Transition and reversal: long-term experience of the MACE procedure in children.

PURPOSE: Analysis of outcomes and follow-up of children who underwent the Malone antegrade continence enema (MACE) procedure in a UK tertiary paediatric surgery unit. METHODS: Children who underwent a MACE procedure from 1998 to 2020 were identified. Demographic and clinical data were obtained from contemporaneous records. Outcomes were categorised as full (success), partial or failure. RESULTS: Ninety-five children were identified for inclusion (chronic idiopathic constipation (CIC, 59), anorectal malformations (ARM, 23) and Hirschsprung's disease (HD, 13)). Mean age at surgery was 9.4 years (3-19 years) and mean follow-up time was 6 years (0.3-16.8 years). Outcomes were successful in 69% of CIC patients, 78% in ARM and 69% in HD. Twenty (21%) underwent MACE reversal after developing independent continence, with a significant difference between groups (CIC 19%, ARM 9%, HD 54%, p = 0.0047). 50% of patients > 16 years old were transitioned to adult services. CONCLUSION: We report a success rate of 72% for MACE procedures in our unit, with a significant difference in reversal rate between diagnostic groups. Long term, a fifth of patients no longer required their MACE. When these patients reach adolescence, those who require ongoing support outside of the paediatric surgery setting should be safely transitioned to adult services.

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.

Extensive Necrotising Enterocolitis: Objective Evaluation of the Role of Second-Look Laparotomy in Bowel Salvage and Survival.

AIM: We investigated the role and outcome of a planned second-look laparotomy (SLL) in preserving bowel in extensive necrotizing enterocolitis (NEC). METHODS: Extensive NECs managed surgically in a tertiary centre in 2006-2009 were retrospectively studied to include patients planned for an SLL. End points were bowel salvage rate and survival outcomes. Results were median (ranges), and statistical significance was P < 0.05. MAIN RESULTS: In 4 years, 34 NECs required a laparotomy, and 9 extensive NECs who required an SLL were included. The gestation at birth was 27 (24-38) weeks, birth weight was 1120 (580-2835) g, and first laparotomy performed on day 34 (2-77) of life, with SLL performed 2 (1-3) days after initial laparotomy. Commonest indications for SLL were doubtful bowel viability and physiological instability. 3 died before SLL. Patients who survived to have an SLL (n = 6) had remaining small bowel length of 41 (25-70) cm, overall small bowel salvage rate 51 % (0-100 %), and 30-day survival 5/6 (83 %). Four patients survived for 1 year, their length of NICU stay was 114 (76-120) postoperative days, time on PN was 84 postoperative days (71 days-17 months), including one patient with short bowel syndrome who achieved enteral autonomy at 17 months; one late mortality had short bowel syndrome after further bowel resection for bowel obstruction, developed intestinal failure associated liver disease, and died before 1 year of life following liver transplant. CONCLUSION: SLL is a viable approach for extensive NEC. It offered bowel salvage rate of 51 % and long-term PN-free survival of 44 %, in the patient group who would have had significant risk of mortality and major morbidity.

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.

Experience of the MACE procedure at a regional pediatric surgical unit: a 15-year retrospective review.

INTRODUCTION: The Malone antegrade continence enema (MACE) procedure is an established treatment option for children with constipation or fecal incontinence. This study retrospectively analyses the management and outcomes of children who underwent MACE procedures at a regional pediatric surgery unit. PATIENTS AND METHODS: Children who underwent a MACE procedure in our unit between 1998 and 2012 were identified. Demographic and clinical data were obtained from contemporaneous records. Using the continence scale described by Malone, overall outcomes were categorized as full, partial, or failure (full: totally clean or minor rectal leakage on night of washout; partial: clean but significant stoma or rectal leakage, occasional major leak and/or still wearing protection but perceived by child or parent to be an improvement; failure: regular soiling or constipation persisted, no perceived improvement, procedure was abandoned). Data entry and statistical analysis were performed using Excel and SPSS (IBM Corp., Armonk, New York, United States). RESULTS: A total of 40 children (29 male) were identified for inclusion. Underlying diagnoses were idiopathic constipation (16), anorectal anomalies (14) and Hirschprung's disease (10). The MACE procedure was performed laparoscopically in 26 cases and using an open technique in 14 cases. Mean age at the time of surgery was 8.9 years (range, 3-19 years) and mean follow-up time was 6.5 years (range, 1-10 years). Outcomes categorized as full were 62.5% for chronic idiopathic constipation (CIC), 71.4% for anorectal malformation (ARM), and 70% for Hirschsprung disease (HD). Overall success rates (full and partial outcomes combined) were 87.5% for CIC, 92.8% for ARM, 100% for HD, and 92.5% for all diagnoses taken together. Eleven MACE procedures (27.5%) were reversed, in seven (17.5%) due to the return of spontaneous and regular bowel movements and in four (10%) due to stomal problems. CONCLUSION: This study identified a high success rate (combining full and partial outcomes) of 92.5% for MACE procedures within our unit. An encouraging finding is that the procedure was reversed in a significant proportion of patients following the return of normal bowel habits.

Pseudocyst and a collar stud abscess: new face of necrotizing enterocolitis.

Meconium pseudocyst formation secondary to antenatal perforation is well described. We present a preterm infant who had intra-abdominal pseudocyst formation following postnatal intestinal perforation secondary to necrotizing enterocolitis (NEC) and secondarily leading to extra-abdominal collar stud abscess. This is new face of NEC and this presentation has not been reported earlier.

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.

Paediatric recurrent herpetic whitlow.

We present a case of recurrent painful blisters of middle phalanx of the left ring finger of a 15-month-old previously healthy and immunocompetent female child. These lesions initially were confused with infective bacterial whitlow, treated with incision and drainage, and later with cigarette burns which led to referral to child protection team. Paediatric dermatologist finally diagnosed after scrapping and virology culture. The patient had recovery following full treatment with topical and systemic acyclovir. She presented again at the age of 4 with recurrence which required topical and systemic acyclovir therapy with good recovery. It is important to be aware of the danger of incorrect diagnosis, raising child protection concerns and management leading to danger of cross infection and serious illness especially in the immunocompromised patients.

Inferior gastroschisis.

Gastroschisis is a common defect of the central abdominal wall, nearly always located to the right of the umbilicus. We report a baby boy with gastroschisis who had a defect on the inferior side of an intact and normal umbilical cord which was managed by primary repair and an uneventful recovery. Such a case has never been reported in the literature. This case contributes towards enlarging the spectrum of gastroschisis.

Massive lower gastrointestinal haemorrhage in a teenager caused by Campylobacter enteritis.

A 14-year-old boy presented with a very unusual complication of massive lower gastrointestinal bleeding mimicking bleeding from Meckel's diverticulum who needed blood transfusions and required mini-exploration. He was found to have Campylobacter enteritis with bleeding from multiple mucosal ulcers in the ileocaecal region, which is presented with a review of the literature.

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.

Bilateral congenital lobar emphysema: how should we proceed?

We report a case of a 4-week-old male infant who presented with increasing respiratory distress and was diagnosed to have bilateral congenital lobar emphysema on chest x-ray. Our approach to the surgical management in two sequential operations of left upper lobectomy followed by right middle lobectomy is discussed.

Lymphangioma of the penis: a rare anomaly.

Lymphatic malformations are known to affect any part of the body. However, lymphangiomas involving the penile skin are rare. We report a case of a cutaneous lymphatic malformation of the penis.

Pyloric duplication in a neonate: a rare entity.

Duplications of the alimentary tract are rare anomalies that have been reported to occur all along the gastrointestinal tract. Of the various alimentary tract duplications, pyloric duplications are extremely rare. We report the case of a 3-day-old neonate who was antenatally diagnosed as having a cystic mass in the abdomen and who presented with vomiting on the 2nd day of life. At operation, a duplication cyst of the pylorus was removed successfully and a pyloroantrectomy performed.