Pediatric total parenteral nutrition. Liver histopathology.

Correlation of clinical data with hepatic histopathology from 31 infants receiving total parenteral nutrition (TPN) suggest chronologic progression of liver disease with long-term TPN. Steatosis and a prominent eosinophil component in portal-tract extramedullary hematopoiesis appear during the first five days of TPN. The former persists for 90 days, the latter for three weeks. Canalicular cholestasis was present after ten days in 84.2% of the livers studied and bile duct proliferation in 63.6% after three weeks of TPN. Moderate to severe portal fibrosis only occurred after 90 days, whereas micronodular cirrhosis developed in one patient after five months of TPN. Lipofuscin-like pigment and hemosiderin were each demonstrated in 90.3% of the livers studied. Our findings suggest that with up to 90 days of TPN most changes should either be reversible or not severe enough to result in liver failure.

G syndrome: a review of the literature and a case report.

In 1969, Opitz et al. reported the first case of G Syndrome (Opitz-Frias Syndrome). They presented their clinical observations on 4 brothers from the 'G Family' who shared a constellation of findings with a generalised tendency to midline defects. Major manifestations of this multiple congenital anomaly syndrome include hypertelorism, prominent forehead, cleft lip and palate, narrow palpebral fissures with epicanthal folds, dysphagia, stridor, laryngotracheal oesophageal clefts, and hypospadias. The most significant manifestation of a midline field developmental defect in these infants is aspiration which poses the greatest threat to life. Urgent evaluation should include a water-soluble contrast oesophagram and aerodigestive endoscopy. In addition to repair of the laryngeal cleft, which occurs in approximately 30% of the cases, a tracheostomy, feeding gastrostomy, and Nissen fundoplication are often needed. We report a male infant born with G. syndrome. BBB Syndrome (Opitz Syndrome) is also discussed which bears a striking resemblance to the G Syndrome; in fact, they may be variants of the same allelic syndrome.

Gonadoblastoma in infancy: indications for early gonadectomy in 46XY gonadal dysgenesis.

A 15-month-old phenotypic female was diagnosed as having 46XY gonadal dysgenesis following karyotyping for Turner-like features. Gonadectomy at the time of bilateral ureteral reimplantation for vesicoureteral reflux showed gonadoblastoma. This case, in which malignancy was already established at 15 months, indicates the need for early gonadectomy, which should be performed at the time of diagnosis.

Feeding gastrostomy button: experience and recommendations.

To circumvent the common problems and complications of long-term gastrostomy management, a skin-level nonrefluxing nonreactive gastrostomy "button" (GB) was designed. This silicon device is self-retaining, employing a one-way (in) valve. For feeding purposes, an adapter is inserted. Since initial results with five prototypes were encouraging, our experience with 90 buttons over a 20-month period forms the basis of this report. The GB is commercially available in three sizes: "small" (1.5 cm shaft length), medium (2.7 cm), and long (4.3 cm). A new narrow shafted prototype with an intermediary shaft length of 2.3 cm is under evaluation. We have analyzed our experience with the GB in 50 children (average age 4.1 years, average weight 12.8 kg) and six adults (average age 75 years) during this span. Ninety GBs were employed (small 46, medium 31, long 7, prototype 6) for a total of 485 patient months. In the pediatric age group, the average time with one or more GBs in place was 8.9 months. Of the 50 children, 30 had one GB only, 11 had two, six had three, and three had four GBs (total 82 GBs). The performance of each GB was under 1 month, 3 (mechanically defective); 1 to 3 months, 20; 3 to 6 months, 25; 6 to 12 months, 26; and over 12 months, 8 (two at 20 months). There were 31 changes in 20 children: leakage through GB shaft, 23 (valve incompetence); accidentally pulled out, 4; leakage around shaft (poor stoma), 2; and deterioration at feeding connection end, 2. One additional GB was purposely temporarily removed when the child underwent a colostomy closure. Seven GBs in children were removed since they were no longer needed. In two children, the GBs were replaced by a conventional catheter at 1 and 6 months post-GB insertion (chronic intermittent bowel obstruction requiring frequent drainage in one and multiple removals of the GB by the child in the other). Difficulties with early feeding adapters (frequent disconnections or shaft stretching) were encountered in five, but this is no longer a problem.(ABSTRACT TRUNCATED AT 250 WORDS)

Streptokinase dissolution of a right atrial thrombus.

A critically ill 20-month-old white male with severe bronchopulmonary dysplasia, cor pulmonale, and intolerance to enteral feeds, required a left percutaneous subclavian Broviac catheter. Echocardiogram showed a right atrial thrombus, resolution of which was demonstrated after infusion of streptokinase through the Broviac catheter for 16 hours. The catheter remained in place, functioning well, for a further 4 months. The patient suffered no bleeding complications, and was spared the prohibitive risk of right atrial thrombectomy. This use of streptokinase has not previously been reported.

Surgery in patients with cystic fibrosis.

A 15-year review of children's hospital patients with cystic fibrosis (CF) who underwent surgery yielded 578 cases in 210 patients (mean 2.7 per patient). The median age was 16 years (range newborn to 43 years). Four hundred procedures were done under general anesthesia and 176 under local. There was one anesthetic complication, respiratory depression in a patient whose MediPort (Cormed, Inc, Medina, NY) was inserted using local anesthesia and sedation. The most frequent procedure was nasal polypectomy, with 165 procedures in 50 patients. The second most common procedures were vascular access procedures: 75 central lines and 29 MediPorts were implanted in 57 patients, complicated by two pneumothoraces. Thoracic procedures included 32 bronchoscopies, 8 lobectomies, 2 pneumonectomies, and 30 pleural strippings. There were three reoperations for bleeding in the pulmonary resection patients. Thirteen newborns underwent a total of 26 procedures for meconium ileus and its complications, with two deaths secondary to respiratory failure and sepsis. These, and one death postlobectomy were the only operative deaths in the entire series of 578 cases (0.5% mortality rate). There were four slings for rectal prolapse; two required removal secondary to infection. Eight patients underwent central splenorenal shunts for portal hypertension, 15 underwent cholecystectomy, 5 underwent Nissen fundoplication, 16 underwent inguinal herniorrhaphy, 2 underwent umbilical herniorrhaphy, 3 underwent orchidopexies, and 4 underwent miscellaneous pediatric surgical procedures. Eleven patients underwent appendectomy for appendicitis; four were ruptured at the time of diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

The spectrum of meconium disease in infancy.

Meconium diseases in infancy cannot be neatly separated into discrete categories of meconium plug syndrome, meconium ileus, and meconium peritonitis; nor can the therapy of each condition. A patient with any of the three may or may not have cystic fibrosis. All babies with any form of meconium obstruction or atresia must have a sweat chloride test to confirm or rule out this diagnosis. Repeated gastrografin enemas can decrease the need for operative intervention in all babies with meconium plug syndrome and in selected babies with meconium ileus. Babies with meconium diseases can now be managed with a low perinatal mortality.

Endodermal sinus tumor: a clinical and pathological correlation.

Endodermal sinus tumors occur in the gonads and in a variety of extragonadal locations, particularly as the malignant component of sacral teratomas. Light and electron microscopic studies as well as the presence of alpha fetoprotein support the concept that these lesions arise from germ cells that migrate from the extraembryonic yolk sac. These tumors in all locations are highly malignant and metastasize to lymph nodes, lung, liver and bone. Peritoneal implants are seen from tumors in the ovary, testis, and following ventriculoperitoneal shunts for yolk sac tumors of the pineal gland. There is no satisfactory therapy for the endodermal sinus tumor, regardless of location, when there are distant metastases at the time of diagnosis; however, we have documented complete regression of metastases in one child following therapy with vinblastine, bleomycin, and cis-platinum. Nine of 13 boys with endodermal sinus tumors of the testis survived in this series of patients. All survivors were treated with orchiectomy and lymph node excision. Surgical excision combined with chemotherapy and radiation led to survivors in children with large extragonadal pelvic and retroperitoneal tumors. We recommend vigorous multimodal therapy with surgery and chemotherapy. Radiation is given to the primary lesion when it is too extensive for initial resection or to eradicate residual disease following chemotherapy. Alpha fetoprotein is a valuable tumor marker.

A spectrum of bronchopulmonary anomalies associated with tracheoesophageal malformations.

The combination of tracheoesophageal and pulmonary malformations is unusual and reportedly carries a high mortality. We have observed six patients with esophageal atresia and tracheoesophageal fistula and one with a bronchoesophageal fistula who had associated bronchopulmonary anomalies ranging from lobar hypoplasia and agenesis to unilateral pulmonary hypoplasia or agenesis. All of the pulmonary malformations were right-sided. Vertebral or rib anomalies were present in five patients, congenital heart disease in two, imperforate anus in one and one patient had radial aplasia and Pierre Robin syndrome. The bronchopulmonary anomalies complicated the surgical care of tracheoesophageal malformations and required radiologic differentiation from aspiration pneumonia and atelectasis. Six of the seven patients survived. Mortality and morbidity were related to complications and associated cardiac anomalies as well as severity of the lung anomaly.

Giant nonfunctioning islet cell tumor requiring pancreaticoduodenectomy and complete liver revascularization.

Pancreaticoduodenectomy with revascularization of the hepatic artery and portal vein was performed on a 17-year-old girl with giant nonfunctioning islet cell tumor of the pancreas. She had a remote history of neonatal hypoglycemia leading to mental retardation and a right Wilms' tumor resected at 8 months. Serum pancreatic polypeptide levels were elevated. Her postoperative course was complicated by an ischemic perforation of the colon, which did not infect her prosthetic vascular grafts. The relationship between her neonatal hypoglycemia, Wilms' tumor, and subsequent islet cell neoplasm is unclear.