John G. Raffensperger

Laparoscopic Swenson pull-through: A comparison with the open procedure

This study was performed to compare the standard open Swenson pull-through (OSP) with the laparoscopic Swenson pull-through (LSP) for Hirschsprungs disease. The Swenson pull-through was performed on eight patients with a rectosigmoid transition zone, during a 14-month period, using one camera port and three working ports. The results were compared with those of 10 patients with a similar lesion treated by the open procedure during an overlapping 19-month period. One laparoscopic procedure was converted to the open version because of technical difficulties. Both methods had a hand-sewn anastomosis approximately 1 cm above the pectinate line. The preoperative variables of age, weight, incidence of colostomy, and incidence of Downs syndrome were similar for the two groups. The operating time for LSP was similar to that for OSP (4 hours 42 minutes v 4 hours 37 minutes, respectively: P = NS). Postoperatively, the laparoscopic group had a shorter hospital stay (5.25 v 8.8 days; P < .05) and had a shorter period until the start of oral intake (2.75 v 5 days; P < .05). The requirement for narcotic pain medication was similar (12.6 v 12.8 doses; P = NS). Early postoperative complications were more common in the open group (3 wound infections, 1 prolonged ileus, and 1 anastomotic leak). No complications occurred in the laparoscopic group. Late postoperative follow-up was too short to compare functional results. The authors conclude that the Swenson pull-through can be performed safely with the laparoscope, with reduced morbidity.

Necrotizing enterocolitis and total parenteral nutrition-associated cholestasis

The proportion of patients with total parenteral nutrition (TPN)-associated cholestasis (TPN-AC) who have necrotizing enterocolitis (NEC) has increased markedly in the past ten years. Little is known about how these diseases affect each other. We retrospectively studied 24 patients with NEC and bowel necrosis or perforation who required surgical intervention. Patients were divided into two groups: those who had received TPN (NEC + TPN, n = 17) and those who had not (NEC, n = 7). As cholestasis was present clinically, or prolonged TPN was anticipated, liver biopsy was done. Bile acid levels were measured in both serum and bile in 13 patients. Six patients, who underwent bowel resection and enterostomy, had a second liver biopsy and measurement of bile acid levels at stoma closure. Our results showed that in 13 patients for whom bile acid levels were measured (NEC + TPN, n = 6) (NEC, n = 7), serum bile acid level was significantly elevated in both groups over normal for age. Biliary bile acid levels were correspondingly depressed in both groups suggesting a failure of bile acid transport. All patients had abnormal liver histology, but the pattern of injury differed between the two groups. Those in the NEC group had biliary stasis and mild hepatocyte degeneration. In contrast, 15 of 17 in the NEC + TPN group had advanced injury specific for TPN-AC. All six patients managed on TPN and partial enteral feeding before a second biopsy had no change in bile acid levels and progression of histologic injury. We conclude that NEC alone can cause functional cholestasis and histologic liver injury but does not cause the specific progressive damage caused by TPN. NEC may make the liver more susceptible to the effects of TPN. Partial enteral feeding does not halt or reverse this injury.

Hepatobiliary dysfunction during total parenteral nutrition is caused by infusate, not the route of administration

Cholestatic jaundice is the major complication of total parenteral nutrition (TPN). Both the intravenous (IV) route of nutrition and the enteral fast have been implicated as causes of TPN-associated cholestasis (TPN-AC). The purpose of this study was to determine whether TPN-AC is caused by the TPN solution itself or the IV route of administration and enteral fast. Prepubescent rabbits (n = 24) were divided into four groups: CONTROL, fed standard lab chow; TPN, received a standard hyperalimentation solution of dextrose, Aminosyn, and lipids via the jugular vein; ENT, received the same hyperalimentation solution via a duodenostomy tube; and OSM, received a polymeric formula (Osmolite) via a duodenostomy tube. After 14 days on these diets, we measured bile flow, bile acid excretion, sulfobromophthalein (BSP) excretion, plasma amino acid profile, serum liver enzymes, and liver histology. Statistical analysis was by analysis of variance. Hyperalimentation solution significantly depressed hepatobiliary function, whether it was given IV or by gut. Bile flow in both the TPN (36.4 microL/kg/min) and ENT (46.2) groups was significantly less than CONTROL (84.5) or OSM (62.9). Hepatic secretory function, measured by excretion of the cholephilic dye BSP, was depressed in both TPN and ENT (57% and 55% of IV dose excreted in bile over 60 minutes, respectively) compared with CONTROL (84%) or OSM (71%). Serum liver enzymes were normal in all groups. Histological injury similar to TPN-AC in humans (portal inflammation and hepatocyte degeneration) was seen in both groups receiving the hyperalimentation solution.(ABSTRACT TRUNCATED AT 250 WORDS)

The management of a cloaca

Abstract As a result of our work with isolated hydrometrocolpos as well as with cloacal lesions we feel that the urinary, genital, and gastrointestinal tracts must all be separated from one another in the newborn period. The consequences of temporizing operations is sepsis and urinary tract infection and declining renal function. We have described and illustrated an operation designed to accomplish this objective.

The feasibility of laparoscopic swenson pull-through

The Swenson abdominoperineal pull-through is the authors preferred treatment for children with Hirschsprungs disease. The present study was performed to determine whether this procedures principles could be applied to laparoscopic techniques. The operations were performed in 13 mongrel dogs--first to optimize techniques, and second to observe postoperative function in the surviving animals. The authors found that the dog is a good model in which to study this procedure, and it appears possible to perform a satisfactory Swenson pull-through with the laparoscope.

The effect of cholecystokinin-octapeptide on the hepatobiliary dysfunction caused by total parenteral nutrition

PURPOSEnPatients on total parenteral nutrition (TPN) commonly have hepatobiliary dysfunction. Interruption of the enterohepatic circulation (EHC) and gallbladder stasis are part of the pathogenesis. Cholecystokinin-octapeptide (CCK-OP), by emptying the gallbladder, stimulates the EHC. This study was performed to determine whether daily CCK-OP infusions can ameliorate the hepatobiliary dysfunction caused by TPN.nnnMETHODSnRabbits maintained on a standard TPN for 12 days were divided into two groups. One group (n = 6) received daily intravenous doses of CCK-OP, and the other (n = 13) received TPN only. A lab-chow-fed (LCF) group (n = 8) served as controls. The authors studied bile flow and bile acid secretion rates, sulfobromophthalein (BSP) secretion, gallbladder emptying in response to CCK-OP, and liver histology.nnnRESULTSnThe LCF group had a bile flow of 82.3 microL/kg/min; that for the TPN-only group was 45.7 microL/kg/min (P < .001). The daily CCK-OP group did not improve more than the TPN-only group, with a bile flow of 45.8 microL/kg/min (P = NS). Bile acid secretion was 0.64 mumol/kg/min for the LCF group, 0.46 for the TPN-only group (P = NS), and 0.46 for the daily CCK-OP group (P = NS). TPN impaired the ability of the gallbladder to empty, and this was restored with daily CCK-OP. In the LCF group, the mean BSP secretion was 81.7% of a 5-mg/kg bolus within 60 minutes, compared with 72.5% in the daily CCK-OP group (P = NS) and 63.5% in the TPN-only group (P < .01). Histological examination of the liver showed that daily CCK-OP produced less periportal inflammation and fibrosis, although all TPN groups had hepatocyte damage in the centrilobular area.nnnCONCLUSIONnStimulation of the EHC with daily CCK-OP infusions during TPN decreased periportal inflammation and fibrosis, maintained gallbladder emptying capacity, and improved organic anion (BSP) secretion, although bile flow and bile acid secretion were not improved, and hepatocyte damage persisted.

Fusiform Dilatation of the Common Bile Duct With Pancreatitis

Abstract We have seen two girls, with fusiform dilatation of the common bile duct. One child also had saccular dilatation of her intrahepatic bile ducts. Each child had suffered from recurrent bouts of abdominal pain and an episode of jaundice, and had an elevated serum amylase prior to operation. Unexplained pancreatitis in children should lead to investigation of the biliary tract. It may be wise to perform an operative choleangiogram on all patients with choledochal cysts to delineate the intrahepatic bile ducts, which may also be involved with saccular dilatation.

Surgical complications of Ehlers-Danlos syndrome in children

The Ehlers-Danlos syndrome is a special challenge to the surgeon because of its relative infrequency and the late onset of overt symptoms, such as hyperelastic skin. We should consider this diagnosis in every child with recurrent inguinal herniae and search for its stigmata. A child with a known Ehlers-Danlos syndrome who requires an elective operation should be carefully studied for coagulation disorders. If there is a history of past injuries or operations in which there was poor healing, the benefits of the proposed operation must be carefully weighed against possible complications. Operations must be carried out with great care to avoid tearing the skin and fascia. Meticulous attention is given to hemostasis to prevent postoperative bleeding or hematomas. Inguinal hernias in these children resemble those seen in elderly patients. The transversalis fascia is thin and the internal ring is hugely dilated. An adult-type repair, with the use of mesh or felt may result in a lower incidence of recurrence. In addition, the Ehlers-Danlos syndrome may be yet another etiology for obscure gastrointestinal bleeding in children. Specific enzyme defects have been identified in 2 of the 7 subtypes of this disease. Further, biochemical study of connective tissue defects may contribute to our knowledge of normal collagen synthesis and wound healing.

Plexiform neurofibromas in childhood

Abstract Sixteen cases of plexiform neurofibroma occurring in childhood have been discussed. Of lesions in the trunk or extremities, only two required multiple procedures, including a below-the-knee amputation. All patients are free of tumor at present, though they carry other stigmata of Von Recklinghausens disease. Of the seven children with cervicothoracic lesions, however, one is dead, two have significant neurologic deficit (severe quadriparesis, 70% arm weakness), and none is free of tumor. One has permanent tracheostomy and three have gross tumor extending to their vertebral column or skull without longtract signs as yet. Though histologically benign, plexiform neurofibroma of the neck and thorax in children must be considered a progressive crippling disease that is extremely difficult to control surgically and carries a grave prognosis.

A long-term follow-up of three patients with biliary atresia

This report reviews the courses of three children who underwent surgery for biliary atresia. They include a patient who is now 25 years of age who underwent correction of an operable biliary atresia in the neonatal period. This patient is alive and well without jaundice. The second is a 21-year-old woman who had a Kasai operation, also performed in the neonatal period; she is well and attending school but does have an elevated bilirubin and signs of liver dysfunction. The third patient, who underwent surgery performed by Dr Willis Potts in 1963 for what appeared to be inoperable biliary atresia, finally underwent a portal duodenostomy after her third operation. She had satisfactory bile drainage but had numerous bouts of cholangitis early in her life. She survived until she had several major episodes of gastrointestinal hemorrhage from varices and finally died after a portal systemic shunt. These three patients illustrate that prolonged survival is possible after a variety of operations performed for biliary atresia. Furthermore, even though these patients were severely jaundiced early on in life and at least two of them had complicated postoperative courses, they are perfectly normal from an intellectual standpoint and have functioned in a normal fashion.