Reconstructing the anal sphincters to reverse iatrogenic overstretching following a pull-through for Hirschsprung disease. One-year outcomes.

BACKGROUND: In patients with Hirschsprung disease (HSCR), soiling may be related to anal sphincter damage following the initial pull-through. No optimal treatment has been developed for such patients, although enemas (rectal or antegrade) have been applied with some success. We present the one-year outcomes of a new technique for anal sphincter reconstruction. METHODS: All patients with HSCR referred from other institutions for post pull-through soiling were studied. Seven patients with patulous sphincters underwent sphincter reconstruction. Six had a full preoperative evaluation and were included in the study. Their 12-month outcomes were assessed. RESULTS: All six patients had soiling without voluntary bowel movements (VBMs). One patient was clean on Malone flushes when referred. Three underwent pre- and post-reconstruction non-sedated three-dimensional anorectal manometry, and objectively were able to close their sphincters following the reconstruction. All patients without Down syndrome (4 of 6) showed improvement in the abbreviated Baylor Continence Scale (4.5 vs. 0.75). One patient has achieved total bowel control without antegrade flushes, three now have VBMs which they did not have before but have occasional accidents and use antegrade flushes intermittently. They reported higher productivity, the ability to participate in sports and be away from home with confidence in their regimen. Two of 6 patients have Down syndrome and required a redo pull-through for other indications and underwent empiric sphincter reconstruction. For these two patients we do not have an outcomes assessment. CONCLUSIONS: A new technique for sphincter reconstruction shows promising results in improvement of bowel control at one year. LEVEL OF EVIDENCE: IV.

The Use of an Inanimate Simulation Model for the Correction of an Anorectal Malformation in the Training of Colorectal Pediatric Surgery.

INTRODUCTION: An anorectal malformation (ARM) is a congenital malformation that requires surgical correction. To acquire the skills needed to perform this complex procedure, an affordable simulation model has previously been developed and validated. The aim of this study is to evaluate the suitability of this ARM model (with perineal fistula) for training in hands-on workshops. MATERIALS AND METHODS: The ARM model consists of a wooden casing with disposable perineal body. Participants in several international pediatric colorectal hands-on workshops in 2019 and 2020 were asked to participate. They were divided in a target group and an experienced group based on experience. All practiced the posterior sagittal anorectoplasty procedure on the model with multimodality guidance. Subsequently, statements on the suitability of the model for use during hands-on workshops were scored on a 5-point Likert scale. RESULTS: A total of 80 participants were included (43 surgical specialists, 13 pediatric surgery fellows, and 25 residents). Nearly, all statements scored at least a mean of >4.0, all scored significantly better than a neutral opinion. The target group (n = 58) scored higher compared with the experienced group (n = 22) on "transferability of the skills to the clinical setting" (means 4.4 vs. 4.0, p = 0.038); however, the "suitability as a replacement for an animal model" scored significantly lower (means 3.6 vs. 3.9, p = 0.049). No other differences were found. CONCLUSION: This affordable ARM model was regarded a suitable model for training during preclinical hands-on workshops and could be used for the specified steps of the procedure.

Validation of a Newly Developed Competency Assessment Tool for the Posterior Sagittal Anorectoplasty.

INTRODUCTION: The correction of an anorectal malformation (ARM) is complex and relatively infrequent. Simulation training and subsequent assessment may result in better clinical outcomes. Assessment can be done using a competency assessment tool (CAT). This study aims to develop and validate a CAT for the posterior sagittal anorectoplasty (PSARP) on a simulation model. MATERIALS AND METHODS: The CAT-PSARP was developed after consultation with experts in the field. The PSARP was divided into five steps, while tissue and instrument handling were scored separately. Participants of pediatric colorectal hands-on courses in 2019 and 2020 were asked to participate. They performed one PSARP procedure on an ARM simulation model, while being assessed by two objective observers using the CAT-PSARP. RESULTS: A total of 82 participants were enrolled. A fair interobserver agreement was found for general skills (intraclass correlation coefficient [ICC] = 0.524, p < 0.001), a good agreement for specific skills (ICC = 0.646, p < 0.001), and overall performance (ICC = 0.669, p < 0.001). The experienced group scored higher on all steps (p < 0.001), except for "anoplasty (p = 0.540)," compared with an inexperienced group. CONCLUSION: The CAT-PSARP is a suitable objective assessment tool for the overall performance of the included steps of the PSARP for repair of an ARM on a simulation model.

A technique to reconstruct the anal sphincters following iatrogenic stretching related to a pull-through for Hirschsprung disease.

Soiling and fecal incontinence are troubling complications which can occur after a pull-through for Hirschsprung disease. They can usually be improved with proper medical management, but in some cases are the result of an anatomic defect related to overstretching of the sphincters and/or damage of the anal canal. For such patients the treatment of this true fecal incontinence is limited to a structured bowel management program with ante- or retrograde enemas to achieve social continence. Herein we report two such patients with overstretched sphincters and loss of the dentate line after an initial pull-through and describe a sphincter tightening technique to improve bowel control.

Factors predicting the need for vaginal replacement at the time of primary reconstruction of a cloacal malformation.

BACKGROUND: A subset of patients with cloacal malformations requires vaginal replacement during their primary reconstruction, increasing the surgical complexity. Identifying factors which predict the need for vaginal replacement would facilitate operative planning. METHODS: We retrospectively reviewed patients who underwent primary cloacal reconstruction at our Center (2014-2018) and assessed the length of the common channel, urethra, and vagina. The presence of hydrocolpos at birth, Müllerian anomalies, sacral ratio, and tethered cord were also assessed between patients who did and did not require vaginal replacement. RESULTS: 50 patients were identified. 17/50 patients (34%) underwent a total urogenital mobilization (TUM), and none required vaginal replacement. 33/50 (66%) patients underwent a urogenital separation. 19/33 (58%) required vaginal replacement. This group had a shorter vagina (4.2 cm vs 6.6 cm, p < 0.01). There was no difference in urethral or common channel length, number of cervices, sacral ratio, presence of a vaginal septum, hydrocolpos, or tethered cord between those who did and those who did not require vaginal replacement. CONCLUSIONS: Urethral and common channel lengths were used to successfully determine the operative plan (TUM or urogenital separation) to reconstruct cloacal malformations. The need for urogenital separation and a shorter vaginal length were predictive of the need for vaginal replacement. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Case series with no comparison groups.

Diagnosis and management of a remnant of the original fistula (ROOF) in males following surgery for anorectal malformations.

PURPOSE: A complication of the surgical management of anorectal malformations (ARMs) is a retained remnant of the original fistula (ROOF) formerly called a posterior urethral diverticulum (PUD). A ROOF may have multiple presentations or may be incidentally discovered during the work-up of ARM after previous surgery. We sought to define the entity and the surgical indications for excision of a ROOF when found. METHODS: We performed a retrospective cohort study of all male patients who presented to our center following previous repair for ARM at another institution, who came for evaluation of problems with urinary and/or fecal continence, from 2014 to 2017. Charts were reviewed for symptoms, original type of malformation, preoperative imaging, treatment, and postoperative follow-up. RESULTS: Of 180 referred male patients, 16 had a ROOF. 14 underwent surgical repair to address this and for other redo indications, and 2 did not require intervention. 13 patients had an additional reason for a redo such as anal mislocation or rectal prolapse. Indications for ROOF excision were urinary symptoms (e.g. UTI, dribbling, passage of mucous via urethra, stone formation), to make a smoother posterior urethra for intermittent catheterization, or for prophylactic reasons. Patients were repaired at an average age of 4.2 years, using a PSARP only approach with excision of the ROOF for all except one patient who needed a laparotomy due to abdominal extension of the ROOF. No patient needed a colostomy. The original ARM repairs of the patients were PSARP (9), laparoscopic assisted (4) and abdominoperineal pullthrough (3). Preoperative evaluation included pelvic MRI, VCUG, and cystoscopy. The ROOF was visualized on 14 of 16 MRIs, 10 of 14 VCUGs, and 14 of 15 cystoscopies. Urinary symptoms associated with a ROOF and ease of catheterization were improved in all repaired cases. CONCLUSION: Patients not doing well from a urinary or bowel standpoint post ARM pull-through need a complete evaluation which should include a check for a ROOF. Both modalities MRI and cystoscopy are needed as a ROOF can be missed on either alone. A VCUG was not reliable in identifying a ROOF. Excision is needed in patients to improve urinary symptoms associated with these lesions and to minimize the small but theoretical oncologic risk present in a ROOF. LEVEL OF EVIDENCE: Level III.

Presacral masses and sacrococcygeal teratomas in patients with and without anorectal malformations: A single institution comparative study.

BACKGROUND: Despite variability at presentation, presacral masses in patients with and without anorectal malformations (ARM) appear histologically similar. The purpose of this study was to identify differences in oncologic outcomes between these two groups. METHODS: A retrospective review was performed utilizing our institutional cancer and colorectal and pelvic reconstruction databases for patients with presacral masses and sacrococcygeal teratomas between 1990 and 2017. Data captured included age at surgical resection, type of ARM, tumor location within the pelvis, tumor histopathology, tumor size, adjuvant chemotherapy, recurrence, and follow-up. RESULTS: Forty-six patients comprised our cohort, of whom 12 had an ARM. The median age was older at resection for those with an ARM (1.4 years; range 1 day to 29.4 years) compared to those without an ARM (9 days; range 0 days to 6.9 years) (p = 0.01). The mean tumor size was 2.5 cm in patients with an ARM compared to 6.0 cm in patients without an ARM (p = 0.036). All patients with ARM had exclusively intrapelvic tumors, and histopathology included mature teratoma (8), yolk sac tumor (1), lipoma (1), and unknown (2). Tumor location for patients with sacral and presacral masses without ARM included exclusively extrapelvic (10), primarily extrapelvic with large intrapelvic component (7), primarily intrapelvic with extrapelvic component (1), exclusively intrapelvic (8), and unknown (8). Histopathology for patients with presacral masses without ARM included mature teratoma (20), immature teratoma (7), yolk sac tumor (3), ganglioneuroma (1), neuroblastoma (1), benign epithelial cyst (1), and unknown (1). Tumor recurrence rate was similar between patients with ARM (n = 3, 25%) and those without an ARM (n = 5, 15%) (p = 0.41). The 5-year event free survival was 65% (95% CI: 25%-87%) in the group with ARM and 81% (95% CI: 60%-92%) in the group without ARM (p = 0.44). CONCLUSION: Sacral and presacral masses in patients with ARM are resected at a later age and are more likely to be intrapelvic. They appear histologically similar and have similar rates of recurrence and malignancy when compared to patients without ARM. LEVEL OF EVIDENCE: III TYPE OF STUDY: Retrospective comparative study.

The Mullerian Black Box: Predicting and defining Mullerian anatomy in patients with cloacal abnormalities and the need for longitudinal assessment.

INTRODUCTION: Most patients with a cloacal malformation have a Mullerian anomaly. We sought to examine our patients with a cloacal malformation to determine the proportion of them we felt we knew their Mullerian anatomy and which proportion we felt would most benefit from longitudinal assessment to define their anatomy, reproductive potential, and risk of outflow tract obstruction after puberty. We also compared the preoperative assessment of reproductive anatomy (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) and intra-operative abdominal findings (at the time of primary cloacal reconstruction or subsequent abdominal procedures) to see how these correlated with and which preoperative assessment tool was most predictive of intraoperative anatomy. We also sought to confirm what we expected to be a lack of ovarian pathology. METHODS: A single site retrospective chart review was performed on all patients with a cloacal anomaly seen between May 2014 and September 2017. Preoperative assessment (pelvic ultrasound, MRI, cloaca gram, and vaginoscopy) and operative reports (both primary reconstruction and later abdominal procedures) were reviewed to ascertain Mullerian and ovarian anatomy. RESULTS: 30 of 36 (83%) of patients had defined Mullerian anatomy after preoperative assessment (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) with or without the addition intraoperative assessment of Mullerian structures obtained during laparoscopy or laparotomy. 19/30 (63%) had duplication of their Mullerian structures. 25/36 (69%) had intraoperative assessment of Mullerian anatomy during laparoscopy or laparotomy. In this group, preoperative assessment with pelvic ultrasound correlated in 4/8 patients (50%), MRI correlated in 3/4 patients (75%), cloacagram in 10/15 patients (67%), and vaginoscopy in 23/25 patients (92%). 14/36 (39%) patients were found to require longitudinal assessment to define anatomy, reproductive potential or risk of outflow tract obstruction after puberty. Patients with ovarian findings described at the time of laparoscopy or laparotomy had no evidence of ovarian pathology. CONCLUSIONS: The majority of patients with cloaca in our series (83%) had their Mullerian anatomy defined by either preoperative assessment and/or findings at the time of laparoscopy or laparotomy. Duplication of the vagina and uterus was the most commonly described Mullerian anatomy (63%) in our series. Vaginoscopy appears to be superior to pelvic ultrasound, MRI, and cloacagram in predicting Mullerian anatomy. Fourteen of our 36 (39%) patients will require longitudinal assessment follow for reproductive potential and/or risk of outflow tract obstruction after puberty as their Mullerian anatomy is not known. There was no evidence of ovarian pathology in any cloaca patient. While we felt as though we could define Mullerian anatomy in most of our patients, any opportunity for intraoperative assessment of Mullerian anatomy should be utilized and therefore teams who are involved in the management of these patients must have a systematic and collaborative method established to ensure that Mullerian structures are thoroughly evaluated intra-operatively and documented in a standardized fashion. TYPE OF STUDY: Retrospective Chart review. LEVEL OF EVIDENCE: III.

Can sacral development as a marker for caudal regression help identify associated urologic anomalies in patients with anorectal malformation?

BACKGROUND AND AIM: The sacral ratio (SR) is a well-established tool to quantify sacral development in patients with anorectal malformations (ARM) and can be used as a predictor of fecal continence. We hypothesized that a lower SR correlated with the presence of urologic and renal malformations. METHODS: We retrospectively reviewed the medical records of patients with ARM treated at our center from 2014 to 2016. We measured the lateral SR as a marker for sacral development and assessed the spine for the presence of tethered cord (TC). Urological and renal anomalies, including single kidney, hydronephrosis, hypospadias, vesicoureteral reflux (VUR), ectopic ureter, and penoscrotal transposition were assessed. Analysis of variance (ANOVA), t-tests, and multivariable linear regression were used to test for differences in SR with consideration of associated urologic malformations and tethered cord. RESULTS: 283 patients with ARM were included for analysis (156 females). The median age was 39months (10-90). Among these, 178 (55.6%) had 1 or more urologic malformations, and 81 (25.3%) had a TC. Hydronephrosis, high-grade VUR (3-5), solitary kidney, and tethered cord were significantly associated with lower SR (p<0.01). In multivariable regression models, the presence of urologic abnormalities remained significantly associated with lower a SR despite the presence or absence of TC (p<0.001). CONCLUSION: SR is a potentially useful indicator of certain urologic anomalies including hydronephrosis, high grade VUR, and solitary kidney in patients with ARM. This association is independent of the presence of TC. A sacral ratio as a part of the VACTERL screening can help the surgeon identify which patients need closer urologic follow up. LEVEL OF EVIDENCE: IV.

Are Senna based laxatives safe when used as long term treatment for constipation in children?

BACKGROUND AND AIM: Senna is a stimulant laxative commonly used by pediatricians, pediatric gastroenterologists, and pediatric surgeons. Many clinicians avoid Senna for reasons such as tolerance or side effects but this has little scientific justification. We recently found several patients we were caring for developed perineal blistering during the course of Senna treatment. Because of this we chose to review the literature to identify side effects in children taking this medication as well as to analyze our Center's experience with Senna's secondary effects. METHODS: We performed a literature review (MEDLINE, PUBMED) using the keywords of Senna, sen, sennosides and children, and pediatric and functional (idiopathic) constipation. We looked for articles with information regarding perineal blisters related to Senna as well as other secondary effects of Senna laxatives in children when used on a long-term basis. We also reviewed the charts of our patients who had previously taken Senna or are currently taking Senna, looking for adverse reactions. RESULTS: Eight articles in the literature reported perineal blisters after administration of Senna laxatives in 28 patients. Of those occurrences, 18 patients (64%) had accidental administration of Senna and 10 (36%) had Senna prescribed as a long term treatment. All of the blistering episodes were related to high dose, night-time accidents, or intense diarrhea with a long period of stool to skin contact. At our institution, from 2014 to 2017, we prescribed Senna and have recorded data to 640 patients. During the study period, 17 patients (2.2%) developed blisters during their treatment. Patients who developed blisters had higher doses 60mg/day; 60 [12-100] vs. 17.5 [1.7-150] (p<0.001). All of the blistering episodes were related to night-time accidents, with a long period of stool to skin contact. 83 (13%) patients presented minor side effects such as abdominal cramping, vomiting or diarrhea which resolved once the type of laxatives were changed or enemas were started. The doses of Senna was not significantly different in these patients 15mg/day [4.4-150] vs. 17.5mg/day [1.5-150]. There were no other long-term side effects from Senna found in the pediatric literature for long-term treatment besides abdominal cramping or diarrhea during the first weeks of administration. We found no evidence of tolerance to Senna in our review. CONCLUSION: There is a paucity of information in the literature regarding side effects of sennosides as a long-term therapy, and to our knowledge, this is the first review of Senna side effects in children. Senna induced dermatitis is rare, but may occur when patients need a higher dose. All of the cases described had a long period of exposure of the skin to stool. Besides the perineal rash with blisters, we could find no other described major side effect with Senna administration in the pediatric population or evidence of the frequently mentioned concern of the development of tolerance to Senna. Pediatric caregivers should advise families of the rare side effect of skin blistering and educate them to change the diaper frequently in children who are not toilet- trained to reduce stool to skin exposure. We can conclude from this review that Senna is a safe treatment option for constipation in children. LEVEL OF EVIDENCE: IV.

Gynecologic anatomic abnormalities following anorectal malformations repair.

BACKGROUND/AIM: Patients may present with gynecologic concerns after previous posterior sagittal anorectoplasty (PSARP) for repair of an anorectal malformation (ARM). Common findings include an inadequate or shortened perineal body, as well as introital stenosis, retained vaginal septum, and remnant rectovestibular fistula. An inadequate or shortened perineal body may impact fecal continence, sexual function and recommendations regarding obstetrical mode of delivery. We describe our experience with female patients referred to our center for evaluation of their previously repaired ARM, with a specific focus on perineal body anatomy and concomitant gynecologic abnormalities. We outline our collaborative evaluation process and findings as well as subsequent repair and outcomes. MATERIAL/METHODS: A single site retrospective chart review from May 2014 to May 2016 was performed. Female patients with a history of prior ARM repair who required subsequent reoperative surgical repair with perineoplasty were included. The decision for reoperation was made collaboratively after a multidisciplinary evaluation by colorectal surgery, urology, and gynecology which included examination under anesthesia (EUA) with cystoscopy, vaginoscopy, rectal examination, and electrical stimulation of anal sphincters. The type of original malformation, indication for reoperative perineoplasty, findings leading to additional procedures performed at time of perineoplasty, postoperative complications, and the length of follow up were recorded. RESULTS: During the study period 28 patients were referred for evaluation after primary ARM repair elsewhere and 15 patients (60%) met inclusion criteria. Thirteen patients (86.6%) originally had a rectovestibular fistula with prior PSARP and 2 patients (13.4%) originally had a cloacal malformation with prior posterior sagittal anorectovaginourethroplasty. The mean age at the time of the subsequent perineoplasty was 4.6years (0.5-12). Patients had an inadequate perineal body requiring reoperative perineoplasty due to: anterior mislocation of the anus (n=11, 73.3%), prior perineal wound dehiscence with perineal body breakdown (n=2, 13.4%), acquired rectovaginal fistula (n=1, 6.6%), and posterior mislocated introitus with invasion of the perineal body (n=1, 6.6%). During the preoperative evaluation, additional gynecologic abnormalities were identified that required concomitant surgical intervention including: introital stenosis (n=4, 26.6%), retained vaginal septum (n=3, 20%) and remnant recto vestibular fistula (n=2, 13.3%). CONCLUSIONS: Patients with a previously repaired ARM may present with gynecologic concerns that require subsequent surgical intervention. The most common finding was an inadequate perineal body, but other findings included introital stenosis, retained vaginal septum and remnant recto vestibular fistula. Multidisciplinary evaluation to assess and identify abnormalities and coordinate timing and surgical approach is crucial to assure optimal patient outcomes. TYPE OF STUDY: Case series with no comparison group. LEVEL OF EVIDENCE: IV.

Does clinic visit education within a multidisciplinary center improve health literacy scores in caregivers of children with complex colorectal conditions?

INTRODUCTION: Health literacy is low in an estimated one-third of the US population. Little is known about the health literacy of caregivers of children with colorectal conditions. The objective of this study was to investigate whether a timed health literacy intervention could improve health literacy in this population. METHODS: We used the BRIEF Health Literacy screening (BHLS) tool on caregivers of children who came to our colorectal clinic. Health literacy was categorized as inadequate, marginal, or adequate. The number of caregivers with adequate health literacy was compared to the number of clinic visits and socioeconomic status. RESULTS: We included 233 caregivers. The average number of clinic visits was 3.5 over 1.2years. At the first clinic visit, 70% (n=98) of caregivers had "adequate" health literacy. Scores improved to 88% (p=0.024) after the fourth visit. Socioeconomic factors were not associated with health literacy. Patients of caregivers with "adequate" health literacy visited our clinic 3.8 times, compared to 2.7 times for those with lower literacy (p=0.006). CONCLUSION: Emphasis on providing an education-based approach at each visit increased health literacy significantly. As expected, health literacy was lowest during the first visit, which we believe is the optimal time to implement educational interventions. TYPE OF STUDY: Case Control/Retrospective Comparative Study. LEVEL OF EVIDENCE: Level III.

Use of a Heineke-Mikulicz like stricturoplasty for intractable skin level anal strictures following anoplasty in children with anorectal malformations.

INTRODUCTION: We introduced a modification of the Heineke-Mikulicz technique to treat intractable skin level anal strictures post posterior sagittal anorectoplasty (PSARP). The aim of this article is to describe the technique and outcome in a series of patients. METHODS: This was a retrospective evaluation of patients who had Heineke-Mikulicz like stricturoplasty performed for a post PSARP skin level stricture over a one-year period. RESULTS: Five patients who were operated using the technique were reviewed. All had severe anal strictures that could admit Hegar dilator sizes 6 to 9 at 16months to 5years after PSARP. All underwent routine dilatations, which became increasingly painful. As an alternative to continued dilatations, an operative procedure was offered. The surgery was done as a day case and lasted 10 to 30min. The anus at the end of the procedure could comfortably accept a Hegar dilator size 14 to 17. None of the patients had a colostomy after the procedure and there were no complications. CONCLUSIONS: The Heineke-Mikulicz like stricturoplasty is a simple surgical procedure that can be done in an ambulatory setting to treat children with intractable skin level anal stricture if this develops following definitive surgery for anorectal malformations.

Decision making among different treatment options for neurologically impaired boys with undescended testis: a multinational pediatric survey.

OBJECTIVE: To determine the attitude of referring pediatricians towards the decision of treatment modalities for undescended testis (UDT) in neurologic impaired boys (NIB). METHODS AND MATERIALS: An online questionnaire was offered to registered pediatricians in Austria and Germany for online completion. RESULTS: 221 male (61.6%) and 138 female (38.4%) pediatricians completed the survey; 326 (90.8%) believe that UDT should be treated according to national guidelines; 31 (8.6%) believe that UDT should be treated according to the parental wish, whereas only 2 (0.6%) tend to no treatment at all. Tumor prophylaxis, further sexual life, legal concerns, risks of anesthesia, and the choice of the parents have major impact on the perception of UDT. Moreover, fertility and limited life expectancy seem to be of minor importance only. In general, Pearson χ2 test could not identify age and sex of pediatricians as significant predictor of how the importance of the treatment of UDT is appraised. CONCLUSION: From the pediatric point of view UDT in NIB is an important issue and should be treated according to guidelines. Nevertheless, this study indicates the problems in decision-making and choosing the best management for UDT in NIB. Undoubtedly, further ethical discussion is needed to optimize treatment of UDT in NIB.

Appendix vermiformis as a left pyelo-ureteral substitute in a 6-month-old girl with solitary kidney.

Extensive ureteral loss in early childhood is a rare but dramatic event. We present the case of a 6-month-old girl with a iatrogenic extensive pyelo-ureteral loss and solitary kidney. She successfully underwent left ureteral substitution using the appendix vermiformis. Left ureteral reconstruction using the appendix vermiformis is a feasible procedure even in very small children.

Online hypospadias support group data analysis.

BACKGROUND: Although hypospadias is the most common malformation of the penis, this condition is virtually unknown to the general population. This lack of information may lead to psychological distress to both parents and patients. In this study the content of an Internet-based hypospadias support group is analyzed. MATERIALS AND METHODS: All comments and postings made by members of an online support group between 2001 and 2008 were analyzed. No personalized data were used for evaluation. RESULTS: At the end of 2008, the group consisted of 535 members. In the study period, 3050 postings were published. Major issues discussed in the group were (a) medical questions, exchange of medical knowledge, experience, and history; (b) psychological, ethical, and philosophical aspects; (c) practical questions and recommendations; (d) recommendations for surgeons; and (e) hypospadias in adults. CONCLUSIONS: Online hypospadias support groups play an important role in how parents and patients cope with the condition. Surgeons dealing with hypospadias should be aware of the importance of Internet-based information in the field of hypospadias. They may benefit from the valuable feedback information from patients and parents to improve their practice and outcome.

Major complication after intrauterine vesico-amniotic shunting.

Bilateral foetal uropathy is the leading cause of chronic renal failure in childhood. Vesico-amniotic shunting (VAS) is a simple, feasible, and widely used procedure for decompressing the foetal urinary system. We report a case of a boy with bilateral foetal uropathy who underwent VAS at a gestational age of 29 weeks. Vesico-abdominal shunt dislodgement occurred and led to urinary ascites and anhydramnios. Postpartal laparotomy showed a shunt perforation between the urinary bladder and the peritoneal cavity.

Neuroendocrine tumor of the common hepatic duct, mimicking a choledochal cyst in a 6-year-old child.

We report a rare case of a neuroendocrine tumor of the extrahepatic biliary tree in a child. A 6-year-old girl who presented with jaundice and pruritus was found to have elevated liver enzymes along with a cystic dilatation of the common hepatic duct. After further diagnostic testing, a working diagnosis of a type I choledochal cyst was established. Laparotomy revealed hydrops of the gall bladder, an elongated and dilated cystic duct and a cystic dilatation of the common hepatic duct. An unusually thickened common bile duct was also noted at the time. Follow-up histology revealed an invasive neuroendocrine tumor which lead to a second look operation in which extended resection and lymphadenectomy was performed. No histological or gross pathological evidence of lymph node metastasis was found. Postoperatively the patient continues doing well 2 years after the original diagnosis was established. Neuroendocrine tumors of the extrahepatic bile duct are extremely rare with only 4 pediatric cases of a total of 51 cases published in the literature. To our knowledge, this is the youngest patient reported so far. The rarity of this entity made it challenging to diagnose a case of a neuroendocrine tumor in an atypical location that radiologically mimicked a choledochal cyst.

Submucosal hematoma presenting as small bowel obturator obstruction in a patient on low-molecular-weight heparin.

Recent studies have shown the efficacy of low-molecular-weight heparin (LMWH) in the treatment of venous thromboembolic disease in children. Compared to unfractionated heparin and coumadin, LMWH has more predictable pharmacokinetics and a reported lower incidence of osteoporosis and heparin-induced thrombocytopenia in children. The overall incidence of severe hemorrhage on LMWH in children is low. To date, there is a single report of a small bowel obstruction in a child secondary to a hematoma while on LMWH. We report the second case of a child, on enoxaparin (Lovenox) therapy, who underwent bowel resection secondary to a completely obstructing small bowel wall hematoma.