Emily Louden

Hydrocolpos in cloacal malformations

INTRODUCTION Hydrocolpos is a condition rarely mentioned in the literature. The purpose of this report is to increase the index of suspicion for hydrocolpos in patients with cloaca and to describe our approach for its treatment with the hope that errors in the management of hydrocolpos can be avoided. METHODS We reviewed 411 medical records of patients diagnosed with cloaca and managed at our Center during the last 26 years. Emphasis was placed on evaluating for the presence of hydrocolpos, type of drainage, and complications related to the persistence of the hydrocolpos. RESULTS One hundred seventeen cloaca patients had an associated hydrocolpos (28.4%). Forty-two cases (36%) were initially managed at other institutions at which the hydrocolpos was not drained. Complications experienced by this group included: multiple urinary tract infections (8), hydrocolpos infection (7), sepsis (7), failure to thrive (6), ruptured hydrocolpos (4), and development of hydronephrosis in previously normal kidneys (2). Forty-one patients (35%) had other modalities of treatment, aimed to drain the hydrocolpos, including vesicostomy (26), intermittent perineal catheterization (8), single aspiration (6), or plasty of the perineal orifice (1). In all of these cases, the hydrocolpos persisted or reaccumulated. Thirty-four patients (29%) underwent an effective drainage of the hydrocolpos at birth; 29 at other institutions, 15 with a tube vaginostomy, 13 with a tubeless vaginostomy, and 1 with a catheter placed and left in the vagina through cystoscopy. Five cases had a tube vaginostomy done by us. In all these cases, the vagina remained adequately drained as demonstrated radiologically. Proper drainage of the hydrocolpos alone, with no urologic intervention, dramatically improved the hydronephrosis in 13 cases. CONCLUSIONS Hydrocolpos in patients with cloacas must be diagnosed and treated early in life. Our preferred approach is a transabdominal indwelling vaginostomy tube. The drainage of the hydrocolpos alone may dramatically improve the hydronephrosis, and therefore, we suggest that only after the hydrocolpos is drained should a urological intervention be contemplated. Failure to drain the hydrocolpos can result in serious complications.

The appendix as a conduit for antegrade continence enemas in patients with anorectal malformations: lessons learned from 163 cases treated over 18 years

INTRODUCTION The antegrade continence enema (ACE) has been shown to be a safe and effective method for managing fecal incontinence in the pediatric population. The purpose of this study was to examine our experience with the ACE procedure using the appendix as a catheterizable conduit in children with anorectal malformations (ARMs). METHODS We reviewed the charts of all patients who underwent an ACE procedure using the appendix as a catheterizable conduit between January 1992 and January 2010. Preoperative diagnosis (ARM type), operative details, functional outcomes, and postoperative complications were assessed. Technical modifications over time included selective cecoplication, implementation of the umbilical V-V appendicoplasty technique, and laparoscopy for cecal mobilization. RESULTS Mean age was 9.9 ± 0.6 years, and 67% were male. The most common preoperative diagnosis was rectourethral fistula in boys (39%) and persistent cloaca in girls (61%). Forty-five complications occurred in 41 patients with an overall incidence of 25.6% (stricture, 18%; leakage, 6%; prolapse, 4%; intestinal obstruction, 0.6%). The incidence of stomal leakage was lower in patients when a cecoplication was performed (2.9% [4/138] vs 29.4% [5/17]; P < .01), and the incidence of stricture was lower in patients when the umbilical anastomosis was created using the V-V appendicoplasty technique (11% [11/100] vs 30% [18/60]; P < .01). Successful management of incontinence was reported by 96% of all patients. CONCLUSIONS The ACE procedure using the umbilical V-V appendicoplasty provides an effective and cosmetically superior means for bowel management in children with ARMs. The rate of late complications is not insignificant however, and preventative strategies should focus on careful operative technique and ensuring compliance with catheterization protocols well past the initial postoperative period.

Reoperation for Hirschsprung Disease: Pathology of the Resected Problematic Distal Pull-Through

Hirschsprung disease, which consists of aganglionosis of the rectum and sometimes more proximal bowel, requires surgical removal of the aganglionic bowel and creation of ganglionated neorectum using proximal normally innervated bowel. The border between aganglionic and ganglionic bowel is irregular; the transition zone features variable quantities of ganglion cells and numerous large nerves. We report the histopathology of pull-through bowel segments resected because of poor postoperative outcome from 30 patients (22 boys, 8 girls). The most common indication for reoperation was severe constipation/obstruction. Transition zone (bowel with at least two nerves ≥40 μm diameter per 400× high-power field, and ganglion cells) or aganglionic bowel (bowel with at least two nerves ≥40 μm per high-power field diameter, but without ganglion cells) was found in 19/30 (63%) resections. In colons resected because of familial adenomatous polyposis, rare high-power fields showed two enlarged nerves; the mean age of those patients (135 ± 49.4 months) was significantly higher than that of the patients undergoing redo pull-through surgery (67.9 ± 42.8 months). Additional pathology included stricture and enterocolitis. Although there are multiple causes for poor outcomes following surgical therapy for Hirschsprung disease, abnormal innervation of the bowel used for pull-through is common. We recommend that intraoperative consultation at primary pull-through procedure include frozen section evaluation of the circumference of the bowel to be used for pull-through to confirm histologically the presence of both ganglion cells and normal-caliber nerves. The criteria used in this study are most suitable for infants and young children.

Posterior cloaca—further experience and guidelines for the treatment of an unusual anorectal malformation

INTRODUCTION The term posterior cloaca refers to a malformation in which the urethra and vagina are fused, forming a urogenital sinus that deviates posteriorly to open in the anterior rectal wall or immediately anterior to the anus. METHODS A retrospective review of 411 patients diagnosed with cloaca was performed to identify the ones with a posterior cloaca. Special emphasis was placed on anatomy, diagnosis, associated anomalies, and outcome in terms of urinary and fecal continence. Surgical treatment was a total urogenital mobilization with a transrectal approach. RESULTS Twenty-nine patients were diagnosed with a posterior cloaca. Of these, 15 had a single orifice at the normal location of the anus with the urogenital sinus opening in the anterior rectal wall. Fourteen had the urogenital sinus opening immediately anterior to the normally located anal opening (2 orifices), which we considered a posterior cloaca variant. Nineteen patients (65%) had hydrocolpos. Twenty-seven patients (93%) had associated urologic anomalies, 12 patients (41%) had gynecologic anomalies, and vertebral malformations occurred in 41% of cases. Other anomalies included gastrointestinal (7 patients), cardiac (5), and tethered cord (2). Late diagnosis occurred in 2 patients. Twenty patients were available for long-term follow-up: 17 are fecally continent, 3 are fecally incontinent, 11 are urinary continent, 5 are dry with intermittent catheterization, and 4 have dribble urine. CONCLUSION The most important characteristic of the posterior cloaca is the high frequency of a normal anus, which differentiates this malformation from the classic cloaca. Often, many associated malformations are present and therefore should be suspected and diagnosed. The main goal during the operation should be to not mobilize the anus and thereby preserve the anal canal. A total urogenital mobilization, transperineally or with a transanorectal approach, is ideal for the repair.

Rectal atresia and stenosis: unique anorectal malformations.

INTRODUCTION Rectal atresia/stenosis is a rare disorder in the spectrum of anorectal malformations and is particularly associated with a presacral mass. These patients are born with a normal anal canal but have a stricture or complete atresia located a few centimeters proximal to the dentate line. We present a surgical technique for the management of these patients, as well as their unique clinical concerns and outcomes. METHODS We reviewed the records of 14 patients with rectal atresia and 3 with rectal stenosis. We describe a novel technique that we have developed for the preservation of the anterior dentate line that was performed in the last 13 patients. RESULTS Rectal atresia/stenosis was associated with a presacral mass in 5 patients (29%). Definitive repair was completed using a circular rectorectal anastomosis in the first 4 patients and an anterior dentate line sparing procedure in the last 13. All patients older than 3 years have demonstrated the ability to have voluntary bowel movements. CONCLUSION With the largest reported series of rectal atresia/stenosis, we have demonstrated a safe and effective technique for repair. Preoperative evaluation must be thorough because a significant number of these patients will have an associated presacral mass.

Meta-analysis of function after secondary shoulder surgery in neonatal brachial plexus palsy.

Background: Shoulder internal rotation contracture, active abduction, and external rotation deficits are common secondary problems in neonatal brachial plexus palsy (NBPP). Soft tissue shoulder operations are often utilized for treatment. The objective was to conduct a meta-analysis and systematic review analyzing the clinical outcomes of NBPP treated with a secondary soft-tissue shoulder operation. Methods: A literature search identified studies of NBPP treated with a soft-tissue shoulder operation. A meta-analysis evaluated success rates for the aggregate Mallet score (≥4 point increase), global abduction score (≥1 point increase), and external rotation score (≥1 point increase) using the Mallet scale. Subgroup analysis was performed to assess these success rates when the author chose arthroscopic release technique versus open release technique with or without tendon transfer. Results: Data from 17 studies and 405 patients were pooled for meta-analysis. The success rate for the global abduction score was significantly higher for the open technique (67.4%) relative to the arthroscopic technique (27.7%, P<0.0001). The success rates for the global abduction score were significantly different among sexes (P=0.01). The success rate for external rotation was not significantly different between the open (71.4%) and arthroscopic techniques (74.1%, P=0.86). No other variable was found to have significant impact on the external rotation outcomes. The success rate for the aggregate Mallet score was 57.9% for the open technique, a nonsignificant increase relative to the arthroscopic technique (53.5%, P=0.63). Data suggest a correlation between increasing age at the time of surgery and a decreasing likelihood of success with regards to aggregate Mallet with an odds ratio of 0.98 (P=0.04). Conclusions: Overall, the secondary soft-tissue shoulder operation is an effective treatment for improving shoulder function in NBPP in appropriately selected patients. The open technique had significantly higher success rates in improving global abduction. There were no significant differences in the success rates for improvement in the external rotation or aggregate Mallet score among these surgical techniques. Level of Evidence: Level IV—meta-analysis.

Factors Related to the Psychosocial Functioning of Youth With Neonatal Brachial Plexus Injuries

OBJECTIVE Owing to the possible visible nature and functional impairments associated with neonatal brachial plexus injuries (NBPI), the current study investigated the relations of injury severity, social support, and coping strategies to social difficulties and self-concept in youth with NBPI. METHODS 88 children (aged 10-17 years) with NBPI and their parent(s) were recruited from a national organization and two brachial plexus clinics. Participants completed a variety of questionnaires during their scheduled clinic visits. RESULTS More social support from classmates was associated with better self-concept and fewer social difficulties. Less frequent use of negative coping strategies was associated with better self-concept and fewer social difficulties and was a significant moderator of the relation between injury severity and self-concept. CONCLUSIONS Clinicians who work with children with NBPI should consider peer support and coping strategies when promoting the psychosocial functioning of these youth.

The Impact of Pediatric Brachial Plexus Injury on Families

PURPOSE To determine the impact of brachial plexus injuries on families to best meet their clinical and social needs. METHODS Our cross-sectional study included families with children between the ages of 1 and 18 years with birth or non-neonatal brachial plexus injuries (BPIs). The consenting parent or guardian completed a demographic questionnaire and the validated Impact on Family Scale during a single assessment. Total scores can range from 0 to 100, with the higher the score indicating a higher impact on the family. Factor analysis and item-total correlations were used to examine structure, individual items, and dimensions of family impact. RESULTS A total of 102 caregivers participated. Overall, families perceived various dimensions of impact on having a child with a BPI. Total family impact was 43. The 2 individual items correlating most strongly with the overall total score were from the financial dimension of the Impact on Family Scale. The strongest demographic relationship was traveling nationally for care and treatment of the BPI. Severity of injury was marginally correlated with impact on the family. Parent-child agreement about the severity of the illness was relatively high. CONCLUSIONS Caretakers of children with a BPI perceived impact on their families in the form of personal strain, family/social factors, financial stress, and mastery. A multidisciplinary clinical care team should address the various realms of impact on family throughout the course of treatment. TYPE OF STUDY/LEVEL OF EVIDENCE Prognostic II.

Risk Factors for Brachial Plexus Birth Injury

Over the course of decades, the incidence of brachial plexus birth injury (BPBI) has increased despite advances in healthcare which would seem to assist in decreasing the rate. The aim of this study is to identify previously unknown risk factors for BPBI and the risk factors with potential to guide preventative measures. A case control study of 52 mothers who had delivered a child with a BPBI injury and 132 mothers who had delivered without BPBI injury was conducted. Univariate, multivariable and logistic regressions identified risk factors and their combinations. The odds of BPBI were 2.5 times higher when oxytocin was used and 3.7 times higher when tachysystole occurred. The odds of BPBI injury are increased when tachysystole and oxytocin occur during the mother’s labor. Logistic regression identified a higher risk for BPBI when more than three of the following variables (>30 lbs gained during the pregnancy, stage 2 labor >61.5 min, mother’s age >26.4 years, tachysystole, or fetal malpresentation) were present in any combination.