Lipoid pneumonia secondary to Vaseline use in a patient with tympanic membrane perforation.

Exogenous lipoid pneumonia is a rare condition caused by aspiration of mineral, vegetable or animal oils. The aspiration of mineral oil is the most common cause of lipoid pneumonia in children. We present a 27-year-old man with a lipoid pneumonia with a history of daily use of Vaseline applied to cotton balls for ear plugging before swimming and shower.

A philosophical approach to conjoined twins.

There are increasing numbers of attempts to surgically separate conjoined twins. Almost every type of conjoined twins have been separated, with varying results. Surgeons must often make the decision as to the desirability and feasibility of separation. These decisions are complicated by ethical problems that involve patient privacy, the allocation of shared organs, and in some instances the necessity for one twin to die to save the other. Although life as a conjoined twin would appear to be intolerable, there are historical as well as current instances of conjoined twins who have progressed to adulthood as relatively well-adjusted individuals. Thus, in some situations, it may be better not to operate on these patients. At one time or another, when individual cases have been studied by theologians, all faiths have agreed that the sacrifice of one twin to save the other is ethical. When one twin is clearly stronger than the other and has the best chance for long-term survival, it also appears to be desirable to give that twin the shared organs that allow gastrointestinal and genitourinary function and ambulation. It is now possible to predict the pattern of shared organs in most cases based on an external examination. It is no longer necessary to subject these patients to prolonged, invasive tests, which may be dangerous and provide at best incomplete information. The surgeon should take great care to protect his patients' privacy and resist efforts by the media to create a "television circus"" over these patients.

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 Hirschsprung's 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 Down's 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.

Intestinal bypass of the esophagus.

Esophageal replacement by a segment of isoperistaltic ileum with cecum or by transverse or left colon will allow near-normal swallowing for many years. The authors reviewed the course of 59 children who had bypass of their entire esophagus and of four whose distal esophagus was resected and replaced. The follow-up period ranges from 1 to 37 years; in 36 cases, it exceeds 5 years. Thirty children had caustic strictures and 25 had either isolated esophageal atresia or atresia with fistula. Two children with esophageal injury caused by foreign body ingestion and two with congenital strictures also required complete bypass. Four patients required resection and replacement of the distal esophagus only; two had acquired strictures from gastroesophageal reflux, one had varices, and one had a teratoma involving the esophagus. A retrosternal isoperistaltic ileocolic segment is our preference for complete esophageal replacement. Forty-eight patients underwent esophageal reconstruction with this procedure. The esophagus damaged by caustic ingestion was left in place in all patients, without any subsequent problem. The authors have not used the distal esophagus for anastomosis in patients with atresia, because this segment may be abnormal; and, in any case, an isoperistaltic cologastric anastomosis does not reflux. The right or left colon or jejunum was used in the other cases. Three children lost an interposed intestinal segment from necrosis even though the bowel appeared to be well vascularized at the end of the operation. Each patient had successful reconstruction using another type of interposition. An intrathoracic leak occurred in one infant. A cervical anastomotic leak developed in 11 children, and a stricture in 13. Strictures were more common in patients who had caustic burns. Three patients required surgery for adhesive intestinal obstruction. A redundant colon transplant with ulceration, and the herniation of an ileal segment into the pleural cavity with obstruction prompted reoperation in two other patients. There were two deaths early in the series, one of which was secondary to postoperative respiratory arrest. The other death occurred in a child who had a caustic pharyngeal burn and chronic aspiration. All patients were seen in our office recently, or they or their parents were interviewed by phone. All of them are taking all of their nutrition by mouth. Forty-three of the 61 survivors have had no difficulty with swallowing. One required reoperation to enlarge the thoracic inlet. Seventeen other have mild dysphagia that does not require treatment. The patients with esophageal atresia or atresia and fistula consistently have not grown as well as those who required replacement for an acquired condition or injury.

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

PURPOSE: Patients 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. METHODS: Rabbits 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. RESULTS: The 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. CONCLUSION: Stimulation 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.

Long-term outcome in children with opsoclonus-myoclonus and ataxia and coincident neuroblastoma.

We reviewed the neurologic and developmental courses in 10 children with opsoclonus-myoclonus ("dancing eyes syndrome") and neuroblastoma. All patients are alive without evidence of neoplastic disease after 8+ to 111+ months of follow-up. All had localized disease and 50% had extraabdominal tumors. Neuroblastomas of nine children had favorable Shimada histologic characteristics, and all tumors had single copies of the N-myc oncogene. After neuroblastoma resection, all patients had persistent opsoclonus-myoclonus or ataxia that responded to therapy with adrenocorticotropic hormone. Nine children had relapses of neurologic symptoms. Three years after resection, six of seven patients with sufficient follow-up were free of symptoms and had discontinued therapy. However, nine children had chronic neurologic deficits, including cognitive and motor delays, language deficits, and behavioral abnormalities. All six patients in educational programs required special assistance. Five children required physical, occupational, or speech therapy. Long-term developmental and cognitive problems should be anticipated in patients with neuroblastoma who have opsoclonus-myoclonus or ataxia or both, and early intervention should be instituted to try to minimize these deficits.

The feasibility of laparoscopic swenson pull-through.

The Swenson abdominoperineal pull-through is the authors' preferred treatment for children with Hirschsprung's disease. The present study was performed to determine whether this procedure's 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.

Total parenteral nutrition-associated cholestasis: clinical and histopathologic correlation.

Cholestatic jaundice is the major complication of total parenteral nutrition (TPN) in infants and children. The pathogenesis of this syndrome is poorly understood. The aims of this study were: (1) to define the histologic liver injury in relation to the clinical course of infants on TPN and (2) to determine whether enteral feeding will reverse or halt these changes. We identified 31 infants treated for severe gastrointestinal disease for whom liver histology was available from 1987 to 1991. Clinical records and liver biopsy (23) or autopsy specimens (13) were reviewed. Five patients had biopsies at two subsequent operations. The clinical diagnosis was necrotizing enterocolitis (24), atresia or stenosis (3), midgut volvulus (2), Hirschsprung's disease (1), and sepsis (1). Twenty-one of 31 infants were premature and had a mean birth weight of 1,868 g. Twenty-five of 31 were on TPN and 28 of 31 had received some enteral feeding by the time of the biopsy. Enteral feeding was begun as early as possible in all infants even if continued TPN was necessary for full support. Cholestasis occurred in 71% of premature infants versus 22% of full-term babies. Infants with cholestasis had been on TPN for a longer time (37 days v 18) with a correspondingly shorter period of enteral feeding (17 days v 27). Mean total bilirubin level was 14 in patients with cholestasis and 5 in those without, but the bilirubin level did not correlate with the extent of histological injury and was frequently normal despite marked histological damage.(ABSTRACT TRUNCATED AT 250 WORDS)

A 25-year experience with renal tumors of childhood.

A retrospective analysis of the medical records of 234 children with renal tumors managed over a 25-year period at the Children's Memorial Medical Center was undertaken to evaluate long-term morbidity and mortality. There was a significant increase in survival over the years of the study. The 5-year survival for patients treated during the period 1985 to 1989 was 94% versus 68% for the period 1965 to 1969. Thirty-three children have died, 15 with known disease progression. Long-term morbidity included scoliosis (39), cardiorespiratory insufficiency (13), hypertension (7), renal insufficiency (7), small bowel obstruction (10), chest wall deformity (3), amenorrhea (1), leg length discrepancy (1), and 1 patient with an esophageal stricture. One patient with cardiomyopathy secondary to adriamycin has recently undergone cardiac transplantation. Five patients with renal insufficiency have required dialysis. Of these five, one patient has had two renal transplants. The presence of distant metastases and positive hilar or regional lymph nodes were the only findings at operation that were associated with an increased mortality (P = .005). There was a significantly increased mortality in those children operated on by general surgeons or urologists at other hospitals (11/43) versus those operated upon at our hospital (22/191) (P = .033). There was no statistical difference in the staging or histology among these children. We feel that the careful and systematic approach of a radical nephrectomy assures accurate staging of the tumor removing gross and microscopic disease in the abdomen.

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)

Familial adenomatous polyposis coli and clear cell sarcoma of the kidney.

Familial adenomatous polyposis coli is an inherited multiple neoplasia syndrome that is associated with an increased risk for development of another primary tumor. We report a case of a 14-year-old boy who had a proctocolectomy for familial adenomatous polyposis coli. He had survived radical nephrectomy, chemotherapy, and radiotherapy for a congenital clear cell sarcoma of the right kidney. Perhaps the presence of the familial adenomatous polyposis gene induces chromosomal instability in affected persons.

Rectal manometry, computed tomography, and functional results of anal atresia surgery.

Children with anal atresia often have compromised function. This is especially common with the anatomic "high lesion." Twenty-five patients have been studied using clinical evaluation, computed tomography (CT), and manometry to make an objective evaluation. The patients were an average of 9.6 years old; the male-to-female ratio was 3:2. Thirty-three percent had acceptable bowel function as defined by a continence score of 1 or 2. Only two (11%) had an abnormal sacrum. The mean rectal pressure was 20 cm H2O and 5 patients (23%) had a normal rectal relaxation reflex. Thirty percent had CT scans that demonstrated an intact external sphincter and puborectalis. In comparing the objective criteria and clinical results no technique could predict continence. CT demonstrated no significant group (P = .046 to .659) and manometry demonstrated no significant group (P = .082 to .752). We found no objective criteria that could evaluate the patient's clinical result or dictate therapy.

Early hepatobiliary dysfunction during total parenteral nutrition: an experimental study.

The etiology and pathophysiology of the liver disease associated with total parenteral nutrition (TPN) are unknown. In this study, we have attempted to define the early changes in hepatobiliary function during TPN in young rabbits nourished totally by the intravenous route for 3, 5, and 15 days, with age-matched rabbits on lab chow serving as controls. A decrease in basal bile flow along with elevations of serum bile acids and cholesterol was seen. The capacity for biliary secretion of sulfobromophthalein and of ursodeoxycholic acid was measured at the end of each diet regimen. Early impairment of biliary sulfobromophthalein (BSP) secretion was seen after 5 days of TPN, with no further deterioration after 15 days. Maximal bile acid secretory rate and bile flow, in response to the ursodeoxycholic acid load, were decreased after 15 days of TPN. Furthermore, after 15 days of TPN, both the volume of gallbladder bile and its bile acid content increased. The combined effects of the enteral fast and the intravenous administration of all nutrients were bile acid sequestration in an adynamic gallbladder with interruption of the enterohepatic circulation. In the parenterally fed rabbit, we have demonstrated bile secretory failure and gallbladder sludge, the two common complications of clinical TPN. These may be the early events that subsequently lead to cholestasis and liver damage in neonates maintained on prolonged TPN.

Choledochal duct cyst: resection with physiologic reconstruction.

BACKGROUND: The accepted surgical treatment of choledochal duct cyst is complete excision and enteric drainage through an intestinal conduit. Peptic ulceration and fat malabsorption have been reported after Roux-en-Y reconstruction. Such long-term complications may be avoided by a technique that simulates normal physiology. METHODS: Twenty-one patients have undergone resection of a choledochal duct cyst in the past 12 1/2 years. The pathologic duct is resected to the level of normal mucosa. A short segment of jejunum with a intussusception valve (1.5 to 2 cm) is interposed between the common hepatic duct and the duodenum. The medical records and all radiographs of each patient were reviewed. Eighteen children were reexamined or the parents were contacted by phone. RESULTS: Twenty of 21 patients recovered without major perioperative complications. Twelve of them are well and have no symptoms at 3 to 12+ years (mean, 6 years) after operation. Four children are currently well 6 to 19 months after operation. Three children were well when lost to follow-up. Two patients have radiographic evidence of incompetence of the interposition valve. One of these, who initially underwent operation at 9 months of age, was reexplored at 10 months and at 10 years for a stricture at the hepaticojejunal anastomosis. The other, a 7-year-old girl who was admitted with jaundice and pancreatitis, has had episodic abdominal pain for 7 years after operation but is well. CONCLUSIONS: The valved jejunal interposition hepaticoduodenostomy offers superior biliary reconstruction after excision of a choledochal duct cyst. Normal physiology is simulated, with bile draining directly into the duodenum. A short conduit prevents stasis, and biliary reflux is minimized with the addition of an intussusception valve.

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.

Polyglyconate suture in pediatric surgery.

The authors have clinically evaluated a synthetic monofilament suture and have found it very satisfactory for all wound closures, esophageal and intestinal anastomoses, and bronchial closures.