Metastatic congenital adrenocortical carcinoma: a case report with tumor remission at 3 1/2 years.

We describe a case of metastasizing congenital adrenocortical carcinoma and a follow-up of 3 1/2 yr. Treatment with surgery and mitotane was associated with multiple complications. The patient was in remission at 3 1/2 yr. Because of the rarity of this condition, we discuss step-by-step problems encountered during management.

Reconstruction of aplasia cutis congenita of the trunk in a newborn infant using acellular allogenic dermal graft and cultured epithelial autografts.

Aplasia cutis congenita, a congenital condition characterized by the absence of all skin layers, occurs mostly on the scalp, but may also involve the trunk and extremities. Conservative treatment using silver sulfadiazine with daily dressing change has been recommended to avoid donor site morbidity and operative risks to the neonate who may have other congenital defects. Others have proposed early surgical intervention to prevent problems associated with delayed wound healing. In this case of a newborn, the authors used an alternative therapy to avoid delayed wound healing and multiple surgical interventions. During one operative procedure, a 130-cm2 lesion on the trunk was covered with allogeneic dermis and cultured epithelial autografts (CEAs). After 2.5 weeks and with three additional applications of CEAs at bedside, 90% of the wound had healed. At 27 months, the grafted area was smooth, and pliable with normal skin texture.

Spastic diplegia as a complication of interferon Alfa-2a treatment of hemangiomas of infancy.

We report the development of spastic diplegia in infants during the course of interferon Alfa-2a (IFN) therapy for potentially life-endangering hemangiomas. Five infants who displayed diplegia were selected from a group of 26 infants treated with IFN. Diplegia persisted in three infants, and in the remaining two significant recovery occurred after IFN was discontinued. Magnetic resonance imaging showed no significant brain or spinal abnormalities except minor to moderate delayed myelination in two patients. Myelination was normal on subsequent radiographic examination in all five patients. We conclude that IFN can adversely affect the immature central nervous system and produce spastic diplegia, which is potentially reversible. We recommend careful clinical assessment of neurodevelopmental status during IFN therapy.

Duodenal trichobezoar caused by compression of the superior mesenteric artery.

Trichobezoars can occur in young women who have a history of trichotillomania, trichophagia, gastric dysmotility, and psychiatric dysfunction. A 6-year-old anorexic girl presented with a fixed right-upper-quadrant abdominal mass. Exploratory celiotomy for a duodenal trichobezoar led to removal of the large foreign body, via a duodenotomy, and prompted a Ladd procedure, in which the duodenum was moved from beneath the compressing superior mesenteric artery to relieve underlying duodenal narrowing.

Common etiologies and new approaches to management of poisoning in pediatric practice.

Accidental exposure to drugs and household products in young children and self-poisoning by adolescents are common clinical problems of important concern to the general pediatrician. This article reviews new developments in the field of pediatric toxicology including 1) epidemiologic data collected from poison centers and hospital admissions that illustrate the changing spectrum of pediatric poisonings; 2) progress in identifying clinical features or laboratory tests predictive of outcome in specific poisoning; 3) advances in and limitations involving decontamination methods and antidote therapy in different toxic ingestions; and 4) recent investigations toward prevention of pediatric poisoning with an emphasis on barriers to compliance.

Corticosteroids in the management of unresected plasma cell granuloma (inflammatory pseudotumor) of the lung.

The case of a 10-year-old girl with plasma cell granuloma of the lung and an associated hypergammaglobulinemia is reported. A thoracotomy and biopsy were done. Total resection would have required a pneumonectomy. She was treated with an immunosuppressive course of prednisone, which resulted in a reversal of the infiltrative process and the hypergammaglobulinemia.

Plexiform neurofibromatosis of the liver and mesentery in a child.

Plexiform neurofibromatosis of the liver was recognized by needle biopsy of the liver in an 11-yr-old boy who had a 2-yr history of diarrhea, intermittent abdominal pain, failure to gain weight and progressive abdominal distention. Imaging studies demonstrated a large retroperitoneal mass; a laparotomy was performed. At surgery, the mesentery was greatly thickened by neurofibromas, and plexiform neurofibroma extended through the hilum of the liver. Light and electron microscopy demonstrated that in addition to the direct involvement by tumor, neural hyperplasia existed throughout the liver. The most distal ramifications of the portal spaces were filled with Schwann cells, bundles of unmyelinated nerves and perineurium-surrounded nerves containing myelinated and unmyelinated fibers. The ultrastructural findings were consistent with stimulation of proliferation of all the portal neural elements and tumoral tissue. The nontumoral response was more than simple hyperplasia because it appeared to result in fibrotic changes in the most involved areas and active breaching of the limiting plate with destruction of hepatocytes and collagen deposition throughout the liver.

Superior mesenteric artery syndrome associated with scoliosis treated by a modified Ladd procedure.

A 16-year-old asthenic girl had idiopathic adolescent scoliosis and superior mesenteric artery (SMA) syndrome. After a Harrington rod procedure, the SMA syndrome produced a complete duodenal obstruction that did not resolve with nasogastric decompression, positioning, or peripheral intravenous nutrition. A complete derotation of the duodenum and the colon and stabilization of their mesentery (Ladd procedure with the Bill modification) resolved the obstruction and was considered the preferred operative treatment for this problem.

Inflammatory pseudotumor of the liver: a rare cause of obstructive jaundice and portal hypertension in a child.

Inflammatory pseudotumor (IPT) of the liver and intrahepatic bile ducts is a rare cause of obstructive jaundice and portal hypertension in the pediatric age group. Because it seems to have a better long-term outcome than many of the conditions with which it may be confused, it is important to recognize the radiologic and pathologic features of this rare lesion so that appropriate therapy may be instituted.

Abnormalities of the vas deferens and epididymis in cryptorchid boys with congenital rubella.

Cryptorchidism was present in 12% of 316 boys with congenital rubella (CR) followed by The Roosevelt Hospital Rubella Project. Eight of these patients, age 4 through 16 yr, had a recent orchiopexy, 4 on the left, 2 on the right, and 2 bilaterally. The vasoepididymal system was absent or apparently obstructed in 60% of the 10 sides. The epididymis was abnormal in 6 instances and the vas deferens in 5. Sixty-one boys of the entire series had an intravenous pyelogram (IVP) that was significantly abnormal in 18%. The 8 patients described all had a normal IVP except for 2 who had a malrotated kidney on the involved side. In 5 of the 8, a known maternal rubella infection has occurred during the first 8 wk of gestation. As the rubella virus is known to interfere with cellular growth and tissue differentiation in early pregnancy, it apparently altered the developing testis and mesonephric duct system. Similar vasoepididymal abnormalities have been described previously in patients with uncomplicated cryptorchidism, inguinal hernia, kidney defects, cystic fibrosis and male sterility. Their presence should alert the clinician to perform an IVP and also consider a diagnosis of congenital rubella.

Ventral hernia with a skin-covered Silastic sheet for newborn infants with a diaphragmatic hernia.

The persistently high mortality rate for newborn infants with a congenital diaphragmatic hernia, which is symptomatic and treated in the first 24 hours of life, is due to multiple pulmonary and vascular factors. This demands the exclusion of any additional compromising elements. The effects of increased intra-abdominal pressure due to replacement of the intestine into the abdomen can be avoided by the formation of a ventral hernia. A large hernia can be produced best by suturing a sheet of Dacron reinforced Silastic to the fascia of a midline abdominal incision and covering it with skin flaps. This nonreactive prosthesis will remain in place for several months and later can be removed simply. This technique was used successfully in three infants and should improve the survival rate of precariously balanced newborn infants operated upon during the first few hours of life.

Outpatient management of pediatric surgical problems.

A number of pediatric surgical problems commonly seen in the outpatient department or physician's office are reviewed. It is particularly important for the primary care physician to recognize the difficulty of accurate diagnosis in children and the advantage of sedation in improving patient cooperation. Hospitalization may be necessary for adequate observation, proper preparation for diagnostic studies, or safe treatment. Simple traumatic injuries or infections can usually be treated effectively without hospitalization if sound surgical principles are observed. Abdominal pain or rectal bleeding calls for a careful investigation for early signs of inflammatory disease or life-threatening lesions requiring surgical treatment.

Evacuation of pericardial, anterior mediastinal, and peripleural air collections in neonatal respiratory distress.

Alveolar rupture in the neonate can result in the dissection of air along vascular sheaths and the formation of abnormal collections within the mediastinal, pleural, pericardial, or peripleural spaces or elsewhere. Intercostal tube placement for pneumothorax is frequently required. A method is presented for the placement of Intracath catheters into pericardial, anterior mediastinal, and peripleural collections of air when other adjustments in treatment have not allevaited clinical distress.