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Inflammatory myofibroblastic tumor is a rare gastrointestinal neoplasm. The authors report the case of a 5-year-old girl who initially presented with gastric outlet obstruction secondary to an inflammatory myofibroblastic tumor along the lesser gastric curvature. A subtotal gastrectomy and wedge resection of a left hepatic lobe nodule were performed. Obstructive jaundice developed one month postoperatively. Computerized tomography, percutaneous transhepatic cholangiography, and selective celiac and mesenteric arteriography showed a mass that involved the left hepatic lobe, with concomitant high-grade biliary obstruction and portal venous encasement. Percutaneous biliary drainage was performed. During laparotomy, the tumor was found to be unresectable, and the patient was referred to the Liver Transplant Service at The Children's Hospital of Philadelphia. A total hepatectomy with temporary portocaval shunt was performed, leaving the inferior vena cava in situ. At the back table, an ex vivo left trisegmentectomy was performed, followed by reimplantation of the posterior segment of the right hepatic lobe. The reimplanted liver segment functioned poorly, and completion hepatectomy with portocaval shunt was performed 24 hours postoperatively, because of severe coagulopathy, intraabdominal bleeding, and hemodynamic instability. The patient's condition stabilized, and she was listed for urgent liver transplantation. The anhepatic state was managed with intermittent plasmapheresis. She had transplantation 72 hours later, and was discharged from the hospital 3 weeks postoperatively after an uneventful recovery. She remains disease-free and has normal liver function 8 months after transplantation.

The case of a neonate with midgut volvulus and severe intestinal ischemia extending from the ligament of Treitz to the midtransverse colon is presented. Management consisted of abdominal silo application at the initial exploration to obviate the adverse physiological consequences of increased intraabdominal pressure from reperfusion edema of the intestine. The majority of the intestine was salvaged, and a short segment of ileum was resected. This method of treatment resulted in optimal ventilatory status, renal function, and cardiac performance. The advantages of temporary prosthetic wound coverage in selected cases of midgut volvulus include greater physiologic stability through the avoidance of "intraabdominal hypertension" and the ability to monitor the status of the intestine at the bedside.

Chronic salivary aspiration results in progressive pulmonary deterioration. Laryngotracheal separation and diversion were performed to prevent salivary aspiration in a patient following complex repair for esophageal atresia with distal tracheoesophageal fistula. This highly effective and potentially reversible procedure should be considered for treatment of chronic salivary aspiration in selected patients.

A case is reported of recurrent laryngotracheoesophageal cleft following two attempted repairs. The anterior approach to the larynx and trachea provided excellent exposure for defining the extent of the recurrent cleft, and allowed precise anatomic repair. Neurovascular structures were easily avoided by this operative method. Of 170 clefts reported in the literature, 19 recurred necessitating reoperation. The anterior approach to the larynx and trachea is appropriate in recurrent clefts and may be preferable at the initial repair.

Splenic salvage following blunt injury can fail when parenchymal bleeding is uncontrollable. To define the usefulness of an argon beam coagulator for hemostasis, we used the instrument in a laboratory trial of partial splenic resection. New Zealand white rabbits, weighing 4 to 5 kg, had sharp excision of the lower half of the spleen. No sutures were used to control hemorrhage from the cut splenic surface or the hilar vessels. Hemostasis was achieved with 2 to 4 seconds of electrocoagulation delivered by a beam of argon gas. All animals survived the procedure and were in good health when killed between the fourth and sixth week following the procedure. At necropsy, the spleen was viable in all animals with no abscess or hematoma. Minimal adhesions from the treated splenic surface to the omentum were found. The scar at the cut surface was 1 mm in depth, and the histology of the remainder of the spleen was normal. In this simulated splenic injury model, argon beam coagulation was uniformly successful in achieving hemostasis. Minimal tissue destruction and lack of infection were noted. The argon beam coagulator may be useful in patients with severe splenic injuries and other situations requiring partial splenectomy.

Cyclic neutropenia is an hereditary disorder of white blood cells, characterized by profound neutropenic episodes approximately every three weeks. Septic complications are usually limited to cutaneous and oropharyngeal infections. A 4-year-old boy with known cyclic neutropenia was in shock with neutropenia, clostridial septicemia, and right lower quadrant peritonitis when he was admitted. At the time of laparotomy, inflammation of the cecum, with no gross perforation, was found; no resection or appendectomy was done. He subsequently developed a right lower quadrant abscess that was drained, resulting in a colocutaneous fistula. For the next 8 months his fistula persisted, with intermittent episodes of fever, increased fistula output, and abdominal pain during his neutropenic periods. Standard nonoperative approaches to healing the fistula failed (ie, elemental feeds, total parenteral nutrition, irrigations, antibiotics, and drains). Attempts to medically abolish his neutropenic episodes using lithium, gammaglobulin, and steroids also failed. Ultimately, he underwent an ileocecal resection with primary anastamosis; the operation was done immediately following a neutropenic episode, in order to allow adequate healing of his anastamosis before his next period of neutropenia. Postoperative course was satisfactory, and he remains well after 8 months follow-up. This case, and several similar previously reported cases, illustrate that cyclic neutropenia may present with serious surgical complications. They also underlines the important role that neutrophils play in the healing of enteric fistulae.

A technique of central venous line (CVL) placement using ECG guidance is described. The method utilizes equipment found in most operating room departments, is easily learned and taught, and obviates the need for x-rays. CVL placement in 50 consecutive infants and children was performed over a 4-month period by the ECG-guided method, with catheter tip position subsequently determined by x-ray study. The technique demonstrated a 96% success and 100% accuracy rate. The two technical failures were both preventable. Equipment malfunction was responsible for one failure. The second failure was secondary to a preexisting supraventricular arrhythmia, and patients with arrhythmias or pacemakers are not suitable candidates for this technique of central venous catheterization. Radiologic methods of CVL placement were necessary in these two patients.

The combined anomalies of pulmonary agenesis with esophageal atresia and distal tracheoesopahgeal fistula are an exceedingly rare and highly lethal association. The longest survivor in the literature is 10 months. We describe two neonates with this association who underwent primary repair shortly after birth and who are alive and well at 24 and 18 months. One of these neonates also demonstrated duodenal atresia. The prime goal in the management of these patients is early protection and preservation of respiratory units. This goal is best accomplished by primary repair of the tracheoesophageal lesion, when feasible, rather than by more complex procedures.

Over a 20-month period, we prospectively studied 41 children with blunt thoracic trauma (BTT) to determine the incidence and morbidity of cardiac injury in this population. Four patients died, and the data were incomplete in 10, leaving 27 for analysis. Serial electrocardiograms (ECG) were abnormal in 36% of the patients studied, serial creatine phosphokinase isoenzyme (CK-MB) ratios were elevated in 31%, echocardiogram showed septal dysfunction in 14%, and pyrophosphate scan showed grade 1 uptake in 14%. There was poor correlation among tests, since only four patients had more than one abnormal test. There was no significant difference in Injury Severity Score or Thoracic Abbreviated Injury Scale between patients with and without abnormal investigations. No patient in the study developed arrhythmias or cardiac failure. In 13 trauma-related deaths undergoing autopsy over the same period, including the four with thoracic trauma, none had evidence of cardiac injury. These results indicate a striking lack of consistency in the diagnosis of posttraumatic cardiac injury in children using standard investigations. The absence of adverse cardiac events in surviving patients and the lack of autopsy evidence of cardiac injury in the trauma deaths suggest that the actual incidence and clinical significance of these lesions in children is lower than generally reported. Children with BTT should be followed clinically, and reliance on screening tests should be avoided.

Biliary atresia is the most common indication for liver transplantation in infants and children, despite the advent of the Kasai operation. Coexisting anomalies, which have been noted in up to 27% of patients with biliary atresia, may form an association known as the "polysplenia syndrome," which includes (1) polysplenia, (2) midgut malrotation, (3) preduodenal portal vein, (4) absent prerenal inferior vena cava with azygos continuation, (5) situs inversus, (6) symmetric liver, (7) hepatic arterial anomalies, and (8) bilobed right lung with hyparterial bronchus. Two of 31 patients undergoing orthotopic liver transplantation for biliary atresia following failed portoenterostomy over the past 11 years manifested the polysplenia syndrome with absent prerenal inferior vena cava. The clinical course of these patients, constellation of anomalies, and technical adjustments required to perform liver transplantation are described. We do not believe that these complex congenital anomalies preclude liver transplantation.

Unilateral agenesis of ribs with a "lung hernia," may cause severe respiratory distress in a neonate. Our experience with the use of a temporary external chest splint for stabilization of the congenital flail chest in one patient is presented. The device facilitated extubation and allowed the infant to grow prior to future surgical reconstruction.