Rare Associations with Posterior Urethral Valves.

Posterior urethral valves are a common cause of congenital bladder outlet obstruction. Known associations include cardiac malformations and gastrointestinal abnormalities. In this case series, we report on two cases of PUV associated with anorectal malformations along with a case of PUV in monochorionic diamniotic twins. We explore the difficulty in achieving a diagnosis and the final management. The association of posterior urethral valves in a patient with anorectal malformation should be suspected in case of associated oligohydramnios or oliguria postnatally. There should be a high index of suspicion in twin pregnancy even if only one of the twins is suspected of bladder outlet obstruction.

Save the foreskin: Outcomes of preputioplasty in the treatment of childhood phimosis.

BACKGROUND: Symptomatic phimosis is a common childhood urology complaint. Circumcision was traditionally the treatment of choice, but its popularity in cases of non-scarred phimosis has been superseded by more conservative methods like preputioplasty. We sought to examine outcomes of preputioplasty for the treatment of non-scarred pathological phimosis in two UK paediatric surgery tertiary centres. METHODS: Retrospective case series selecting cases performed in both departments over a 4 year period (January 2012-December 2015). INCLUSION CRITERIA: non-scarred pathological phimosis treated with preputioplasty. EXCLUSION CRITERIA: diffuse scarring of foreskin or presence of balanitis xerotica obliterans (BXO), preputioplasty performed as part of hypospadias repair. Outcome measure was treatment success as evidenced by fully retractile prepuce at follow up. Follow up occurred between 3 and 24 months. RESULTS: We identified 126 patients, 6 were excluded due to the above criteria. Median age was 13.4 years (range 10 months-18 years). Median follow up was 13 months (range 3-24 months). 115 patients (96%) had successful treatment as evidenced by satisfactory post-operative cosmesis and complete resolution of phimosis at follow up. Recurrence of phimosis occurred in 5 patients (4%). Mean time of recurrence was 6 months, with a median age of recurrence of 15.3 years (range 10.7-16.7 years). All patients with recurrence were successfully treated with circumcision. CONCLUSION: Foreskin conserving methods like preputioplasty are a valid option in the treatment of non-scarred pathological phimosis.

Double trouble: A rare case of posterior urethral valve and Cobb's collar.

Posterior urethral valves (PUV) are the most common cause of bladder outlet obstruction (BOO) in boys. Cobb's collar, a rare narrowing of the bulbar urethra, is one of the lesser-known causes of congenital urethral obstruction. We present a case of both congenital anomalies occurring concomitantly, in a preterm baby presenting with urinary ascites.

13 ribs as a predictor of long gap esophageal atresia: myth or reality? Analysis of associated findings of esophageal atresia and abnormal rib count.

BACKGROUND: The presence of 13 pairs of ribs on pre-operative chest x-ray has been postulated to be an indicator for long gap esophageal atresia (EA). This study sought to determine the validity of this theory and identify associated pathological conditions in patients with EA and abnormal rib count. METHODS: Babies with EA from January 2005 - December 2012 were retrospectively analyzed. Information was gathered from neonatal health records and operation notes. Chest x-rays were reviewed to determine rib count. Long gap EA was defined as failure to achieve primary esophageal anastomosis. Statistical analysis performed with Fisher's exact test. RESULTS: Seventy-six patients were identified. Eight patients had long gap EA, with none of these patients having 13 pairs of ribs. Paradoxically, 10 patients with esophageal atresia +/- trachea-esophageal atresia (EA +/- TEF) and supernumerary ribs underwent primary repair. Nine patients had 11 pairs of ribs, of which 2 had pure EA and a long gap. Using Fisher's exact test to compare the groups of supernumary ribs and non-supernumary ribs there is a p value of 0.587. VACTERL association was identified in 40% of those with supernumerary ribs. Various associated syndromes and concomitant abnormalities were identified. CONCLUSION: We found no association between 13 pairs of ribs and long gap in esophageal atresia. Those with 13 pairs of ribs were more likely to have associated anomalies, although this was not statistically significant. Our cohort of patients was found to have a range of pathology related to genetic syndromes, further atresias, and malformations, which is well known to be associated with children born with EA +/- TEF. LEVEL OF EVIDENCE: Prognosis study - level IV.

Modified PATIO repair for urethrocutaneous fistula post-hypospadias repair: operative technique and outcomes.

BACKGROUND: To describe a modification of PATIO repair for urethrocutaneous fistula repair and evaluate its outcome. METHODS: We studied 15 boys who underwent modified PATIO repair from Jan 2010 to Sept 2015. Parameters studied included age, type of hypospadias, age at first urethroplasty, hypospadias repair technique, number of urethroplasties required, location of fistula, time gap between urethroplasty and fistula repair, method of fistula repair, and outcome of fistula repair. RESULTS: Mean age of the studied patients was 67.6 months (38-139). Type of hypospadias was Coronal = 3, subcoronal = 8, mid-penile = 2, prominal penile = 1, and penoscrotal = 1. Ten patients had single urethroplasty, while two patients had two urethroplasties, details not available for three patients. Average age at urethroplasty was 43.4 months (18-110). 12 patients had Snodgrass repair, Mathieu = 1 patient, tubularised plate repair = 1 patient, and unknown = 1 patient. Location of fistula was coronal in nine patients and subcoronal in six patients. Average operative time was 47.2 min (30-68). Follow-up is available for 12 patients out of which 2 patients had recurrent fistula, one of which was successfully treated by the redo modified PATIO method. CONCLUSION: Modified PATIO method is technically easy method for urethrocutaneous fistula repair, with less operating time and good postoperative outcomes.

Paediatric trauma with hyperamylasemia.

In this case report, we describe a paediatric case of hyperamylasemia following parotid trauma. A 12-year-old boy was hit by a motorcycle, sustaining only superficial lacerations to the face overlying the parotid. A hyperamylasemia was noted, and further characterised by a fractionated isoamylase test, as being predominantly of the salivary type. Serum lipase levels were low. Based on these investigations, pancreatic injury was judged unlikely with the minor parotid trauma being the probable source of the hyperamylasemia. The patient was spared further unnecessary investigations and managed conservatively.

Trilogy of foregut atresia without genetic abnormality: exception to the Martinez-Frias syndrome.

We present a case of oesophageal atresia with distal tracheo-oesophageal fistula and duodenal atresia, which later on was detected to have cystic variant of biliary atresia. He underwent primacy repair of oesophageal atresia with ligation of distal tracheo-oesophageal fistula and duodenoduodenostomy. He later developed features of obstructive jaundice, and on investigation was diagnosed to have cystic variant of biliary atresia which was initially confused with iatrogenic biliary obstruction. Exploration and operative cholangiogram with biopsy confirmed cystic variant of biliary atresia and underwent successful Kasai procedure. Our case effectively demonstrates a rare triology of foregut atresia without an apparent genetic association. It highlights stepwise systematic management of foregut atresias in this patient and demonstration of cystic variant of biliary atresia. Even though these are rarely seen, still the clinician should be aware of such a possibility and should adopt a multimodality approach to diagnose and an aggressive approach to manage the condition.

Embryology of the Absent Vas Supported by 2 Cases of Congenital Unilateral Absence of Vas With Varied Associations.

Congenital absence of the vas occurs in up to 1% of men. Congenital unilateral absence of the vas deferens can be related to cystic fibrosis transmembrane conductance regulator mutations or in 79% of cases, renal agenesis. We present a case of each, diagnosed in children at operation for elective inguinal hernia repairs. One patient had associated ipsilateral renal agenesis with a normal cystic fibrosis screen. The other patient had an ipsilateral pelvic kidney and a mutation detected on cystic fibrosis screening. Current understanding of the embryology of the relationship between these defects would seem to be supported by our cases.

Red herring vaginal discharge.

Labial hair tourniquet syndrome is a rare condition that can be easily misdiagnosed and ultimately lead to irreversible damage. An 11-year-old premenarche girl presented with a 5-day history of pain and swelling in the labia with associated vaginal discharge. The general practitioner treated her with clotrimazole without improvement. On examination, there was an oedematous swelling of the right labia with a proximal hair tourniquet. Local anaesthetic was applied and the hair removed with forceps. There was instant relief of pain and the discharge stopped within 24 h. The patient was sent home with a course of antibiotics.

Closing left gastroschisis with vanishing left testis.

We report a baby boy with gastroschisis with left non-palpable undescended testis who had a defect on the left side of an intact and normal umbilical cord and had associated testicular atrophy and abnormalities of the ductus deferens. They were successfully managed by primary repair and had uneventful recovery. Subsequent inguinal exploration confirmed blind ending vas deferens and vanishing left undescended testis. Our case confirms vascular accidents at the narrow abdominal wall defect can lead to vanishing testis following attempts at closing gastroschisis making the defect narrow and compromising the blood supply to the testis.

Primary group A streptococcal septic shock syndrome simulating perforated appendicitis in a previously healthy girl.

A previously healthy 6-year-old girl that initially presented with presumed viral gastrointestinal infection for a week and later developed catastrophic primary peritonitis and septic shock requiring resuscitation and emergency exploratory laparotomy without any identifiable intra-abdominal cause of the sepsis and the peritoneal exudates grew group A streptococci. Appropriate antibiotic therapy was instituted and she made a complete recovery.

Laparoscopic management of impalpable testes: comparison of different techniques.

PURPOSE: Laparoscopy is an important modality for management of impalpable testes. We present long-term outcomes of intra-abdominal testes managed by either single stage orchidopexy or two-stage Fowler Stephen's orchidopexy (FSO) over 12 years. METHODS: Data were prospectively collected and retrospectively analyzed on patients who underwent laparoscopy for impalpable testes between 1998 and 2010. Demographic data, intra-operative findings, management, histology and follow-up findings were collected and analyzed. Fisher's Exact test was used for statistical analysis. RESULTS: Laparoscopy was performed for 168 impalpable testes (78 left, 58 right and 16 bilateral). Patients were between 8 months and 15 years of age (median 1 year 10 months). Ninety-three testes were found to have cord structures entering the inguinal ring (canalicular), 65 were intra-abdominal and 10 had blind ending vas and/or vessel. Fifty-seven (34%) testes were atrophic and underwent orchidectomy; 100 (60%) testes underwent orchidopexy: either two-stage FSO (48) or single stage orchidopexy (52) and 10 (7%) had findings consistent with 'vanishing testes'. Histopathologically, the excised remnants (34%) showed no viable testicular tissue. The follow-up was a median of 8 months (3 months to 6 years). Four patients were lost to follow-up (two each after FSO and single stage orchidopexy) while, two FSO are awaiting follow-up. At follow-up, 36/44 testes (FSO) and 13/13 testes (single stage orchidopexy) are in the scrotum and of good size. Eight testes had atrophied after two-staged FSO. CONCLUSION: Canalicular testes are often difficult to palpate (55%). Laparoscopy allows direct visualization and definitive management. There is no statistically significant difference between the results following single stage orchidopexy or two-stage FSO for impalpable testes.

Excision of massive bladder diverticula in Menkes disease.

Menkes disease is a rare disorder of copper metabolism. Massive bladder diverticula are a characteristic urological abnormality associated with this condition. In this case report, we describe excision of such diverticula with good postoperative results in terms of bladder function. There are only three previous instances reported of excision of these diverticula, and the postoperative outcome has not been described previously.

Gonadoblastoma and Turner syndrome.

PURPOSE: The presence of a Y chromosome in the extrascrotal gonad of patients with intersex disorders has been associated with a high risk of GB and, potentially, GCT. Recently, modern sophisticated genotyping has revealed a subgroup of TS cases with a mosaic karyotype expressing a Y chromosome. We sought to evaluate this group of patients and address their risk of gonadoblastoma. MATERIALS AND METHODS: We reviewed the records and genotyping of all females newly diagnosed with TS between 1990 and 2002 at Children's Hospital in Denver. All patients with TS and Y chromosome mosaicism underwent gonadectomy, and the specimens were evaluated for the presence of gonadoblastoma on histological analysis and to identify Y chromosome on genotyping. RESULTS: A total of 192 girls with a clinical diagnosis of TS were identified between January 1990 and December 2002. Seven records were unavailable and 19 patients did not have karyotypic analyses available in the hospital charts. Of the remaining 166 patients 67 exhibited mosaic cell lines, of whom 8 had 45,X0/46,XY mosaic pattern and 59 had mosaic patterns without Y chromosomal elements. All 8 patients with Y mosaicism underwent uneventful laparoscopic gonadectomy on an outpatient basis. One patient observed to have bilateral dysgenetic gonads after gonadectomy was excluded from the study. Gonadoblastoma (bilateral 2 patients, unilateral 1) was detected in 3 of 7 patients (43%) with Y mosaicism. CONCLUSIONS: In our series 4.8% of evaluable patients with TS carried a 45,X0/46,XY karyotype. Gonadoblastoma can be evident even at an early age in streak gonads with Y mosaicism and may be bilateral. We recommend prophylactic laparoscopic gonadectomy of streak gonads in patients with TS who carry a Y mosaic genotype, because fertility is not an issue, surgical morbidity is minor and there may be a high potential for malignant transformation of gonadoblastomas in this population.

High scrotal (Bianchi) single-incision orchidopexy: a "tailored" approach to the palpable undescended testis.

Our aim was to evaluate the utility of the high scrotal orchidopexy (Bianchi) approach for palpable undescended testis (UDT) and to assess long-term follow-up. We reviewed the records of orchidopexies performed between 1999 and 2002. The patients were then categorized by intraoperative exam under anesthesia as to whether their testes were palpable or nonpalpable. All palpable UDT that were initially thought to be amenable to a single high scrotal approach (Bianchi) were then reviewed. These cases were then analyzed to assess the impact of patient age, initial location of the testis, and prior inguinal/scrotal surgery with respect to the necessity to convert to a standard two-incision technique, and to analyze success and complications at 6-12-week and 1-year follow-up. Two hundred and nineteen orchidopexies were performed on 204 patients over this 4-year period. There were 178 testes palpable, and the transscrotal approach was used in 85 patients (100 orchidopexies). The preoperative positions of the testes that were thought to be amenable to Bianchi technique included the following: gliding (19), secondary trapped (25), superficial inguinal pouch (42), and location within the inguinal canal (2), while the remaining 12 testes were ectopic. Six patients required conversion to a traditional inguinal approach because of insufficient cord length via the single incision to allow the testis to lie in the scrotum. All patent processes vaginalis were ligated via the scrotal incision, regardless of their size. All patients, except for one who had a testis in the superficial inguinal pouch, had palpable testes of stable size and in a dependent position at 6-12-week follow-up. Of the 62 children who returned for 1-year follow-up, all had findings identical to those at their initial 6-week visits, with no atrophy or secondary reascent. Postoperative complications included transient postoperative scrotal hematoma in a single patient. The single failure underwent a successful two-incision orchidopexy for secondary reascent and a resultant trapped testis. Children with primary palpable undescended, gliding, or trapped testes can be managed successfully through the transscrotal route in the majority of cases. With use of a tailored approach to the palpable UDT, an additional groin incision is necessary only for a minority of appropriately selected cases.

Laparoscopic Palomo varicocele ligation in children and adolescents: results of 103 cases.

PURPOSE: We evaluate our experience using the laparoscopic Palomo varicocele ligation (LPV) technique in male children and adolescents with varicoceles. MATERIALS AND METHODS: Between September 1994 and September 2002, 122 varicoceles were treated using LPV with either mass cord vascular clip application (68) or LigaSure (Valleylab, Boulder, Colorado) vascular sealing (54). All procedures were performed as day surgery cases and patients were allowed to return to normal activities as soon as they could tolerate them. Followup was scheduled for 6 to 12 weeks and 1 year postoperatively. Testicular size was evaluated using a Prader orchidometer. RESULTS: Of the 122 patients 103 were evaluable at the initial postoperative visit and 96 at 1-year followup. Patient ages ranged from 9 to 19 years (mean 14.6). Operating time was 9 to 52 minutes (mean 28), although in the last 2 years (28 cases) mean operative time has decreased to 14.6 minutes. Indications for surgery included ipsilateral testicular hypotrophy in 84 cases, symptoms in 3 and parental choice in 16. At followup only 1 recurrent varicocele (vascular sealant group) was identified which was subsequently re-treated successfully using vascular sealant LPV. At 1 year 64 of 78 testes (82%) demonstrated catch-up growth and there was no evidence of testicular loss or persistent hypotrophy in the others. Reactive hydroceles were identified in 6 patients equally distributed between the 2 LPV techniques. Surgery was required on 2 of these hydroceles due to size, and the other 4 are small and are being observed. Temporary scrotal emphysema occurred in the vast majority of patients and 1 patient required laparoscopic closure of a small sigmoid serosal tear. No patients required narcotic medications for greater than 48 hours. CONCLUSIONS: LPV is a highly successful method to correct varicoceles in young males with catch-up growth similar to series using other accepted standard techniques. LPV can be performed safely and rapidly as an outpatient and allows early return to activity. As with the open Palomo technique, hydrocele is a bothersome complication that may require a second surgical procedure if correction is warranted.

Vaginal construction using sigmoid colon in children and young adults.

OBJECTIVE: To evaluate the age at which patients who required vaginal replacement (an uncommon procedure in children) were diagnosed, and the cause of their anomaly, and to relate these variables to the surgical outcome. PATIENTS AND METHODS: Patients who had vaginal replacement at the author's institution between 1990 and 2002 were reviewed retrospectively. Depending on the age at reconstructive surgery, patients were divided into pre- and postpubertal groups. RESULTS: A neovagina was constructed in 23 patients during the study period; sigmoid colon was used in 20 but not in two patients with cloacal exstrophy and in one with Mayer-Rokitansky-Kuster-Hauser syndrome (MRKHS). These cases were excluded from the analysis of outcomes and complications. Group 1 comprised patients diagnosed and treated before puberty and group 2 those diagnosed and/or treated afterward. In group 1 the presenting diagnoses included androgen insensitivity syndrome (AIS) in six patients, MRKHS in two, cloacal exstrophy in two, vaginal tumour in one, Müllerian duct renal aplasia cervicothoracic somite dysplasia, vertebral abnormalities, anal atresia, cardiac anomalies, tracheo-oesophageal fistula, and/or oesophageal atresia, renal abnormalities and limb defects syndromes in one each. In group 2 the presenting diagnoses included MRKHS in seven, AIS in two, and congenital adrenal hyperplasia in one. Complications included superficial wound infection (two patients), recurrent introital stenosis, and blind loop mucocele, complete stenosis of perineal neovaginal opening (one each) and dyspareunia in three. Neither age nor pelvic habitus (android vs gynaecoid) influenced the outcome, and the cosmetic results were excellent in all the patients. CONCLUSION: Isolated sigmoid neovaginal construction appears to be applicable to many diagnoses and in patients at any age. Although an android pelvis can present technical challenges, in this experience it was not associated with a greater complication rate. The long-term satisfaction with the sigmoid neovagina for intercourse, especially in those constructed before puberty, still requires long-term evaluation.

Octyl-2-cyanoacrylate as a routine dressing after open pediatric urological procedures.

PURPOSE: Octyl-2-cyanoacrylate Dermabond, Ethicon, Inc., Sommerville, New Jersey is a synthetic tissue adhesive that has recently been used for skin closure in the treatment of minor lacerations. We assess its effectiveness as a sole dressing after open pediatric urological procedures. MATERIALS AND METHODS: Between February and August 2003 we prospectively evaluated patients undergoing extragenital open and laparoscopic pediatric urological procedures at our institution. All open incisions were closed in layers using a final layer of self-absorbing subcuticular stitches for the skin before applying Dermabond only at the skin level. In laparoscopic cases Dermabond alone was applied to port sites (3 mm or less) and instrument sites without any sutures beneath the skin. No adjuvant occlusive dressings were applied to any of these wounds. All patients were allowed to bathe and return to activity immediately postoperatively. RESULTS: During the study period 146 patients with 200 incisions were identified (open 146, laparoscopy 54). Of these children 103 (142 incisions) returned for followup during the period of study with only 1 complication identified. This 6-month-old child presented the evening of surgery with omental prolapse through the umbilical port (3 mm port) requiring urgent closure. In no case was there an appreciable healing problem on surgeon or parental examination and no wound infections occurred. CONCLUSIONS: Dermabond alone provides a simple coverage for a myriad of pediatric urological surgical wounds. Early bathing and return to activity do not appear to impact negatively on wound healing when a simple skin barrier is used in place of standard dressings.