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Persistent hyperinsulinemic hypoglycemia of the neonate (PHHN) usually presents in the neonate or infant. Although rare, the possibility of PHHN should be considered in all neonates who have unremitting hypoglycemia. Untreated hypoglycemia results in severe neurological disability or death. An inappropriately elevated serum insulin level and low serum ketone and free-fatty-acid concentrations in the presence of hypoglycemia confirm the diagnosis. Early diagnosis and aggressive medical management have reduced the morbidity associated with PHHN. Pancreatic resection is necessary for long-term control of hypoglycemia when medical therapy fails. Ninety-five percent pancreatectomy has been the procedure of choice at the author's institution and other pancreatic centers. However, a review of the authors' experience and of in the English-language literature has demonstrated a 33% failure rate, requiring further surgery or medical management. Furthermore, long-term follow-up showed that diabetes develops in most children who undergo 95% pancreatectomy. The high failure rate of 95% pancreatectomy and the ultimate development of diabetes in virtually all children suggest the alternative approaches should be considered.

Aortopexy is the more extended treatment for severe tracheomalacia, when it fails, reintervention and other procedures are necessary. We present our experience in the treatment of this pathology.Tracheomalacia, bronchomalacia and tracheobroncomalacia cases during a twelve year period (1983-1995) were reviewed (type, age, symptoms, surgical procedures and results were collected).Nineteen patients (9 males, 10 females) presented tracheobronchomalacia in our Hospital. Diagnosis was made by bronchoscopy in all of them: nine cases of tracheomalacia, five bronchomalacias and five suprastomal malacias were found. Mean diagnosis age was twenty four months (newborn-twelve years). Ten patients were managed nonoperatively, five with anterior cricoideal suspension and two cases were treated by aortopexy; one of them, after 2 months of respiratory stridor and recurrent respiratory arrest, needed an endoscopically placed intraluminal expandable Palmaz Prothesis. This prothesis was removed two years later. In this moment, the patient is doing well without stridor or respiratory symptoms.

Twenty-five central venous lines (two external 23 subcutaneous ports) were placed in 19 boys with haemophilia A (n = 17) or B (n = 2). The mean age of the boys was 4.9 years (range 0.2-15.3 years). The haemophilia was severe (factor level1%) in 18 boys and moderate (factor level 3%) in one. Three boys had circulating inhibitors and three were positive for human immunodeficiency virus (HIV)-1 antibody. Central venous lines were placed to facilitate intermittent factor replacement therapy (n = 6), long-term factor prophylaxis (n = 9), induction of an immune tolerance protocol (n = 2) or therapy for acquired immunodeficiency syndrome (AIDS)-related complications (n = 2). The ports remained in place for 15795 days (mean 687 days, range 11-2059 days). The frequency of port-related sepsis was 48% (11/23 ports in eight boys) or 0.7 port infections per 1000 patient days. Ports were removed from five boys with an unresolved infection (four with Staphylococcus aureus sepsis and one with Pseudomonas sp. sepsis). Other complications requiring port removal included a catheter tip placed too high in the venous system (n = 1), severe persistent pain associated with needle access of the port (n = 1) and a subclavian vein thrombosis (n = 1). Both the benefits and risks of a subcutaneous port should be considered when deciding whether to place this device in a very young child with haemophilia.

Tracheomalacia describes a generalized or localized weakness of the trachea that results in excessive narrowing of the tracheal lumen during expiration or whenever intrathoracic pressure increases. Severe forms of tracheomalacia are characterized by life-threatening apneic spells, inability to extubate the airways, and episodic pneumonia. Aortopexy has proved to be an effective method of treatment for most of these conditions when seen in children. When aortopexy fails to prevent tracheal obstruction, a support applied to the external circumference of the trachea can be useful to prevent airway collapse.

The purpose of this study was to assess the accuracy of CT for determining surgical resectability of hepatoblastoma before and after chemotherapy.Preoperative and postoperative CT scans of 15 children with surgically confirmed hepatoblastoma were retrospectively reviewed and correlated with surgical findings and pathologic analysis.Before chemotherapy, 12 of the 15 children had unresectable tumors because of bilobar involvement, metastatic disease, or vascular extension. After chemotherapy, tumor volumes decreased by 20-98%, with increased areas of low attenuation and calcification. Periportal areas of low attenuation (four of nine), abdominal adenopathy (four of four), and lung nodules (four of six) disappeared after chemotherapy. On the basis of CT and clinical findings, all tumors were thought to be resectable. Surgical correlation showed that the extent of hepatic tumor had been overestimated on immediate preoperative CT scans in three children (tumor was staged as bilobar, but only single-lobe resection was required). Correlation between areas of low attenuation in the tumor and necrosis in pathologic specimens was poor. Tumor invasion of periportal lymphatics was seen in one of five children with periportal areas of low attenuation. Postoperatively, five of 13 children had CT abnormalities, three at the resection margins with calcification, low attenuation, or both. Hepatic or abdominal disease has not recurred, and 12 of the 14 children who survived surgery are well. One child who had abnormal findings on preoperative chest CT died of pulmonary metastases. Another in whom chemotherapy was limited because of toxic effects died after tumor recurred in the liver.Pretreatment CT scans cannot be used to predict ultimate resectability of hepatic tumors; nor are preoperative scans always accurate for judging exact lobar involvement. Absence of disease as shown on CT scans correlated with absence both at surgery and at follow-up. Postoperative hepatic changes are common and do not necessarily reflect recurrent or residual tumor.

Since January 1986, 15 children with hepatoblastoma received three to six cycles of chemotherapy with cisplatin (20 mg/m2/day x 5 days) and doxorubicin hydrochloride (25 mg2/m2/day x 3 days) every 3 weeks before surgery. The extent of the tumor was defined by computerized tomography scan of abdomen and chest and diagnosis confirmed by percutaneous liver biopsy before therapy was started. Tumors in 10 children were considered unresectable at diagnosis because of pulmonary metastases, extensive bilobar involvement, or venous involvement. Volume of tumor reduction ranged from 35% to 95% and was independent of tumor histologic findings. After a minimum of three chemotherapy cycles, excision was undertaken when tumor volume decreased to a size at which hepatic resection was feasible and safe. Chemotherapy complications that were not serious did not delay surgery or result in tumor growth during treatment. Complete surgical excision was possible in 13 children, including 10 who had had unresectable tumors and five with pulmonary metastases. Only three resections of more than one liver lobe were required, and partial lobectomy was possible in four children. One operative death and three postoperative complications, one severe, occurred. Cyclic chemotherapy was restarted 4 to 6 weeks after surgery until a total of six courses were given. Twelve children (eight whose tumors were originally unresectable) completed treatment 3 to 56 (median, 21) months ago and have no evidence of disease. Two other children currently undergoing therapy may have residual disease. The results to date far surpass historic survival rates, which ranged about 25%.

A series of 18 children suffering from persistent idiopathic neonatal hypoglycemia (PINH) is reported. Medical and surgical managements are described in detail. All patients subjected to surgery had failed medical treatment. These patients were divided into two groups: 1) 85% pancreatectomy leaving the uncinate process in situ, and 2) 95% pancreatectomy leaving a small rim of pancreatic tissue along the duodenum and the common bile duct. The spleen was preserved in all cases. Two out of 5 children of group 1 required further resection of the pancreas for persistent hypoglycemia and were converted to 95% pancreatectomy. Since 1981 95% pancreatectomy was exclusively employed. Only one patient required insulin for 3 weeks postoperatively. Histopathology and immunohistochemistry revealed islet cell dysplasia and islet cell nuclear hypertrophy in the majority of cases, 35% of the patients had focal adenomatosis. Better control of hypoglycemia is achieved by primary 95% pancreatectomy and, thus, 95% pancreatectomy is recommended as the initial procedure in the treatment of PINH.

Antireflux surgery was performed in 234 children over a 5-year period; 153 were neurologically impaired (NI) and 81 were neurologically normal (NN). Initial presentation, demographic data, and type of antireflux operation were similar in the two groups. Eighty-six percent of the NI group versus 30% of the NN group had gastrostomy tubes placed. The incidence of late postoperative complications was 26% in the NI group and 12% in the NN group (P less than .01). During the late postoperative period, NI children underwent reoperation four times as frequently as NN children (19% v 5%, respectively; P less than .01). Wrap herniation accounted for 38% of complications and 59% of reoperations in the late postoperative period. Mortality due to aspiration occurred in 9% of the NI group versus 1% of the NN group. Combined failure rate (reoperation plus aspiration-induced deaths) was 28% in NI and 6% in NN (P less than .01). We conclude that neurological status is the major predictor of operative success and that wrap herniation due to crural disruption is the most common cause of operative failure.

We present the case of an infant with a cystic hygroma investigated by lymphoscintigraphy. The source of lymph flowing into the hygroma was identified as were some ectatic lymphatics feeding it. Lymph flow was very rapid. The implications for therapy are discussed. Lymphoscintigraphy can assess many aspects of lymph flow not accessible by other means.

Change in abundance of /sup 2/H and /sup 18/O in dietary water during a doubly labeled water energy period may introduce error into the calculated carbon dioxide production rate (RCO/sub 2/). To examine the accuracy of /sup 2/H/sub 2/(/sup 18/)O during changing nutritional regimens, we compared /sup 2/H/sub 2/(/sup 18/)O and periodic open-circuit respiratory gas exchange (RGE) in postsurgical infants who were changing from parenteral to alternative parenteral and/or oral nutrition. The two methods were compared before and after correction for shifts in isotopic abundance of the infant water pools during the energy-expenditure period. Baseline corrections were predicted using the difference between abundances of the initial body water and final nutrient solutions. Before isotopic correction, /sup 2/H/sub 2/(/sup 18/)O underestimated RCO/sub 2/ in eight subjects by 11.8 +/- 20.1% (mean +/- SD). After correction, agreement between the two methods improved; the underestimate was then -8.7 +/- 12.9%. To obtain maximum precision of /sup 2/H/sub 2/(/sup 18/)O, subjects should be maintained on the same nutritional regimen before and during the study unless valid correction formulae are used.

Several different malignant and benign tumors of the liver are seen in children. They pose a significant challenge for the pediatric surgeon because they reach a very large size before discovery and hepatic resection is usually necessary for cure. Surgical mortality and morbidity are still significant.

The combination of isotope scanning and angiography is extremely valuable in the preoperative and postoperative evaluation of abdominal diseases, particularly hepatic disease. This technique can accurately detect, show the extent of and in many instances predict the nature of various hepatic abnormalities.Armed with this information, the pediatrician or surgeon is able to best decide whether surgery, irradiation or medical management are indicated.

Evaluation of 115 children who had irradiation to the liver reveals an increase in abnormality of the parameters studied when large volumes of liver are irradiated or when the liver is irradiated following resection. To some extent, this is dependent on dose but abnormalities have been observed at doses below 2,499 rads and as low as 1,200 rads. Possibly, this may be related to the increased sensitivity of the younger child who generally received these lower dosages.Although evaluation in the chronic phase shows no significant difference of abnormality of age as related to dose, there does appear to be a slight trend in the acute phase of irradiation to implicate sensitivity of children under 1 year.

Hepatoblastoma was associated with signs of isosexual precocity in an 11-month-old boy. Circulating gonadotropic hormone levels were elevated. Gonadotropic activity was demonstrated by bioassay in extracts of primary and metastatic tumor, and in media surrounding long-term tissue culture of the primary tumor. Morphology of the endocrine glands and bioassay of the pituitary gland obtained at autopsy indicated the presence of gonadotropins and other tropic hormones despite the physiologically abnormal hormone status. These ae the first direct studies of pituitary hormone content in this syndrome.

Severe stress can produce ulceration and uncontrollable hemorrhage in the pediatric age group. Modern angiographic techniques, using embolization of a Silastic balloon to occlude a visualized bleeding vessel, can successfully control hemorrhage and avoid surgical intervention and its resultant disordered gastric physiology. Recently this technique was used to control hemorrhage from the gastroduodenal artery in a 1-year-old child with severe respiratory sepsis. This is the youngest reported child in whom the technique has been used to control bleeding from this vessel.

Circular myotomy of the proximal esophageal pouch has allowed end-to-end esophagoesophagostomy in infants with wide gap esophageal atresia. Occasionally, the proximal pouch ends so high in the thorax that the circular myotomy is technically impossible under conventional means. Exteriorization, mobilization, and myotomy of the proximal esophageal pouch via a separate cervical incision has allowed us to overcome this limitation of an inaccessible upper pouch and has permitted esophagoesophagostomy. This technique has been performed successfully in two infants with good results and no morbidity over a follow-up period of 1 1/2 to 3 1/2 years.

Injury patterns and use of passenger restraints were studied in 91 children injured while riding in motor vehicles and admitted to The Hospital for Sick Children, Toronto, from June 1984 through December 1985. Of theses, 44 had used restraints and 38 had not. Nine were excluded from the study because restraint use could not be determined. Age and sex distributions were closely matched in both study groups. The no-restraint group had more massive head injuries, thoracic injuries, and liver and spleen injuries than the restraint group. Classic "seatbelt syndrome" injuries were seen in the seatbelt group. The overall Injury Severity Scores were not significantly different between the groups. Despite mandatory legislation, many children do not use restraints, and many who do still suffer severe or fatal injuries. We conclude that better compliance with existing passenger restraint laws and more effective restraint systems are needed.

Total parenteral nutrition TPN has been used to treat or prevent malnutrition in 65 children with a variety of solid tumors and leukemia in the past 7 years. TPN was used in 58 patients with gastrointestinal complications of surgery, chemotherapy, or radiation; in 2 patients for preoperative correction of malnutrition; and in 8 who were entered into a prospective study. During TPN, general nutrition and appearance improved in all patients. Weight gain was noted in most. Despite gastrointestinal complications, which usually require the interruption of chemotherapy and radiation, treatment could be continued at full dose in 31 children with nutritional support by TPN. TPN was discontinued in 6 patients when blood cultures became positive. Sepsis was treated successfully by removal of the central venous catheter in all 6 and administration of antibiotics in 3. TPN appears to be safe and effective means of combating malnutrition which may occur with cancer and its therapy.

Acute and chronic starvation is often associated with childhood cancer. Total parenteral nutrition (TPN) with 20% glucose and 3.0% amino acids, and minerals and vitamins was instituted to treat or prevent malnutrition in 41 children with cancer, ages three months to 18 years. TPN was required for anorexia, vomiting and diarrhea associated with anti-cancer therapy in 33 patients for intestinal complications or surgery in nine, and for preoperative correction of malnutrition in two. During TPN, general nutrition and appearance improved in all patients. Weight gain was noted in most. Despite gastrointestinal complications which usually require the interruption of chemotherapy and irradiation, in 21 children treatment could be continued at full dose with nutritional support by TPN. TPN was discontinued in six patients when blood cultures became positive. Sepsis was treated successfully by removal of the central venous catheter in all six and administration of antibiotics in three. No metabolic complications were noted. TPN appears to be a safe and effective means of combating the malnutrition which may occur with cancer and its therapy.

Forty children with localized resectable intestinal non-Hodgkin's lymphoma were seen between 1948 and 1974. Survival was related to the extent of disease at presentation and to therapy. No deaths occurred after 15 months and no recurrences occurred after 13 months after diagnosis. Six of eight stage IE patients (75%) and nine of 29 stage IIE patients (31%) have survived a minimum of 2 years; one of the three stage IVE patients has survived 17 years. Unfavorable prognostic findings at surgery were serosal involvement, presence of tumor at the surgical margins, mesenteric nodal involvement, and the presence of abdominal fluid or blood. Paraortic nodal involvement or multiple primary foci were universally fatal. Eight of 11 patients (73%) treated with surgery and whole abdominal irradiation (greater than or equal 2000 rad) with or without chemotherapy have survived. Seven of 18 patients (39%) treated with surgery and low dose chemotherapy have survived. One of seven patients treated with surgery and localized or low dose radiation therapy with or without chemotherapy has survived. Four patients treated with surgery alone died. Bone marrow and central nervous system involvement occurred after previous disease relapse elsewhere.

Four patients are described who have manifested unusual reactions to combined irradiation and chemotherapy when irradiation of the liver was involved. Two had had partial resection of the liver and irradiation of all of the remaining liver tissue; each of the others, with an intact liver, may have had compromise of liver function by infection and/or by lower dose irradiation of the left lobe of liver.We suggest that irradiation of the liver, especially in patients who receive chemotherapeutic agents which may require liver for detoxification or excretion, be very carefully administered, even at a minimal dose. Any underlying compromise of the liver must be evaluated carefully. In each patient, hemoglobin levels were normal throughout the clinical course.

A 21-month-old male with congenital aplasia of the thymus (DiGeorge's syndrome) underwent implantation of thymus fragments from a 16-week-old female foetus. Abnormalities of lymphocyte function as manifested by failure to reject a skin allograft, to develop delayed hypersensitivity to Monilia and dinitrofluorobenzene, or to respond to phytohaemag-glutinin were promptly rectified. There was no evidence of a graft-versus-host reaction.