Perineal skin bridge and levator muscle preservation in neutral sagittal anorectoplasty (NSARP) for vestibular fistula.

The incision in limited posterior sagittal anorectoplasty for vestibular fistula (VF) extends from the coccyx to the fistula with the rectum identified by partial sagittal division of the levator muscle. In anterior sagittal anorectoplasty, the perineal incision extends from the fistula opening to the posterior margin of the external sphincter complex while preserving intact the levator muscle. We describe a modification of the operation for repair of VF, the neutral sagittal anorectoplasty (NSARP), which preserves both a perineal skin bridge between the neo-anus and the posterior fourchette and the levator muscle. Leaving the perineal skin bridge and the levator muscle intact could be important both from the aspects of perineal wound-healing and functional outcome. The skin incision in NSARP extends from the coccyx to the anterior limit of the external sphincter muscle complex as defined by muscle stimulation. An artery forceps passed through the VF facilitates the location and exposure of the rectum. The levator muscle is left intact. The dissection and closure of the VF is done entirely from within the opened rectum. There is an intact perineal skin bridge between the neo-anus and the posterior fourchette at the completion of the NSARP. In 12 consecutive patients with VF, NSARP prevented the risk of wound complications occurring between the neo-anus and the posterior fourchette. A diverting colostomy was safely avoided in our last five patients. NSARP does not involve any division of the levator muscle and it also retains an undisturbed wad of tissue that could function as the perineal body. All five patients who are 3 years or older demonstrate voluntary bowel movements. Preserving the perineal skin bridge and the levator muscle in NSARP have contributed to the improvement of aesthetic appearance of the perineum and faecal continence in our patients.

The management of combined oesophageal and duodenal atresia.

Duodenal atresia (DA) in babies with oesophageal atresia (OA) is associated with significant morbidity and mortality. The management protocol for this combination of anomalies is not well defined and evolving. The aim of this study was to review our experience with combined OA and DA and to note the evolving trend in management at Sydney Children's Hospital. Over the last 30 years, 225 babies with OA have been treated at our institution. Ten babies had associated DA. A total of 19 anomalies were noted; one child had multiple lethal anomalies and received no treatment. In three babies the diagnosis of associated DA was missed initially. For the nine babies who were treated, four had a primary OA repair followed by a delayed DA repair; one had cervical oesophagostomy, gastrostomy and DA repair; and two had a simultaneous repair of OA and DA with a gastrostomy. The last two patients in this series have had a primary simultaneous repair of both anomalies without a gastrostomy. The complications noted in the nine patients treated included anastomotic strictures in five, recurrent tracheo-oesophageal fistula in one, food bolus obstruction in one, and a megaduodenum requiring tapering in one. We believe that adopting a management protocol of primary simultaneous repair of both anomalies without a gastrostomy is justified. There was no apparent increase in morbidity and mortality with such an approach.

The radiologist says malrotation: does the surgeon operate?

The management of malrotation when it is an incidental finding is unclear. This retrospective study reports an analysis of radiological and operative findings in a series of 71 patients. There were no deaths. We report a false-positive rate of 15% for upper gastrointestinal contrast studies reported as showing malrotation. Our findings and a review of the literature demonstrate that in the asymptomatic child over 2 years of age, the evidence supporting mandatory correction of malrotation is weak.

Cisapride and caesarean section: their role in babies with gastroschisis.

OBJECTIVE: The objective of this study was to compare the neonatal postoperative course and morbidity for patients with gastroschisis who received cisapride with those who did not receive cisapride. STUDY DESIGN: Data were obtained by review of the medical records of all the patients with gastroschisis who were admitted to Sydney Children's Hospital between January 1984 and December 1995. Data were compared between 15 babies who received cisapride with 27 who did not. The mode of delivery and outcome of babies in whom gastroschisis was diagnosed antenatally was compared with those who were diagnosed at birth. RESULTS: Duration to the commencement of feeds, attainment of full feeds and the length of hospital stay were not statistically different between these two groups, with or without cisapride (p = > or = 0.1). There were more elective Caesarean sections in the antenatally diagnosed group compared to those detected at birth and the outcome of these two groups showed no statistically significant difference. CONCLUSIONS: Our study identified no benefit from cisapride therapy in babies with gastroschisis and also there was no benefit from elective Caesarean section for babies with antenatal diagnosis of gastroschisis.

Congenital spigelian hernia with cryptorchidism.

Congenital spigelian hernia (SH) is a rarity. The authors present two cases of congenital SH with undescended testis, and examine the significance of the concurrence of these events.

Gastroschisis and exomphalos: recent trends and factors influencing survival.

The present study examined the treatment and survival of patients with gastroschisis and exomphalos in the 5-year period January 1982-December 1987, at the Prince of Wales Children's Hospital. There were 15 cases of gastroschisis and 17 cases of exomphalos. The influences of temperature on arrival, birthweight, method of repair and associated anomalies on survival were examined. More patients presenting with gastroschisis survived than those with exomphalos (14 of 15 compared with 10 of 17, respectively). Of all the factors examined, the presence and nature of associated anomalies is the most important in determining survival.

Prophylactic colectomy in Ehlers-Danlos syndrome with colonic ectasia.

An adolescent boy with clinical features of Ehlers-Danlos syndrome developed ectasia of the colon leading to intractable constipation and clinically imminent colonic perforation. Total colectomy with ileorectal anastomosis in two stages was performed eliminating not only his intractable constipation but also the risk of potentially lethal perforation. This case illustrates an approach that may reduce the high mortality rate reported in patients with Ehlers-Danlos syndrome and colonic perforation.

Model of TPN-associated hepatobiliary dysfunction in the young pig.

Gallbladder 'sludge' and cholestasis are two common complications associated with total parenteral nutrition (TPN), but the aetiology of each is uncertain. An animal model has been developed in the young pig which demonstrates these two complications. Five female piglets, of Landrace Large White Cross variety weighing 4.5-5.9 kg, received nutritional support for 2 weeks with a continuous infusion of TPN solution at a dose of 150 kcal kg-1 day-1. The solution was 35 per cent dextrose, 5 per cent L-amino acids with conventional electrolyte, mineral and vitamin additives. No lipid was used in the solution. Five weight-matched animals were used as controls. All animals in the TPN group developed 'sludge' in their gallbladders, decreased basal bile flow, decreased bile salt excretion and a diminished response to bile salt stimulated bile flow, as compared with controls. There was no abnormality in routine liver function tests or liver histology. It is concluded that TPN therapy in this animal model is associated with the appearance of gallbladder 'sludge', and cholestasis as demonstrated by direct bile flow studies. It is suggested that this bile flow abnormality is due to a decrease in bile salt dependent and bile salt independent fractions of canalicular bile flow. The model provides the opportunity to investigate TPN related hepatobiliary dysfunction in an animal that has similar liver function to man and comparable nutritional requirements.

Effect of haemorrhage on hepatic glutathione concentration: an experimental study in the pig.

Depletion in the hepatic concentration of reduced glutathione (GSH) may potentiate liver injury resulting from toxic metabolites. Patients who have had severe haemorrhage frequently develop hepatic dysfunction and we have shown that the hepatic GSH level is decreased in these patients. The present study has examined the time-course effect of a 30% haemorrhage on the hepatic GSH level by sequential biopsies of the liver. Acute and chronic experiments were performed and, in both, the hepatic concentration of GSH fell during the first 6 h after haemorrhage; this fall was followed by a significant rebound elevation at 24 h. In the chronic haemorrhage experiment the hepatic GSH level was normal at 1 week after haemorrhage. Thus, the susceptibility of the liver to toxic metabolite injury after haemorrhage persists for less than 24 h in this experimental model and haemorrhage appears to have no long term effect on the hepatic GSH concentration.

Pancreatic abscesses.

This paper presents the clinical features and problems in the management of 34 patients with pancreatic abscesses. In the majority of patients the abscesses developed following an attack of pancreatitis due to alcohol or gallstones. The abscesses were usually multilocular, and often had spread widely in the retroperitoneal space. Invasion into surrounding viscera or the peritoneal cavity occurred in 12 instances, and eight patients developed major bleeding into the abscess cavity. Obstructive complications (affecting bowel, common bile duct and large veins) occurred in eight patients. Twelve of the 34 patients (35 per cent) died, most deaths being due to failure to control sepsis (seven patients) or to massive bleeding from the abscess cavity (three patients). The mortality of this condition is likely to remain high, but may be reduced by better drainage techniques at the initial exploration. The importance of the infra-mesocolic approach for drainage is emphasized.

Cholescintigraphy in acute cholecystitis: use of intravenous morphine.

Conventional cholescintigraphy (60 patients) and a modified protocol (59 patients) were compared in 74 females and 45 males with acute cholecystitis. In the modified protocol, intravenous morphine (0.04 mg/kg) was administered whenever the gallbladder was not seen 40 minutes after injection of Tc-99m-pyridoxylideneglutamate (36/59). Accuracy was 98% with morphine, compared with 88% for the conventional protocol; specificity improved from 83% to 100% with no loss of sensitivity (96% in both groups). Low doses of morphine are well tolerated and can result in a highly accurate diagnosis of acute cholecystitis without the need for delayed imaging.

Factors influencing hepatic glutathione concentrations: a study in surgical patients.

1. Hepatic total glutathione (reduced plus oxidized) and oxidized glutathione levels were measured in operative wedge liver biopsy specimens obtained from 70 patients. 2. The effects on hepatic reduced glutathione (GSH) level of four potential risk factors (abnormal liver histology, abnormal preoperative tests fo liver function, drug ingestion and protein malnutrition) were examined. 3. Each of the four risk factors was associated with a significant reduction in hepatic GSH. Multivariate analysis indicated that only three of these risk factors (abnormal liver histology, drug ingestion and protein malnutrition) had independent effects in reducing hepatic GSH. 4. Twenty-five of the 70 patients studied did not have any of the four risk factors. The mean hepatic GSH level in these patients was 3.92 mumol/g of liver wet weight (SD 0.62), giving a 95% reference range of 2.71-5.14 mumol/g of liver net weight.

Possible association of angiosarcoma with oral contraceptive agents.

Angiosarcoma of the liver has been reported to be associated with the long-term use of androgenic-anabolic and oestrogenic steroids. It has been suggested that this tumour might develop in women after long-term exposure to oral contraceptive steroids, although this association has not yet been reported. We present here the clinical and pathological findings in a 42-year-old woman who died of hepatic angiosarcoma after taking oral contraceptive steroids for 10 years.

PIP: HAS (hepatic angiosarcoma) has been associated with exposure to vinyl chloride, "Thorotrast," radium inorganic arsenic and androgenic-anabolic steroids. This case reports a possible association between HAS and oral contraceptive steroids. A 42-year old patient presented with a 4-month history of epigastric fullness and symptoms of esophageal reflux. A large epigastric mass from the left lobe of the liver was revealed at physicial examination. The patient had been taking oral contraceptives for 10 years but discontinued its use the year before after a hysterectomy for uterine fibroids. She consumed 10 g/week of alcohol, and smoked 10 cigarettes a day. There was no previous history of liver disease. A liver scintigram, ultrasonography, and selective hepatic arteriography revealed an avascular mass in the left lobe of the liver. Laparotomy was performed, revealing a huge, partly cystic and irregular mass in the left lobe of the liver, adhering to the stomach and transverse colon. Multiple biopsies showed the mass to be largely necrotic, and features of the viable portions were highly suggestive of HAS. She died 3 1/2 weeks after the procedure. At autopsy, histological examination confirmed the diagnosis of HAS, as well as the metastatic deposits in the diaphragm, small bowels, pancreas, adrenal, lung and pleural cavities. Although it is not known whether oral contraceptive use is definitely related to the development of liver tumor in this patient, there has been evidence suggesting that oral contraceptive steroids may induce the tumor-precursor stage observed after exposure to agents which are accepted as causing HAS. If an association between oral contraceptive use and HAS is established, it will confirm the hypothesis of Falk et. al. that certain environmental agents produce a tumor-precursor lesion which can develop into adenoma, hepatocellular carcinoma, or HAS.

Benign biliary strictures associated with chronic pancreatitis and gallstones.

A series of twenty-seven patients with benign non-traumatic biliary strictures is presented. Fifteen strictures were associated with chronic pancreatitis, and were typically long, tapered strictures of the intrapancreatic portion of the common bile duct. Twelve were associated with choledocholithiasis; these were usually short-segment and occurred both above and below stones in the duct. These strictures are important surgically, since they may produce pain, cholestasis and cholangitis, stone formation, and biliary cirrhosis. They may be difficult to distinguish from bile duct or pancreatic carcinomas. In the patients with chronic pancreatitis, treatment by biliary bypass was effective in the presence of cholestasis, but was ineffective for chronic pain. Treatment by biliary bypass or sphincteroplasty was highly effective in those patients with choledocholithiasis.

Traumatic haemobilia treated by hepatic artery ligation.

A 22-year-old woman developed haemobilia following suture of an apparently superficial liver injury. The diagnosis was made on the basis of the clinical features, duodenoscopy, and liver function test. Selective hepatic angiography and liver scintiscan demonstrated a cavity in the right lobe on the liver, with the bleeding arising from a branch of the right hepatic artery. She was treated by cholescystectomy, exploration of the common bile duct with removal of clots, and ligation of the right hepatic artery. Subsequent T tube cholangiography and liver scintiscans demonstrated complete resolution of the cavity, and there was no further bleeding. Experience with this patient suggests that contrary to previous recommendations, haemobilia associated with an intrahepatic cavity may be successfully treated by hepatic artery ligation.