Total sequestration of the right lung.

A 2-day-old baby with severe respiratory distress and cyanosis was admitted to the hospital with a totally opacified right pleural cavity. Preoperative studies demonstrated absence of the right pulmonary artery, and a patent ductus arteriosus, and a left pulmonary artery pressure of 85/45 mm. Hg. The hypoplastic right lung received its entire blood supply from multiple vessels through the diaphragm. The infant's condition improved initially after a right pneumonectomy, but she died of progressive respiratory insufficiency at the age of 7 months.

Pediatric esophageal perforation.

Although esophageal perforation in children is associated with a spectrum of disease different from the one in adults, management is essentially the same for both groups. Over the past 11 years, 12 patients ranging in age from 2 days to 10 years were treated for 13 perforations. Perforation in the adult is associated with a high mortality rate. All children in this series survived. Injury was secondary to instrumentation in 10 cases and three resulted from the chronic erosion of a trapped foreign body. Of the 10 acute perforations, five occurred 12 days to 5 months after lye ingestion. Anastomotic narrowing was a factor in three other cases. Seven of the acute perforations involved the thoracic esophagus and were associated with a high morbidity rate. Management consisted of operative as well as nonoperative approaches. Consideration must be given to several clinical variables including location, cause, predisposing factors, underlying illnesses, associated injury, and promptness of medical attention.

Mini-thoracotomy and chest tube insertion for children with empyema.

In contrast to the two prior decades, empyema in children has become a rare disease during the past 10 years. Thirty-two children with empyema from all causes were treated at The Children's Memorial Hospital in Chicago in the 11 years between 1970 and 1982. Twenty cases followed postinfectious pneumonia, most commonly due to Staphylococcus aureus. The remaining patients had either an esophageal leak or a pleural infection following an intrathoracic operation. Fourteen of our patients were managed with a "mini-thoracotomy," which allowed accurate drainage of purulent material, débridement of fibrinous exudate, and rapid expansion of the lung with prompt relief from fever and toxicity. In our hands, this procedure provided results which were superior to those obtained with simple closed chest tube drainage. In addition, during this period of time, ultrasound examination of the chest has provided a rapid, accurate technique for the early detection and localization of fluid accumulations within the pleural cavity. Pre-drainage thoracentesis can be accurately guided by ultrasonic imaging.

Brain heterotopia in the lungs. A rare cause of respiratory distress in the newborn.

This article documents the presence of multifocal, bilateral glial heterotopia in the lungs manifesting as symptomatic lung masses during the newborn period. Prior to the present report, this rare form of heterotopia was known to occur exclusively in anencephalic infants. The observed bilateral pulmonary disease had an important cystic component, clinically and radiologically similar to cystic adenomatoid malformation. Theoretically, several explanations may be elaborated to account for the presence of central nervous tissue in the lungs: embolic events; protrusion of embryonic brain structures that are subsequently dislodged from their normal site; in-situ transformation of local developing tissues; and teratoma with predominance of neural tissue. The evidence is insufficient to conclude in favor of any of these explanations.

Surgical management of tracheosophageal fistula complicating caustic ingestion.

This report outlines principles of management for extensive corrosive esophageal burns in children complicated by formation of a tracheoesophageal fistula (TEF). Direct operative attack on the fistula usually is unsuccessful, since the tracheal tissues are so damaged that they will not hold sutures. On the basis of experiences with six children, we suggest the following plan of management: (1) early investigation of suspected TEF with thin barium or Dionosil; (2) early tracheostomy using a short, plastic tube; (3) end cervical esophagostomy with closure of the distal stump of the cervical esophagus; (4) gastrotomy; (5) complete disconnection of the intra-abdominal esophagus from the stomach. This can be completed in a single operation and leaves the thoracic esophagus containing the fistula completely isolated so that the trachea is protected from contamination by saliva and gastric juice. The esophageal mucosa in all cases has been destroyed so extensively by the corrosive material that the esophagus heals as a band of muscle and scar. If protected from continuous contamination by saliva, the trachea heals itself with little long-term defect. The esophagus is replaced with colon or a gastric tube 6 to 12 months later when the child is in good health. All four children treated by this regimen have survived and are able to eat normally.

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.

Gastroschisis in 106 consecutive newborn infants.

Primary tissue closure of gastroschisis remains controversial. Some surgeons routinely place a silicone rubber sheet silo over the exposed bowel, planning a staged closure. In the past 14 1/2 years, we have cared for 106 newborns with gastroschisis, closing the defect primarily in 80%. The success of this technique depends on enlarging the abdominal cavity and decreasing the volume of bowel that must be replaced in the peritoneal cavity. Thorough preoperative rectal irrigation should evacuate all meconium. After undermining the skin around the abdominal wall defect for only 1 cm, a midline subcutaneous fasciotomy is created from the xiphoid to the pubis. The abdominal wall is then stretched in all quadrants beginning at the flanks. The eviscerated small bowel can often be returned without enlarging the initial skin defect. The skin is closed with subcuticular absorbable sutures reinforced by long skin tapes. The small ventral hernia that results is closed at about 1 year of age. Fascia could be closed primarily in 28% of these patients, and 17% required a prosthetic pouch. The duration of postoperative ileus and length of hospital stay were statistically significantly shorter in the infants who underwent primary closure. Even though more complicated patients were included in the primary closure group, the incidence of mortality and morbidity was not higher than in patients treated with silicone rubber pouches. Deaths were inevitable in five infants with gangrenous bowel, multiple anomalies, and extreme prematurity. Deaths were related to sepsis in three infants and were the result of operative or anesthetic technique in four. Only two preoperative factors were prognostic of morbidity and mortality: gestational age (but not birth weight) and the presence of intestinal ischemia or atresia.

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.

The gracilis sling for fecal incontinence.

During the past 20 yr, we have transplanted the gracilis muscle to the perianal position for use as a sphincter to achieve continence in 7 completely incontinent children, ages 7 to 16 yr. Complete continence was achieved in three, three were improved and one transplant became infected and failed completely. The procedure has been performed as originally described by Pickrell. The bowel must be impeccably clean prior to the operation and the muscle must be sutured as tightly as possible about the anus. These two details, in addition to postoperative training, are essential for a successful result.

Who should pay for resident education?

House staff stipends and other costs related to maintaining a residency are listed on hospital budgets as "education." This has become a tempting target for hospital administrators and third-party payors, who ask, "Should the patient pay for medical education?" We have studied the workload of house officers on a typical pediatric surgical service in a children's hospital. General surgical residents spend 56.3 hours per week in patient care. This does not count "lag" time or "on call" time, but work that someone else--either a physician, technician, or nurse practitioner would do if there was no residency. This study, as well as others, clearly indicates that residents provide an enormous amount of patient care. It is perfectly justified to include the costs of house officers in the hospital budget.

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.

Eikenella corrodens infections in children.

Techniques for obtaining, transporting, and culturing bacteriologic specimens have improved in recent years. The laboratory is now identifying rare fastidious organisms in surgical infections, which were previously unknown to clinicians. One of these organisms, Eikenella corrodens, was found in 28 children over 5 years. It was most commonly found in patients with perforated appendices or in wounds with oral contamination. Eikenella most often occurs in multibacterial infections but has also been grown in pure culture. When this bacteria is found in a patient with a serious surgical infection the wound must be widely opened and debrided of necrotic tissue. Prolonged antibiotic therapy based on culture and sensitivity is necessary to prevent prolonged or recurrent infection.

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.

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.

Long-term complications in a premature infant with tracheoesophageal fistula.

This is a report of the short- and long-term complications in a premature infant with tracheoesophageal fistula, including those related to central venous alimentation, seizures, chylothorax, bronchopulmonary dysplasia, dental erosions, gastroesophageal reflux, pulmonary problems, and gall stones. It offers analyses of possible alternate methods and treatments, which may have provided a better course.

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.

Liver abscess.

A liver abscess may occur secondary to an umbilical vein catheterization or infection in other parts of the body, and is associated with immune deficiencies, especially chronic granulomatous disease. There are no specific signs and symptoms, but an unexplained fever with upper abdominal tenderness and an enlarged liver were present in all of our cases. A liver-spleen scan is the most useful diagnostic test, demonstrating a filling defect in the liver. A single abscess may be unroofed and drained. When multiple abscesses are found, as many as possible are drained, but long-term specific antibiotic therapy is the most important treatment.

Congenital colonic atresia.

Nineteen cases of congenital colon atresia are presented with two deaths resulting in a mortality rate of 10.5%. The authors conclude that operative management of atresias proximal to the splenic flexure should include resection of the dilated proximal colon with primary ileotransverse colostomy or ileosigmoidostomy. Atresias distal to the splenic flexure should be managed with a colostomy with later establishment of gastrointestinal continuity.

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.

Pediatric annular pancreas: twenty years' experience.

Characterization of pediatric annular pancreas is provided by this analysis of 24 cases (22 neonates, 1 infant, 1 child). Salient observations include: (1) Presentation is affected by the degree of duodenal obstruction at birth and by coexistent anomalies. (2) Polyhydramnios usually accompanies complete high intestinal obstruction by annular pancreas. (3) Primary biliary interruption was not encountered and jaundice was not unusually prevalent. (4) There is a high incidence of associated anomalies. (5) Duodenal bypass by duodenoenterostomy was employed with excellent results.