar defect or niche, as the defect may be found at the endocervical canal or in the lower uterine segment. In any case, while diagnoses appear to be increasing, the incidence of the defect is unknown.

More research on risk factors and treatment is needed, but the literature, as well as our own experience, has demonstrated that this treatable defect should be considered in the differential diagnosis for women who have undergone cesarean section and subsequently have abnormal bleeding or staining, pelvic pain, or secondary infertility, especially when fluid is clearly visible in the cesarean section defect.

Diagnosis, symptoms

An isthmocele forms in the first place, it is thought, after an incision scar forms and causes retraction and dilation in the thinner, lower segment of the anterior wall and a thickening in the upper portion. There is a deficient scar, in other words, with disparate wound healing on the sides of the incision site.

The defect and its consequences were described in 1995 by Dr. Hugh Morris, who studied hysterectomy specimens in 51 women with a history of cesarean section (in most cases, more than one). Dr. Morris concluded that scar tissue in these patients contributed to significant pathological changes and anatomical abnormalities that, in turn, gave rise to a variety of clinical symptoms including menorrhagia, dysmenorrhea, dyspareunia, and lower abdominal pain refractory to medical management.

Distortion and widening of the lower uterine segment and “free” red blood cells in endometrial stroma of the scar were the most frequently identified pathological changes, followed by fragmentation and breakdown of the endometrium of the scar, and iatrogenic adenomyosis (Int. J. Gynecol. Pathol.1995;14:16-20).

Several small reports and case series published in the late 1990s offered additional support for a cause-and-effect correlation between cesarean scar defects and abnormal vaginal bleeding. Several years later, the link was strengthened as more investigators reported connections between the defects and various symptoms. These reports were followed by published comparisons of imaging techniques for the diagnosis of isthmoceles.

Diagnosis of the defects can be made with transvaginal ultrasound (TVUS), saline infused sonohysterogram (SIS), hysterosalpingogram, hysteroscopy, and magnetic resonance imaging (MRI). With any modality, imaging is best performed in the early proliferative phase, right after the menstrual cycle has ended.

Courtesy of Dr. Kirsten Sasaki

Comparisons of unenhanced TVUS and SIS – both of which may be easily performed in the office and at a much lower cost than MRI – have shown the latter technique to be superior for evaluating isthmoceles. Distension of the endometrial cavity makes the borders of the defects easier to delineate, which enables detection of more subtle defects and improves our ability to measure the size of defects.

This advantage was described by in 2010 by Dr. O. Vikhareva Osser and colleagues, who performed both TVUS and SIS in 108 women with a history of one or more cesarean sections. They identified more scar defects with SIS than with TVUS (Ultrasound Obstet. Gynecol. 2010;35:75-83).

Another benefit of SIS over TVUS and hysterosalpingogram is that one can measure the thickness of the remaining myometrium overlying the isthmocele, which is especially important knowledge for patients considering another pregnancy. As a result, we have relied on this technique to diagnose every case within our practice. I will perform SIS in a patient who has a history of one or multiple cesarean sections and symptoms of abnormal bleeding, pelvic pain, or secondary infertility as part of the basic work-up.

Similarly, an observational prospective cohort study of 225 women who had undergone a cesarean section 6-12 months prior compared TVUS and gel-infused sonohysterogram (GIS), and found that the prevalence of a niche – defined as an anechoic area at the site of the cesarean scar, with a depth of at least 1 mm on GIS – was 24% with TVUS and 56% with GIS (Ultrasound Obstet. Gynecol. 2011;37:93-9).

The abnormal bleeding is often described by patients as spotting or bleeding that continues for days or weeks after menstrual flow has ended; it is believed to result from an accumulation of blood in the defect and a lack of coordinated muscle contractions, which leads to continued accumulation of blood and menstrual debris. Dysmenorrhea and chronic pelvic pain are thought to be associated with iatrogenic adenomyosis and/or a chronic inflammatory state created when accumulated blood and mucus are intermittently expelled. Secondary infertility can occur, it is believed, as accumulated fluid and blood interfere with the endocervical and even the endometrial environment and disrupt sperm transport, sperm quality, and embryo implantation. Difficulty in embryo transfer may also occur because of the distortion caused to the endometrial cavity. Many of the isthmoceles that we and others have diagnosed have been in patients undergoing invitro fertilization. The patients are often found to have an accumulation of fluid in the endometrial canal and isthmocele during stimulation for either a fresh or frozen embryo transfer, thus necessitating the cancellation of their cycle.

Treatment

The choice of treatment depends upon the patient’s symptoms and desire for future fertility, but it can include hormonal treatment, hysteroscopic resection, transvaginal repair, a laparoscopic or robot-assisted approach, and hysterectomy.

Little has been published on nonsurgical treatment, but this may be considered for patients whose primary symptoms are bleeding or pain and who desire the least invasive option. In a small observational study of women with an isthmocele and bleeding, symptoms were eliminated with several cycles of oral contraceptive pills (Fertil. Steril. 2006;86: 477-9).

Courtesy of Dr. Kirsten Sasaki

Hysteroscopic isthmocele correction or resection are the surgical techniques most frequently described in the literature, but, as with other surgical approaches, studies are small. Hysteroscopic repair has typically involved the use of electrical energy to desiccate or cauterize abnormal tissue and eliminate the outpouching in which blood and fluid accumulate. Hysteroscopic resection is another technique that has also been championed.

However, for patients who desire future pregnancy, we do not recommend a hysteroscopic approach because it does not reinforce the often-thinning myometrium covering the defect. We are concerned that if this area is simply desiccated or resected, and not reapproximated, the patient will be at greater risk of pregnancy-related complications, including cesarean scar ectopic pregnancy with potential uterine dehiscence.

Laparoscopic repair was first described by Dr. Olivier Donnez, who rightly pointed out that the laparoscopic approach offers an optimal view from above during dissection of the vesico-vaginal space. Dr. Donnez used a CO₂ laser to excise fibrotic tissue, followed by laparoscopic closure (Fertil. Steril. 2008;89:974-80).

We have had success with a laparoscopic approach that uses concomitant hysteroscopy. The vesico-uterine peritoneum is incised over the anterior uterine wall, and the bladder is backfilled so that its boundaries may be identified prior to further dissection. With the area exposed, we perform a hysteroscopy to determine the exact location of the isthmocele. As the hysteroscope enters the thinned out isthmocele, the light will be more visible via laparoscopic visualization.

When performing conventional laparoscopy, the isthmocele is excised with an ultrasonic curved blade. We use this instrument because it has no opposing arm and because it enables precise tissue dissection in multiple planes. With harmonic energy, we can limit tissue dessication and destruction, lowering the risk of future pregnancy-related complications. Monopolar scissors are best when a robotic approach is used.

Once the isthmocele is resected, the clean edges are sutured together in two layers. The first layer is sutured in an interrupted mattress-style fashion, to prevent tissue strangulation and necrosis. We use a monofilament nonbarbed delayed-absorbable 3-0 PDS suture on a CT-1 needle – a choice that limits tissue trauma and postoperative inflammation.

Courtesy of Dr. Kirsten Sasaki

Sutures are initially placed at each angle with one or two sutures placed between. These sutures must be placed deep to close the bottom of the defect. A second layer of suture is then placed to imbricate over the initial layer of closure. We utilize 3-0 PDS in a running or mattress style, or a running 3-0 V-Loc suture. Our patients return after 1-3 months for a postoperative image, and are instructed to wait at least 3 months after surgery before attempting conception.

In our experience, of more than 10 patients, symptoms ceased in all patients whose surgery was performed for the indication of abnormal uterine bleeding. The follow-up on our series of patients who underwent the procedure for secondary infertility is ongoing, but the preliminary results are very positive, with resolution of intrauterine fluid in all of the patients, as well as several successful pregnancy outcomes.

A recent systematic review of minimally invasive therapy for symptoms related to an isthmocele shows good outcomes across the 12 included studies but does not offer evidence to favor one treatment over another. The studies show significant reductions in abnormal uterine bleeding and pain, as well as a high rate of satisfaction in most patients after hysteroscopic niche resection or vaginal or laparoscopic niche repair, with a low complication rate (BJOG 2014;121:145-6).

Pregnancies were reported after treatment, but sample sizes and follow-up were insufficient to draw conclusions on pregnancy and delivery outcomes, according to the review. As the reviewers wrote, following patients through their next delivery in larger, higher-quality studies will help provide more guidance for selecting the best isthmocele treatments and implementing these treatments into practice.

Dr. Sasaki reported having no financial disclosures relevant to this Master Class.