Spontaneous biliary perforation presenting as gastric outlet obstruction

Spontaneous biliary perforation (SBP) is a rare, surgically correctable cause of jaundice in neonates. The presenting feature is usually biliary ascites, and in rare cases, biliary peritonitis. This article reports a case of SBP, which presented with features of gastric outlet obstruction, leading to an erroneous preoperative diagnosis. Most probably this is the first report of such an unusual presentation of SBP. The child underwent exploratory laparotomy and a bilio-enteric bypass with drainage of the right subhepatic space, which led to a prompt resolution of the symptoms     

Porcelain gallbladder with extrahepatic bile duct obstruction in a child

An extrahepatic bile duct obstruction was diagnosed in a 13-year-old boy presenting with pruritus, abdominal pain and jaundice. Several weeks after sphincterotomy and biliary stenting via endoscopic retrograde cholangiopancreaticography which relieved the obstruction, the patient was operated on. Severe fibrosis encased the extrahepatic biliary tract, so only cholecystectomy was performed because planned hepaticojejunoanastomosis could jeopardize the vascular supply to the liver. Histopathology showed calcification of the gallbladder wall and chronic fibroproliferative changes in the surrounding tissue. The stricture of extrahepatic biliary duct resolved after 3 years of repeated replacement of stents. The stenting was thereafter terminated. In the following 3 years no dilation of intrahepatic bile ducts and no laboratory signs of cholestasis recurred and the now 19-year-old boy is doing well. Neither a case of porcelain gallbladder with extrahepatic bile duct obstruction in a child nor a successful treatment of the obstruction by long-term stenting has been described in the literature yet.     

Patchplasty with expanded polytetrafluoroethylene and skinplasty for the closure of large congenital abdominal wall defects

A combined technique of patchplasty with expanded polytetrafluoroethylene (PTFE) and a special skinplasty is described for congenital abdominal wall defects where the abdominal cavity needs enlargement but skin closure is primarily possible. The patchplasty consists of suturing two half-moon-shaped, expanded PTFE leaves to the sides of the muscular abdominal wall, then approximating them under observation of intra-abdominal pressure by monitoring five parameters (urinary bladder pressure, central venous pressure, transcutaneous oxygen saturation, ventilation pressure, and mean arterial pressure). The skin is incised in a horizontal manner according to the tension lines. However, after closure, regional necrosis of the skin in the mid-portion above the incision is often observed due to lack of sufficient subcutaneous tissue at this site. Therefore, a skinplasty is done as a preventive measure, resecting this potential site of necrosis. The final appearance of the skin is an inverted T-shape. The surgical and monitoring techniques are described and illustrated. Correspondence to: A. Rokitansky     

Risk factors for abdominal wall defects

In the last decades, the prevalence of gastroschisis (GS) has increased worldwide. The purpose of this study was to identify maternal risk factors explaining the described gain and to identify differences between GS and omphalocele (OC). A case-control design was used to compare GS (n = 36) and OC (n = 18) mothers to control group (CG; n = 30) matched for maternal age. Specialized questionnaires and mothers' prenatal records were used, and participants completed a structured interview. Focus was on medical history, changing nutrition, drug consumption, and external risk factors. The local ethics committee approved this study. GS mothers were significantly younger (mean 23.00; median 24; SD ±5) than OC (P = 0.007; mean 28.61; median 28, 19-41; SD ±5.1) and CG (P = 0.001; mean 30.77; median 31, SD ±6.2). Mothers with abdominal wall defects (AWD) ingested antibiotics more often (P = 0.008) than CG. Socioeconomic characteristics, for example, level of profession, of GS mothers was significantly lower than OC (P = 0.039) and CG (P = 0.05) mothers, and their cohabitation time was shorter (P < 0.05; mean 35 month/median 24 month, SD ±35.8). Incidence of OC significantly increased after hormonal treatment (P = 0.022) and invasive prenatal diagnostics (P < 0.05) compared to GS. GS mothers took folic acid prophylaxis less often than OC (P = 0.02). Smoking, illicit drugs, and external risks like herbicides showed no influence, but GS mothers drink significantly more often alcohol (P = 0.05). We confirmed an increased risk for GS if several factors such as young maternal age, short cohabitation time, and usage of antibiotics coincide with alcohol consumption and associated immune diseases. OC increased after hormonal treatment and invasive prenatal diagnostics.     

Lower abdominal wall flap for closure of large diaphragmatic defects

A method of closure of diaphragmatic herniae with large defects is described in two children using a muscle flap of internal oblique and transversus abdominus from the inferior aspect of the wound. The advantages are that the flap is large, easily accessible, autologous, assists in resultant wound closure, and requires no separate incision. It differs from previously described flaps as it is taken from below the subcostal wound and is not limited in size or length by the costal margin. Correspondence to: W. D. A. Ford     

Myocutaneous sartorius flap for major congenital abdominal wall defects

Management of giant omphalocele/gastroschisis remains unsatisfactory: primary fascial closure is not always possible, prosthetic applications lead to problems of infection and separation, and escharification or skin closure results in ventral hernias. We have therefore investigated the possible use of myocutaneous flaps in such situations. The myocutaneous flap, comprising a skin pedicle isolated along with the underlying muscle, has two major attractions: (1) the good blood supply minimises risks of infection and flap separation; and (2) the muscle pedicle provides a strong fascial-muscular closure. The flap has to fulfill four criteria: (1) it is viable; (2) it provides adequate coverage; (3) it reaches the defect; and (4) its transposition does not result in functional disability of the organ from which it originates. Based on an anatomical study of 20 post-mortem specimens in eight neonates and two adults, including contrast injections, we have found the sartorius musculocutaneous flap to be the ideal solution. The sartorius muscle is relatively more bulky in neonates than in adults. The major blood supply, which enters between its upper third and lower two thirds, can maintain a 7 × 3-cm flap. With the hips immobilised in flexion, the flap can reach the xiphisternum without tension. Our study suggests that it is feasible to repair neonatal abdominal wall defects of up to 7 × 6 cm by sartorius musculocutaneous flaps. In particular, this approach appears ideally suited for reconstruction in cloacal exstrophy and for secondary repair of ventral hernias resulting from escharification, skin closure alone, or failed prosthetic applications in giant omphalocele/gastroschisis.Offprint requests to: P. K. H. Tam     

Acquired disorders of the biliary tract in children

Acquired diseases of the biliary tract are less common in children than in adults. Gallstones, sclerosing cholangitis and biliary complications after liver transplantation are the most frequent problems but biliary obstruction can be secondary to pancreatitis, tumours and biliary sludge and bile leaks are a recognized risk following trauma to the liver. Biliary disease may be asymptomatic and suspected due to abnormal liver function tests or ultrasound scan. Right sided abdominal pain, features of biliary obstruction or sepsis occur in symptomatic children. Identification and relief of biliary obstruction requires a collaborative approach involving paediatric hepatologist, surgeon and radiologist. In those with long standing cholestasis, as can occur in sclerosing cholangitis, supportive management of itching and nutrition improves quality of life. The outcome following liver transplantation for those progressing to end stage liver disease is very good.     

Anterior abdominal wall closure in bladder exstrophy

Anterior abdominal wall closure without pelvic osteotomy for bladder exstrophy can be very diffecult and more often than not calls for various reconstructive measures. A technique is described that involves bilateral detachment of the rectus abdominis from the pubic ramus, midline vertical closure, and refixation to the pubic rami after medial and caudal advancement. A relaxing fascial incision may be required. This technique has been used in 15 patients and has obviated the need for pelvic osteotomy, thereby decreasing the postoperative morbidity. It has resulted in good cosmetic repair in all patients in addition to the ease of abdominal wall closure. The healing has been remarkably good. This technique is particularly useful in children coming for primary surgery after the neonatal period. Correspondence to: V. Bhatnagar     

Spontaneous resolution of extrahepatic biliary obstruction in a neonate

A patient with spontaneous resolution of complete extrahepatic biliary obstruction shortly after the neonatal period is described. Liver biopsy prior to resolution revealed widened portal tracts and extensive fibrosis; these changes normalized following resolution. The aetiology was not established but possibilities included a choledochal cyst, localized inflammation; for example, pancreatitis, a calculus of the common bile duct or a congenital membrane. Management of this patient would have been advanced if there were a paediatric size side-viewing duodenoscope which would have diagnostic and therapeutic potentials.     

Muscular flaps for closure of giant abdominal wall defects

In this article we describe the use of muscle flaps in closing giant abdominal wall defects. The technique used to take the flaps from the anterior rectum, internal and external oblique muscles are shown. We emphasize the advantages of using this technique, which avoids the use of synthetic materials, such as Teflon and Marlex mesh. Correspondence to: Antonio F. Gallardo Meza     

Traumatic abdominal wall hernia: an unusual bicycle handlebar injury

Traumatic hernia of the abdominal wall is rare. The mechanism of injury can be severe such as a motor vehicle accident or relatively minor such as a fall onto a bicycle handlebar. In children traumatic abdominal wall hernia can occur even after relatively minor trauma and these cases typically have no associated intra-abdominal injury. We report a traumatic hernia of the anterior abdominal wall in a 7-year-old boy following a fall from a bicycle. A review of the literature revealed only nine similar reports. The presence of localised pain, bruising, and a reducible swelling or a cough impulse suggests the diagnosis. Primary repair with prosthetic material is the preferred treatment.     

Are abdominal wall defects and external genitalia anomalies randomly expressed in some families?

Familial cases of isolated abdominal wall defects with variable expressivity in more than one generation have rarely been observed. We report four affected individuals within a small three‐generation family with either variable non‐syndromic abdominal wall defects or external genital anomalies. We discuss the possible transmission of non‐syndromic abdominal wall defects. It could be hypothesized that similar developmental defects may result in anomalies like hypospadias in males or developmental anomalies of the labia minora or labia majora in females.     

End-results of experimental gastroschisis created by abdominal wall versus umbilical cord defect

An experimental study was conducted to determine the end-results of two different defects on the anterior abdominal wall: an abdominal wall defect (AWD) versus an umbilical cord defect (UCD) using chick embryos. The AWD was created by leaving an intact skin bridge between the defect and the umbilical cord in group l; the UCD was created on the umbilical cord near the junction of the skin in group 2. At the end of incubation, the intestines appeared hemorrhagic in the AWD group, but not in the UCD group. During microscopic examination, hemorrhagic areas were observed in the bowel wall and mucosal villi in the AWD group but not in the UCD group. The end-result of the defect causing the physiological umbilical hernia resulted in bowel damage resembling the classic picture of gastroschisis (GS). We conclude that the site of the defect in GS is not the abdominal wall itself, but the physiological umbilical hernia.     

Isolated hypoplasia of abdominal wall muscles associated with fetal ascites

We report the case of an infant born after parvovirus B19‐induced fetal hydrops, who presented at birth with bilateral abdominal wall laxity, which was more evident on the flanks. Imaging exams revealed congenital hypoplasia of oblique abdominal muscles not associated with other anatomical abnormalities except for small liver calcifications. We review the medical literature and identify similar cases associated with fetal ascites. We propose that isolated hypoplasia of abdominal wall muscles can be associated with fetal ascites from various causes, and represents a separate condition from prune belly syndrome.     

Prenatal Closure of Abdominal Defect in Gastroschisis: Case Report and Review of the Literature

With the routine use of fetal imaging studies during prenatal care, increased numbers of unusual intrauterine events are now detected. Prenatal closure of the abdominal defect in gastroschisis is an example. We report a 34 5/7–week stillborn who had prenatal closure of a ventral abdominal wall defect, which had been seen earlier on fetal ultrasound examination. Two ultrasound examinations performed at 15 1/7 weeks and 19 1/7 weeks showed a mass of exteriorized bowel that herniated through the abdominal defect, just to the right of the umbilical cord. At 30 1/7 weeks, no exteriorized bowel was seen, but thickened and dilated intraabdominal bowel was identified. No abdominal defect or exteriorized bowel was found at autopsy. There was a severely dilated proximal jejunum with the absence of the rest of the small intestine and the right side of the colon. The remaining left side of the colon was small and blind proximally. Six similar isolated examples have been reported since 1991. Prenatal closure of an abdominal defect was associated with long-segment atresia of the midintestine in each case. We believe that the spontaneous closure of this abdominal defect was associated with atresia and resorption of exteriorized bowel. It is likely some of the cases of long-segment atresia may in fact be associated with closed gastroschisis. Received December 18, 2000; accepted April 25, 2001.     

A comparison of prosthetic materials used to repair abdominal wall defects

Large abdominal wall defects may require a prosthesis for closure. The aim of our study was to identify the best material for abdominoplasty in pediatric patients. One hundred twenty-eight Wistar KY strain male rats (3 weeks old) were used. All animals underwent celiotomy via a midline skin incision. They were divided into seven groups as follows: the animals in groups 1 through 6 underwent full-thickness abdominal wall excision 3 cm in diameter. The animals in group 1 underwent primary closure. In groups 2 through 6 the defect was closed with prosthetic material. In Group 7, a sham operation was performed. Daily weights were measured. The animals were killed after 3 and 9 weeks. Adhesion scores were assigned for each group. Vicryl mesh resulted in the fewest adhesions and had no effect on weight gain in the developing rats. Accepted: 11 June 1997     

A simple and inexpensive technique of upper abdominal wall retraction in pediatric liver surgery and transplantation

Abstract:  In majority of centers, pediatric liver surgery and transplantation involves a team of four at any given time: the surgeon, the first and second assistants, and the instrument nurse. This creates considerable crowding around both operative field and operating table. Mechanical devices have been occasionally employed to solve this problem, but most table-mounted devices are designed for adult patients. Based on our experience with pediatric living donor liver transplantation, we developed a simple, safe, and inexpensive method of upper abdominal wall retraction to facilitate surgical exposure and avoid over-crowding in the sterile field. The key points of this technique are the use of the Mercedes incision for liver transplantation or right subcostal incision with upper abdominal midline extension for hepatic resection and an adult-designed Kent retractor. A pediatric-designed Kent retractor is expensive, unnecessary, and may even cause complications as rib fractures and nerve paralysis. We used this technique in 142 consecutive pediatric living donor liver transplants and 16 major hepatectomies in children without any complication resulting from the exposure. The presented technique is simple, safe, reliable, and inexpensive. It can be used in pediatric liver surgery, as well as general pediatric upper abdominal operations.     

Long-term results following repair of neonatal abdominal wall defects with Gore-Tex

Animal studies and clinical experience in adults suggest that the Gore-Tex patch, because of its strength and lack of reactivity, is superior to other synthetic materials as a fascial replacement. We report our experience with Gore-Tex for the repair of neonatal abdominal wall defects when direct fascial closure was not possible. Between January 1985 and July 1992, 84 patients underwent repair of an omphalocele or gastroschisis. Ten of these were repaired with a Gore-Tex patch. Follow-up averaged 2.2 years (range 13–63 months). Data collected were graft life (time from insertion to removal), patch-related complications such as exteriorization (exposure of the patch due to dehiscence of the overlying skin), presence or absence of local and/or systemic infection, and patch separation (separation of the sutured edge of the patch from the fascia). The ease of removal as well as the appearance of the wound after patch removal was documented. Patch-related complications were divided into early (30 days postoperatively), and late (> 30 days). All ten patients required graft removal because of patch-related complications. The median time to removal was 90 days (range 20–540). There were a total of 12 complications in the ten patients. Three had early complications; three had exteriorized patches and two had concomitant local infections. Seven patients had late complications. Exteriorization was less common in this group, occurring in two cases. Local infections occurred in five of seven patients. Patch removal was necessary to clear the infection in all cases. No patient developed systemic sepsis or enteric fistulae. The patch was removed easily and there were no bowel-to-patch adhesions. Fascial closure was successfully accomplished in all patients regardless of the size of the initial defect. In no case did removal of the patch and secondary closure of the abdominal wall result in a ventral hernia. This study demonstrates that: (1) Gore-Tex patch closure of neonatal abdominal wall defects is associated in all cases with either early or late exteriorization and/or infection; (2) removal is easy and complication-free because it does not incorporate into the tissue and is relatively non-reactive; and (3) following removal of the patch it is possible to successfully close the abdominal wall defect. We conclude that Gore-Tex is a useful synthetic material for closure of neonatal abdominal wall defects, but should be considered a temporary bridge to subsequent fascial closure.     

Definitive abdominal wall closure using a porcine intestinal submucosa biodegradable membrane in pediatric transplantation

Abstract: Abdominal closure in children less than 10 kg following liver or kidney transplantation can be challenging. Excessive pressure attained from a tight closure can result in abdominal compartment syndrome, graft compromise and loss. Concerns over using prosthetic patches are that of infection and dehiscence. We report a series of definitive abdominal wall closure using a biodegradable membrane from porcine intestinal submucosa (Surgisis^®; Cook Biotech Incorporated, West Lafayette, IN, USA). A prospective collection and follow up of liver and kidney transplant patients weighing less than 10 kg who required abdominal wall augmentation with Surgisis^® in order to achieve satisfactory closure. There were 10 liver and two renal transplant patients. The average weight of the liver transplant patients was 6.6 kg (5.4–8.5 kg) and the renal 9.8 kg. The average area of Surgisis^® used was 71.2 cm² (25–160 cm²) and length of follow up was 15.3 months (1–27 months). Concomitant measures to aid abdominal closure included bilateral recipient nephrectomy for the renal patients and reduction by 33% of the lateral segmental grafts in two liver transplant patients. Delayed closure occurred in all patients except one and the average days to closure from the first surgery was 3.75 days (0–6 days). Following liver transplantation one patient died from multiple organ failure at one month secondary to hemophagocytosis from underlying combined immune deficiency syndrome and one patient with hepatic artery thrombosis was salvaged at re‐exploration. There were two wound complications, one patient developed two small sinuses and some skin dehiscence which healed over four months and the second developed a skin sinus following trans‐patch liver biopsy which healed in three wk. Both had positive microbial cultures but neither necessitated removal of the graft. There were no incisional hernias. Surgisis^® is a safe method for facilitating abdominal closure in pediatric transplant patients. It appears to have long‐term durability with no incisional hernias on short‐ and medium‐term follow up, and is fairly resistant to infection.