Hepatic pulmonary fusion in neonates.

OBJECTIVE: This report discusses the relationship of supradiaphragmatic hepatic tissue that is fused to the lung (through a diaphragmatic defect) with pulmonary hypoplasia-a new constellation of findings. CONCLUSION: Hepatic pulmonary fusion should be suspected in instances of apparent diaphragmatic hernia characterized by mediastinal shift towards the hypoplastic lung or when the mediastinum does not shift away from the mass.

Congenital tracheal anomalies in the craniosynostosis syndromes.

The authors present the case of a 12-year-old girl with Pfeiffer's syndrome who underwent successful resection of a tracheal cartilaginous sleeve (TCS) for treatment of sleep apnea. There is growing recognition of the inclusion of TCS in the spectrum of congenital cartilage malformations seen in patients with craniosynostosis (CS) syndromes. This case demonstrates the difficult therapeutic challenge created by the combination of hypopharyngeal and intrinsic airway abnormalities present in CS patients. The early recognition of TCS in these patients may provide the opportunity for improved outcome in this severely affected subgroup of CS patients with otherwise high mortality.

Evaluation of contrast media for bronchography.

BACKGROUND: Bronchography is occasionally needed for the evaluation and management of some congenital pulmonary anomalies as well as some acquired diseases, usually of the tracheo- bronchial tree. There is currently no effective, approved contrast agent for this imaging techniq ue. OBJECTIVE: We evaluated five agents (barium sulfate, iohexol, propyliodone oily, propyliodone aqueous, and perflubron) in terms of image quality, histologic changes, and effects on hemodynamics, blood gases, and standard laboratory tests in New Zealand White rabbits. MATERIALS AND METHODS: Animals were anesthetized and intubated. Each contrast agent (0.25 ml/kg) was administered intratracheally. Three animals in each group had intravenous lines placed for blood sampling and blood pressure monitoring and were sacrificed at 1 h. An additional three animals for each agent were sacrificed at 24 h and 1 week after imaging. Blood samples were taken immediately before contrast instillation and at 1 h postbronchography. Fluoroscopic images were recorded on standard VHS video tape and evaluated in blind fashion. Segments of lung tissue and bronchi were obtained for histologic examination. RESULTS: Necrosis and/or inflammatory infiltrates were noted in 78 % of the bronchograms performed with propyliodone aqueous, 67 % with propyliodone oily, 55 % with perflubron, and 33 % with iohexol 120, 240 and 350. No histologic damage was observed with barium. The propyliodones gave the best-quality imaging results and the most histologic changes. Iohexol, in any concentration, gave the least acceptable images and a moderate number of histologic changes. Barium sulfate demonstrated acceptable images with virtually no histologic changes. CONCLUSION: From the histologic and imaging results, barium is the best available contrast material for bronchography.

Extracorporeal membrane oxygenation as salvage in pediatric surgical emergencies.

Extracorporeal membrane oxygenation (ECMO) has become an established therapy for acute neonatal and pediatric respiratory failure. On an institutional level, once an ECMO program is well established, ECMO can be viewed as a logical extension of critical care for multisystem organ failure. The question left unanswered is "Should anyone die without being offered ECMO?" The authors reviewed a 10-year clinical experience with ECMO and its application as salvage therapy in pediatric surgical emergencies. Eight patients with life-threatening multisystem organ failure, from diverse causes, were treated; the survival rate was 50%. All survivors were neurologically intact at the time of discharge. The success of ECMO, coupled with improvements in technique, apparatus, and expertise, has allowed application of ECMO as an invasive extension of intensive care to diverse patient groups. These results have encouraged the authors to expand their indications and to push the "envelope" in offering ECMO to critically ill infants and children with life-threatening organ failure.

Hirschsprung's disease: three decades' experience at a single institution.

Experience with 250 patients treated for Hirschsprung's disease over the past three decades shows several interesting features. Only 58% had rectosigmoid disease. Twenty-six percent had long-segment disease, and 12% had total colon aganglionosis. This distribution did not change significantly over the decades. The male:female ratio was 3.3 in all groups, but dropped to 2.2 with total colon disease. The mean age at diagnosis decreased from 18.8 months during the 1960s to 2.6 months in the 1980s. The mean age at which the pull-through procedure was performed also dropped from 34 months to 15 months. Twenty-three patients died: 17 in the 1960s and three in each of the succeeding decades. The Swenson procedure was predominant during the 1960s, but was only 2% of the pull-throughs in the 1980s. The endorectal pull-through increased from 22% in the 1960s to 71% in the 1980s. In the 1970s and 1980s the Duhamel accounted for 26% of the pull-throughs. With a mean follow-up of 51 months, the mean number of episodes of post pull-through enterocolitis per patient was 0.53. This increased from 0.44 in the 1960s, to 0.52 in the 1970s, to 0.67 in the 1980s. The incidence of post-pull-through enterocolitis increased somewhat with the length of the bowel involved, and was greatest with the Duhamel (0.95 episodes per patient), less with the Swenson (0.60), and least with the endorectal pull-through (0.38). In contrast, the incidence of complications other than enterocolitis was greatest with the Swenson (20%), less with the endorectal pull-through (17%), and least with the Duhamel (13%).(ABSTRACT TRUNCATED AT 250 WORDS)

Balloon dilatation of postoperative tracheal stenosis.

Three patients who had previously undergone linear augmentation tracheoplasty developed postoperative stenosis within the grafted segment. In each patient, balloon catheter dilatation successfully enlarged the stenotic airway obviating secondary reconstruction in two.

Pancreatitis in childhood. Experience with 49 patients.

Pancreatitis in children is not common but can be associated with severe morbidity rates. We have treated 49 children with pancreatitis over the past 12 years ranging in age from 1 month to 18 years. One third of the patients had biliary tract disease as an etiology, with nearly half of these being related to underlying hematologic disease, usually sickle cell anemia. Another third of the pancreatitis was due to trauma, and one third of these were related to child abuse. Other etiologies were systemic disease (6 patients), congenital anomalies (8 patients), and idiopathic (3 cases). Eighty-two per cent of the patients presented with abdominal pain, but four children, all less than 4 years old, presented with an abdominal mass. Twenty-nine patients required 33 operations for pancreatitis. Fifteen of the 16 patients with biliary tract disease and all patients with congenital anomalies required operation. Six of the 16 patients with trauma required operation and none of those with systemic disease. As in adults ultrasonographic examination and CT scan are most important in the diagnosis; medical treatment consists of intravenous (I.V.) fluids, nasogastric suction, and total parenteral nutrition (TPN), and risk factors can help predict the severity of the disease while amylase alone is not related to severity. Different from adults, in children an etiology can usually be determined. The common etiologies, biliary tract disease, trauma, and congenital anomalies frequently require operation.

Hemorrhagic complications during extracorporeal membrane oxygenation: prevention and treatment.

Hemorrhage related to systemic heparinization is the major complication of extracorporeal membrane oxygenation (ECMO). Intracranial hemorrhage (ICH) is the most devastating complication. ICH developed in 13 of our 25 ECMO patients (52%). Six died, six survived with normal neurologic function, and one is severely impaired. In nine of 13 patients (69%) ECMO was discontinued when serial cranial ultrasounds showed progressive ICH. Seizures developed in six infants while receiving ECMO, and ICH developed in all. There is a correlation between hypertension and ICH. A hypertension index (hours systolic BP greater than 90/hours receiving ECMO) was 0.1 +/- 0.12 for infants without ICH and 0.37 +/- 0.28 for infants with ICH (P less than .05). ICH developed in 79% of the patients with an index greater than 0.1. Twenty neck explorations were required in the first 20 patients for incisional bleeding (mean blood loss, 21.9 +/- 18.0 mL/kg/d). We now use fibrin glue following cannulation and have done only one neck exploration in the last five patients (mean blood loss, 2.8 +/- 2.2 mL/kg/d, P less than .05). Endobronchial bleeding has responded to phenylephrine lavage and increased positive end-expiratory pressure. We have controlled pleural space bleeding with topical thrombin. None of the hemorrhagic complications encountered correlate with the activated clotting time or the amount of heparin used. There is an increased risk of hemorrhage associated with platelet counts less than 100,000/microL for 75% of a day (P less than .05) so that aggressive platelet transfusion remains important in preventing hemorrhagic complications during ECMO.

Occlusion of abdominal aorta in the newborn.

This is a report of a case of primary occlusion of the abdominal aorta in the first day of life that was managed operatively. Thromboembolic occlusion of the abdominal aorta is a surgical emergency. When promptly recognized and managed with arteriotomy and thromboembolectomy, morbidity and mortality should significantly decrease.

Transpleural repair of esophageal atresia without a primary gastrostomy: 240 patients treated between 1951 and 1983.

Esophageal atresia with distal tracheoesophageal fistula has been treated at one institution between 1951 and 1983 primarily by prompt transpleural repair without gastrostomy. Two hundred and seventy one patients are assigned to three time periods: (1) 1951 to 1963, prior to modern intensive care; (2) 1964 to 1973, the initial era of mechanical ventilation; and 1974 to 1983, the era of readily available ventilators and TPN. During these time periods mean birthweight decreased from 2,780 g to 2,670 g to 2,500 g, while the incidence of associated anomalies increased from 41% to 44% to 55%. Eleven patients had no operation or gastrostomy only and died. Twenty (predominantly in the early years) had staged repairs with initial gastrostomy and fistula ligation. Primary anastomosis was done in 240 patients regardless of birth weight, 229 of which were by the transpleural approach. Gastrostomy was performed primarily in 28 patients for varied indications. Operative mortality (definitive as well as staged repairs) fell over the three time periods from 44% to 15% to 7%. Anastomotic leaks occurred in 20% of patients who underwent primary repair regardless of the time period. The mortality associated with a leak, however, decreased from 88% to 47% to 0%. Anastomotic strictures requiring more than two dilatations occurred in 15% in all periods. The overall incidence of recurrent fistula was 5%. Of 200 patients surviving the initial hospitalization, follow-up longer than 2 months is available for 156 patients with a median follow-up of 30 months. There were 15 late deaths.(ABSTRACT TRUNCATED AT 250 WORDS)

Congenital diaphragmatic hernia.

Congenital diaphragmatic hernia continues to be a critical problem in neonatal surgery. Despite the apparent simplicity of the anatomic defect, the physiology is complex, and survival remains uncertain. Surgical success has been achieved, but we recognize that the barrier to survival is pulmonary parenchymal and vascular hypoplasia as well as the complex syndrome of persistent fetal circulation. In many ways the problem of diaphragmatic hernia is as much of an enigma to today's physician-scientist as it was to Bochdalek in the nineteenth century. The treatment of respiratory distress after repair of congenital diaphragmatic hernia has brought out the most creative and innovative efforts of pediatric surgeons in both the laboratory and the intensive care unit.

End-tidal CO2 and tissue pH in the monitoring of acid-base changes: a composite technique for continuous, minimally invasive monitoring.

The infrared CO2 analyzer continuously monitors the CO2 tension in exhaled air at end-tidal expiration. In experimental animals, we found a consistent relationship between PaCO2 and end-tidal CO2 (ET.CO2) in the normal steady state, and in acid-base disturbances (respiratory acidosis and alkalosis, and hypoperfusion acidosis). Paired data analyses of PaCO2 (X) and ET.CO2 (Y) yielded correlation coefficients of r = 0.98 (Y = 0.96X + 4.43) during progressive hypercarbia (PaCO2: 32----110 torr), and r = 0.93 (Y = 0.89X + 0.93) during hyperventilation hypocapnia (PaCO2: 41----14 torr). The relationship between PaCO2 and ET.CO2 was seen during hypovolemic shock if pulmonary perfusion was maintained uniform in all areas of lung. The ability of the ET.CO2 sensor to predict instantaneously the PaCO2 makes it attractive enough to be used in conjunction with the subcutaneous tissue pH(pHe) sensor in the management of acid-base disturbances. After hypercarbia (FiCO2 0.15 X 40 min; PaCO2/ET.CO2: 100/101 torr), when the dogs were returned to room air, abruptly both the ET.CO2 and pHe sensors were sensitive to the changes in Fi.CO2. But the response of the ET.CO2 was swifter. The advent of transcutaneous gas monitors has shown that intermittent blood gas analyses, however frequent, are inadequate for the monitoring of the rapidly altering blood gas status in the acutely ill. The ability of the pHe sensor to identify whole-body acidosis and alkalosis combined with the speed and ease of the ET.CO2 monitor in pinpointing hypercarbic and hypocarbic states makes this two-parameter system suitable for the continuous, noninvasive monitoring of the critically ill.

Pancreas divisum: a cause of pancreatitis in childhood.

The anatomic congenital anomaly pancreas divisum has recently been implicated as a cause of acute pancreatitis. The associated stenosis of the minor papilla has been postulated to cause pancreatitis by producing a relative obstruction to the high volumes of dorsal pancreatic secretion. We present a 10-year-old boy with acute pancreatitis associated with pancreas divisum to illustrate this as a cause of pancreatitis in childhood.

Significance of mediastinal metastatic disease in Wilms' tumor of favorable histology.

Although the results of the National Wilms' Tumor Study suggest poor outcome for children with metastases to intra-abdominal, intracranial, skeletal, skin, diaphragm, or mediastinal sites, the incidence and course of mediastinal disease has not been described. Fifty-four newly diagnosed Wilms' tumor patients at Children's Hospital of Michigan (1972-1980) were studied for patterns of intrathoracic metastases. All had favorable histology; mean age (MA) at diagnosis was 4.5 years. Initial chemotherapy ranged from single agent to three-drug combination (actinomycin, vincristine, adriamycin). Intrathoracic metastases occurred in 14 (26%) from 0-28 months (median, 3.5 months) from diagnosis (MA, 5.8 years). Upon the discovery of intrathoracic metastases all received radiation (1,200-2,000 rads) to the chest, some with supplementary doses to the mediastinum. Five children with parenchymal and one with parenchymal and hilar metastases appearing 0-28 months after the time of diagnosis survive disease-free 44-127 months after metastases appeared (MA, 6.3 years). Eight children developed parenchymal and mediastinal metastases (MA, 5.4 years). Time from diagnosis to parenchymal metastases was 0-7 months; time from diagnosis to mediastinal metastases was 0-16 months; and in four, both sites appeared simultaneously. Death occurred in all eight patients 7-25 months from diagnosis. Only one child had evidence of recurrence outside the chest. Potential risk factors and influence on survival are discussed.

Continuous monitoring of pH in the tissue mode: evaluation of a miniature sensor during acidosis and tissue hypoperfusion.

The in vivo performance of a 20G copolymer pH sensor, needlelike in configuration, was studied in the normal dog, and dogs made acidotic by the constant infusion of lactic acid, or by the induction of tissue perfusion defects. Sensors were placed at two extravascular sites in the leg, deep subcutaneous (pHe/sc), and intramuscular in the adductor (pHe/im). This pH sensor is a silver wire capped by a H+-specific polymer; it has a built-in reference system. Its electrochemical characteristics and in vivo performance are similar to those of glass pH electrodes. The continuously monitored values were compared with discrete arterial blood gas analyses at 10 to 20 minute intervals. The baseline values in 15 dogs under general anesthesia were: pH/art 7.331 +/- .042, pHe/sc 7.291 +/- .076, and pHe/im 7.265 +/- .102 (mean +/- SD; n = 45 observations each). During metabolic acidosis (lactic acid infusion), the direction and rates of change were similar in pHe/sc and pHe/im. Tissue perfusion defects were induced by moderate-to-severe hemorrhage (single or repeated bleeds) or operative shock (splenectomy and exteriorization of bowel). Both pHe/sc and pHe/im fell sharply, with a more gradual drop in pH/art. In those who survived after infusion of shed blood or dextran-40, pHe recovered rapidly. In the moribund, pHe continued to deteriorate. This pH sensor is a sensitive prognosticator of acid-base changes in the tissue. The in vivo drift is small: 0.008 pH per hour. The placement of the sensor via an intracath cannula in the subcutaneous tissue of the thigh is recommended.(ABSTRACT TRUNCATED AT 250 WORDS)

A case of vasoactive intestinal polypeptide secreting ganglioneuroma complicated by parasitic infestations.

A case of vasoactive intestinal polypeptide secreting ganglioneuroma associated with watery diarrhea and hypokalemia is presented. Diagnosis was delayed due to the presence of two parasitic infestations. After surgical removal of the tumor, the diarrhea resolved.

Asymptomatic intracardiac metastasis from osteosarcoma: a case report with literature review.

Osteosarcoma very rarely metastasizes to the heart. Thirteen cases have been reported in the literature so far. Diagnosis in some of these cases was made during investigation for severe cardiac failure and in most of them at autopsy. Our patient, a 13-year-old girl, showed right pulmonary metastases on chest x-ray 1 year after above knee amputation for osteosarcoma of the distal femur. Routine preoperative computerized axial tomography (CT Scan) revealed a calcified lesion in the heart in addition to the pulmonary metastases. She was very active and completely asymptomatic. Two-dimensional echocardiography, angiography, and right and left heart catheterizations were done. This revealed a large mobile metastatic lesion in the right ventricle. The intraventricular tumor was successfully removed, and 12 days later she had a second thoracotomy for removal of pulmonary metastases. Nine months after her intraventricular metastasis was removed she developed a solitary right pulmonary metastasis. This was successfully resected. Now, 10 months later, she is disease free and completely asymptomatic.