Umbilical hernia following gastroschisis closure: a common event?

PURPOSE: To assess incidence and natural history of umbilical hernia following sutured and sutureless gastroschisis closure. METHODS: With audit approval, we undertook a retrospective clinical record review of all gastroschisis closures in our institution (2007-2013). Patient demographics, gastroschisis closure method and umbilical hernia occurrence were recorded. Data, presented as median (range), underwent appropriate statistical analysis. RESULTS: Fifty-three patients were identified, gestation 36 weeks (31-38), birth weight 2.39 kg (1-3.52) and 23 (43 %) were male. Fourteen patients (26 %) underwent sutureless closure: 12 primary, 2 staged; and 39 (74 %) sutured closure: 19 primary, 20 staged. Sutured closure was interrupted sutures in 24 patients, 11 pursestring and 4 not specified. Fifty patients were followed-up over 53 months (10-101) and 22 (44 %) developed umbilical hernias. There was a significantly greater hernia incidence following sutureless closure (p = 0.0002). In sutured closure, pursestring technique had the highest hernia rate (64 %). Seven patients underwent operative hernia closure; three secondary to another procedure. Seven patients had their hernias resolve. One patient was lost to follow-up and seven remain under observation with no reported complications. CONCLUSIONS: There is a significant umbilical hernia incidence following sutureless and pursestring sutured gastroschisis closure. This has not led to complications and the majority have not undergone repair.

Esophageal atresia and malrotation: what association?

INTRODUCTION: Esophageal atresia/tracheo-esophageal fistula (EA/TEF) has an incidence of approximately 1:3,500. The incidence of malrotation is thought to be 1:200-500. We attempted to define the incidence of a combination and discuss the implications. METHODS: This was a retrospective review of all patients admitted to a single institution with a diagnosis of EA or EA/TEF or TEF between April 1981 and January 2013. Patients were included if the position of the duodeno-jejunal flexure (DJF) was determined by upper GI contrast study (UGIS), surgery or post-mortem. RESULTS: Case notes were reviewed for 235 patients. In the EA type A group, 3/28 (11 %; 95 % CI 3.7-27.2 %) had malrotation, significantly higher than the reported incidence of malrotation in the general population (p = 0.0008). All three patients in this group were symptomatic with one patient found to have a volvulus at emergency surgery. In the type C group, 6/196 (3 %, 95 % CI 1.4-6.5 %) had malrotation, significantly higher than the incidence reported for the general population (p = 0.0033) but not significantly different to that of the type A group (p = 0.0878). There were no patients with malrotation identified in any other EA/TEF type. In total, 9/235 (3.8 %; 95 % CI 2.0-7.2 %) patients with EA had malrotation, significantly higher than the 5/1,050 (0.48 %) reported for the general population (p = 0.0002). CONCLUSION: There is a high incidence of malrotation in patients with pure EA. In the type A group an attempt to identify the DJF position at gastrostomy siting and/or performance of UGIS in the neonatal period should be undertaken. There should also be a low threshold for UGIS in all EA/TEF patients.

The role of parenteral nutrition following surgery for duodenal atresia or stenosis.

PURPOSE: In our institution, some children routinely receive parenteral nutrition (PN) following surgery for duodenal atresia/stenosis, while others do not. Our aim was to compare growth and infection rate between these two treatment strategies. METHODS: This was a retrospective study of all children undergoing surgery for duodenal atresia/stenosis over 7 years. RESULTS: Of the 54 children, 19 commenced PN soon after surgery (the 'Initial PN' group). Of the remaining 35 children, 13 (37 %) subsequently required PN (the 'Delayed PN' group). The remaining 22 never received PN (the 'Never PN' group). The proportion of patients experiencing clinically suspected sepsis was higher in those receiving PN ('Initial' plus 'Delayed'; 41 %) compared with those who never received PN (14 %; p = 0.04). The 'Initial PN' and 'Never PN' groups did not show a significant change in weight Z score over time. However, the 'Delayed PN' group showed a significant decrease in weight Z scores from the time of operation to the time of achieving full enteral feeds, and failed to catch up by the time of last follow-up. CONCLUSION: Children with duodenal atresia/stenosis can be managed without PN. However, a third of these children subsequently require PN, lose weight centiles, and have a high rate of sepsis.

Umbilical polyp in infants and children.

AIM OF THE STUDY: The umbilical polyp is a rare congenital lesion resulting from the persistence of omphalomesenteric duct (OMD) enteric mucosa at the umbilicus. Exploration of the abdomen to exclude the presence of associated OMD remnants is controversial. The aim of this study was to evaluate the need for peritoneal cavity exploration in children with umbilical polyp. METHODS: All umbilical lesions (n = 53) excised between 1995 and 2005 in a single institution were reviewed to identify patients with umbilical polyp (n = 13). This is characterised histologically by the presence of gastrointestinal mucosa. A follow-up study of patients with umbilical polyp was performed. Data are reported as median (range). RESULTS AND CONCLUSIONS: All 13 patients underwent excision of an umbilical polyp at a median age of 15.1 months (3.1 - 80.5). All presented with a discharging polyp (associated with bleeding in 9) which did not respond to topical silver nitrate. Median diameter of the lesions was 0.5 cm (0.2 - 1). Histology revealed the presence of small bowel mucosa in 11 (associated with pancreatic tissue in 1 and gastric mucosa in 1) and large bowel mucosa in 2. All patients underwent inspection and probing of the base of the polyp after its excision. In 6 patients an associated OMD anomaly was suspected and exploration of the peritoneal cavity was performed (mini-laparotomy in 5 and laparoscopy in 1). No OMD anomaly was found. The 7 children who did not undergo exploration of the abdominal cavity remain asymptomatic after 5.8 years (0.9 - 13.7) follow-up. An umbilical polyp can be present in the absence of other OMD anomalies. Exploration of the peritoneal cavity in children with an umbilical polyp does not seem to be necessary.

Laparoscopic surgery in children is associated with an intraoperative hypermetabolic response.

BACKGROUND: Laparoscopic surgery is thought to be associated with a reduced metabolic response compared to open surgery. Oxygen consumption (VO2) and energy metabolism during laparoscopic surgery have not been characterized in children. METHODS: We measured respiratory gas exchange intraoperatively in children undergoing 19 open and 20 laparoscopic procedures. Premature infants and patients with metabolic, renal, and cardiac abnormalities were excluded. Anesthesia was standardized. Unheated carbon dioxide was used for insufflation. VO2 was measured by indirect calorimetry. Core temperature was measured using an esophageal temperature probe. RESULTS: We found a steady increase in VO2 during laparoscopy. The increase in VO2 was more marked in younger children and was associated with a significant rise in core temperature. Open surgery was not associated with significant changes in core temperature or VO2. CONCLUSIONS: Laparoscopy in children is associated with an intraoperative hypermetabolic response characterized by increased oxygen consumption and core temperature. These changes are more marked in younger children.

Resection and primary anastomosis is a valid surgical option for infants with necrotizing enterocolitis who weigh less than 1000 g.

HYPOTHESIS: Primary anastomosis following intestinal resection is a valid surgical option in the treatment of infants with necrotizing enterocolitis (NEC) who weigh less than 1000 g. DESIGN: Retrospective case series. SETTING: Tertiary neonatal surgery referral center. PATIENTS: All infants with confirmed NEC weighing less than 1000 g admitted to our intensive care unit over 4 years. INTERVENTION: For infants requiring laparotomy and intestinal resection, primary anastomosis was performed whenever the clinical condition permitted. MAIN OUTCOME MEASURES: Short- and long-term survival, length of intensive care unit stay, and complications. RESULTS: Fifty-one infants with NEC who weighed less than 1000 g were admitted during the study period. Twelve infants underwent intestinal resection and primary anastomosis (median weight at surgery, 0.83 kg; range, 0.6-0.96 kg). One infant developed recurrent NEC, requiring further surgery, but there were no anastomotic leakages and no strictures. The median postoperative stay on our intensive care unit was 14 days (range, 2-49 days). All 12 infants survived their episode of acute NEC, and 8 are alive, with a median follow-up of 34.2 months (range, 4.7-48.4 months). Only 1 death was related to NEC. During the same period, 14 infants underwent intestinal resection and stoma formation. Ten survived the acute episode, and 6 are alive at a median follow-up of 24.0 months (range, 13.1-33.9 months). The median postoperative intensive care unit stay was 18 days (range, 2-74 days). Necrotizing enterocolitis-related complications occurred in 8 of these infants. CONCLUSIONS: The outcome of infants with NEC who weigh less than 1000 g and undergo primary anastomosis is comparable to that of infants treated using alternative techniques. It is, therefore, a valid surgical option in selected infants. The mortality in this group of infants is high.

Diarrhea after resection of advanced abdominal neuroblastoma: a common management problem.

BACKGROUND: After resection of advanced abdominal neuroblastoma, children may have persistent postoperative diarrhea. Until recently, the magnitude of this problem had not been appreciated. METHODS: To assess the incidence, severity, and management of chronic postoperative diarrhea in these patients, we reviewed the case notes of all children with stage III or IV abdominal and pelvic neuroblastoma who underwent tumor resection in our hospital between January 1985 and September 1996. We classified the severity of diarrhea as follows: mild, less than 3 loose stools per day; moderate, 3 to 5 loose stools per day; and severe, more than 5 loose stools per day and/or urgency, incontinence, or nocturnal diarrhea. RESULTS: Seventy-seven children underwent resection during this period, and 23 (30%) had postoperative diarrhea, classified as mild in 11 patients, moderate in 7, and severe in 5. Dissection around the superior mesenteric and celiac arteries was associated with a significantly higher incidence of diarrhea. Fifteen children (65%) received treatment with loperamide, which reduced but did not abolish symptoms. Twelve children subsequently died of progressive neuroblastoma. Of the 11 surviving children (mean duration of follow-up, 8.4 years), 8 have persistent loose stools. CONCLUSIONS: Diarrhea, probably resulting from disruption of the autonomic nerve supply to the gut during clearance of tumor from the major vessels of the retroperitoneum, is common after resection of advanced abdominal neuroblastoma. Many children require long-term treatment to slow intestinal peristalsis, and a few have severe and unremitting diarrhea. More effective medical management of this complication is needed.

Operative trauma to the genitourinary tract in the treatment of anorectal malformations: 15 years' experience.

OBJECTIVES: To determine the incidence of urologic trauma during surgical correction of anorectal malformations (ARM). METHODS: The records of 228 children with ARM were retrospectively reviewed. RESULTS: Operative trauma to the genitourinary tract was seen in 12 patients, all male. Of 133 patients with a high ARM, 92 underwent an abdominoperineal pull-through and 41 underwent a posterior sagittal anorectoplasty (PSARP); the incidence of urologic trauma was 12% and 0%, respectively. In the subgroup of male infants with a high ARM (99 patients), the incidence of genitourinary trauma was 11%; in those with a low anomaly, the incidence was 1 of 43 (2%). There were 3 urethral tears, 4 urethral strictures, 5 urethral diverticula, 2 vas deferens injuries, and 1 ureteric injury. Nine of the 12 patients do not have any long-term sequelae resulting from the injuries; however, 2 are now infertile and 1 has urinary incontinence. Four patients are additionally compromised because of an absent or nonfunctioning kidney on one side. CONCLUSIONS: In children with ARM, those undergoing a conventional pull-through repair are more likely to have a genitourinary injury than those undergoing the PSARP, and boys with high ARM are at greatest risk.

Associated urologic anomalies in low imperforate anus are capable of causing significant morbidity: a 15-year experience.

OBJECTIVES: To determine the incidence and morbidity caused by urologic anomalies in patients with low imperforate anus. METHODS: The case records and imaging studies of 95 patients who underwent surgery for low imperforate anus over a 15-year period were retrospectively reviewed. Follow-up ranged from 2 to 18 years (mean 5.4). RESULTS: Genitourinary abnormalities were seen in 29 patients (31%), whereas 10 (11%) had lumbosacral abnormalities. A micturating cystourethrogram was performed in 48 patients, 18 of whom were shown to have vesicoureteric reflux and 2 of whom required surgical correction. Three additional patients required bilateral ureteric reimplantation: 2 had megaureters whereas 1 had bilateral ectopic ureters. Four patients had evidence of chronic renal failure; these included 1 patient with reflux nephropathy, 2 with bilateral dysplastic kidneys, and 1 with neurogenic bladder. The patient with reflux nephropathy underwent renal transplantation at the age of 18 years. Two patients had proximal hypospadias and 4 had undescended testes. A neurogenic bladder was documented in 5 children, 4 of whom had lumbosacral abnormalities. One of these had a spinal lipoma and required excision of the lipoma and untethering of the spinal cord. CONCLUSIONS: We conclude that 30% of patients with low imperforate anus would have associated urologic anomalies, several of which are capable of causing significant morbidity. Thus, all patients with imperforate anus, whether high or low, need to be screened for associated urologic anomalies.

Gastric malignant schwannoma in a child.

Malignant schwannoma (malignant peripheral nerve sheath tumour, MPNST) is a high grade sarcoma with a potential for local recurrence and distant metastasis that may occur at any site in the body where there is neural tissue. MPNST is rare in children and is unreported in the stomach in the paediatric age group. MPNST presents either as an expanding mass, with or without pain, or in the gastrointestinal tract with haemorrhage or obstruction. Many cases occur without evidence of neurofibromatosis but thers is a reported association between MPNST and neurofibromatosis of up to 50%. MPNST has a higher incidence at sites of previous irradiation. Treatment is by complete surgical excision. Radiology is of value in initial diagnosis and staging prior to surgery. The definitive diagnosis is made on the histopathological appearance and immunohistochemical profile. The findings on barium meal, abdominal ultrasound and CT are presented.

The surgical challenge of neuroblastoma.

The first successful excision of a neuroblastoma took place in 1916. For many years there was no other form of treatment, and the outlook remained dismal. The use of radiotherapy (1928) and subsequently combination chemotherapy (1965) had a modest impact. More accurate staging has allowed a more coherent approach to diagnosis and treatment. Surgery has not been standardized to the same degree. A review of the author's experience with 129 patients over a 10-year period confirms that surgery is the mainstay of treatment for localized nonadvanced disease (stages I and II). Fourteen of 15 such patients are alive and disease-free. Combination chemotherapy may shrink advanced tumors (stages III and IV) and ablate metastases. Subsequent complete surgical excision does not improve survival. Approximately two thirds of those with stage III disease and one third of those with stage IV disease survive regardless of whether excision is complete. The present results do not support the value of complete surgical excision.

Repeat pull-through for Hirschsprung's disease.

BACKGROUND: A repeat pull-through for Hirschsprung's disease is undertaken when the original procedure has failed. The reasons for failure include a retained aganglionic segment, stricture, fistula, or persisting incontinence. METHODS: All patients who underwent a redo pull-through from 1982 through 1996 were included. Twenty-two patients were underwent surgery; 20 notes were reviewed. The mean age at operation was 6 years (range, 1 to 13). Mean time between operations was 5.1 years (range, 0.5 to 13). RESULTS: Postoperative complications occurred in five patients. These included wound infection, recurrent rectal septum, division of a vas, compartment syndrome, and an anastomotic stricture. Follow-up was for 6.5 years (range, 0.2 to 12). The mean number of bowel movements was 2.2 per day (range, 1 to 6). Fourteen (of 19) patients were continent or soiling less than once per week. Soiling occurred more than once a week in four, but two of these were incontinent after the first operation. One has a colostomy. CONCLUSION: A repeat pull-through is a worthwhile procedure when dealing with an anatomic problem such as retained Hirschsprung's disease, stenosis, or fistula.

The Malone (antegrade colonic enema) procedure: early experience.

PURPOSE: The aim of this study was to assess the results of the Malone (antegrade colonic enema) procedure for fecal incontinence. METHODS: By a retrospective review of patients treated between 1990 and 1996 in a tertiary referral center, 36 patients were treated with a Malone procedure. Age at operation was 8.3 (range, 3 to 14) years, the mean period of follow-up was 39 (range, 9 to 72) months. The indication was fecal soiling in 35 and chronic constipation in one. The underlying diagnosis was an anorectal anomaly in the majority of patients. The appendix was used in 30 patients and a cecal flap in six, and a submucosal antireflux procedure was also performed in 10. In 35 patients, a circular stoma was created and in one a V flap was used. Antegrade colonic enemas were performed daily in 10, alternate days in 23, and in three patients the stoma was no longer used. Enemas were performed with a 10F catheter using a mixture of phosphate enema (or liquorice) and saline. RESULTS: Fecal soiling was completely controlled in 28 patients, and eight children soiled more than once a week. Complications occurred in 15 patients; the main problem was stenosis of the conduit, which occurred in 9 of 30 appendiceal stomas and three of six cecal stomas. Stomal stenosis was treated with surgical revision in eight patients. Additional complications were reflux through the stoma (n=2), pain on catheterisation (n=1), and small bowel obstruction (n=1). In one patient the Malone has been converted to a colostomy. CONCLUSIONS: The Malone procedure is a simple technique that can effectively control fecal incontinence in the majority of cases. It appeared to be better in older children. Stomal stenosis is a frequently encountered problem that may require surgical revision.

Xiphoomphaloischiopagus tripus conjoined twins: successful separation following abdominal wall expansion.

A case of xiphoomphaloischiopagus tripus conjoined twins is reported. Particular emphasis was paid to the large abdominal wall defect that would be produced by separation, and the complications resulting from the use of intraperitoneal Silastic tissue expanders are described. As a result of adequate tissue expansion and the use of a vascularized pedicle graft from the fused limb, primary abdominal wall and pelvic closure was possible without complications of wound healing. The consistent finding of a shared lower urinary tract with one ureter from each infant entering each bladder should be anticipated in this type of twinning.

Separation of thoraco-omphalopagus conjoined twins with complex hepato-biliary anatomy.

This report describes separation of thoraco-omphalopagus conjoined twins and management of the hepatobiliary complications that ensued. The livers were fused and, although both infants had gallbladders, the extrahepatic biliary trees were fused. After separation, obstructive jaundice developed in one twin and she required a biliary diversion. The other twin had an external biliary fistula that only closed after resection of a segment of liver with no connection to the ipsilateral biliary tree. Closure of the abdominal wall defects with biodegradable patches proved very satisfactory and eliminated the problems with persistent wound sinuses related to nonabsorbable mesh.

The failure rate of surgery for gastro-oesophageal reflux.

PURPOSE: The aim of this study was to document the presenting symptomatology and radiological findings of failed fundoplication, to determine the risk factors involved in recurrent gastro-oesophageal reflux, and to assess the results of revisional surgery. METHODS: Sixty-six patients requiring redo fundoplication during the past 15 years were studied. Sixty children had their initial fundoplication performed at our institution. RESULTS: The median time from initial fundoplication to the diagnosis of failure was 1.5 years. The main presenting symptoms were severe retching (n = 33), recurrent vomiting and aspiration (n = 26) and intolerable gas bloat (n = 7). Severe dysphagia occurred in two children with tight Nissen fundoplications. Contrast radiographic studies demonstrated fundoplication failure and correlated with the operative findings in 85% of cases. Risk factors for failure comprised hypertonic cerebral palsy, severe learning difficulties, oesophageal atresia, CHARGE syndrome, and tracheomalacia. The cause for the fundoplication failure included herniation of the fundoplication into the posterior mediastinum (failed crural repair, n = 30), disruption of the wrap (n = 10), combination of herniation and disruption (n = 22), and a tight wrap (n = 4). After revisional surgery, 13 children had persisting symptoms, five of whom underwent a third antireflux procedure. CONCLUSIONS: The predominant cause of fundoplication failure is herniation into the posterior mediastinum, which occurred most frequently in children with hypertonic cerebral palsy. This may be prevented by meticulous attention to accurate apposition of the crura at initial procedure. Redo fundoplication failed to resolve the preoperative symptoms in 20%, with five children requiring a third fundoplication.

Problems with intestinal lengthening.

The surgical problems encountered in treating three infants with short-bowel syndrome are reported. The two survivors demonstrate many of the problems associated with the very short-bowel syndrome and both are dependent on parenteral nutrition despite undergoing intestinal lengthening.

Primary anastomosis in apple-peel bowel syndrome.

During a 15-year period, 12 infants with apple-peel bowel were treated; the first 4 by initial enterostomy with delayed anastomosis and the remaining 8 by primary anastomosis. All four infants in the enterostomy group required parenteral nutrition, compared with only six of the eight who had a primary anastomosis. Seven of the eight infants treated by primary anastomosis were in hospital for less than 50 days, (however, the remaining child was an in-patient for over 2 years, but did suffer from short-bowel syndrome with only 17 cm of small intestine). In comparison, all four of the enterostomy group were hospitalised for between 70 and 175 days. All twelve patients survived.

Oesophageal atresia: at-risk groups for the 1990s.

The authors analyzed the outcome for 357 infants with oesophageal atresia and 15 with H-type tracheoesophageal fistula treated from 1980 through 1992. Survival according to Waterston risk categories was 99% for group A, 95% for group B, and 71% for group C. Presently, with optimal management, virtually all infants in groups A and B should survive. When examining the risk factors in the infants who died, two criteria were found to be important predictors of outcome: birth weight of less than 1,500 g and the presence of major congenital cardiac disease. A new classification for predicting outcome in oesophageal atresia is proposed: group I: birth weight > or = 1,500 g, without major cardiac disease, survival 97% (283 of 293); group II: birth weight < 1,500 g, or major cardiac disease, survival 59% (41 of 70); and group III: birth weight < 1,500 g, and major cardiac disease, survival 22% (2 of 9).

Effectiveness of fundoplication in early infancy.

PURPOSE: The aim of this study was to evaluate the results of fundoplication performed in the first 4 months of life. METHODS: Case-notes of 66 patients less than 4 months old who underwent fundoplication between January 1986 and July 1997 were reviewed. The mean length of follow-up was 24 months (range, 3 to 115 months). Diagnosis of gastrooesophageal reflux (GOR) was based on a barium meal in 55 patients or 24-hour pH monitoring in 23 patients. RESULTS: Fundoplication was performed at a median age of 9 weeks (range, 3 to 15 weeks). Associated anomalies were identified in 56 infants and included oesophageal atresia or tracheooesophageal fistula (n = 19), lung abnormalities (n = 7), congenital diaphragmatic hernia (n = 4), and various other complex syndromes including neurological disorders (n = 26). Isolated GOR occurred in 10. Ninety percent of infants with isolated GOR improved clinically after the fundoplication compared with 64% of those with associated anomalies. Only 58% of infants in whom GOR developed after oesophageal atresia repair improved after fundoplication. Vomiting was reduced after fundoplication in 76% of infants, apnoea and/or respiratory symptoms improved in 66%, whereas failure to thrive incidence improved in only 38% of infants. Revision of the fundoplication was required in 16 (24%) patients. There were eight deaths, all of which occurred at least 6 months after the fundoplication. CONCLUSIONS: Fundoplication in early infancy is unsuccessful in a high proportion of patients. The highest incidence of failure and redo fundoplication occurred in infants with associated anomalies. The best results were achieved in infants with emesis and respiratory symptoms.