Superficial carotid artery secondary to cervical teratoma.

Cervical teratomas are rare tumors, presenting as a mass in the neck at birth. Affected infants usually have respiratory compromise and dysphagia, secondary to tracheal and esophageal compression. Treatment consists of airway control, followed by surgical excision, which may be complicated by marked displacement of normal tissues.

Fine needle aspiration cytology of pancreatoblastoma with immunocytochemical and ultrastructural studies.

The cytologic features of a pancreatoblastoma (infantile adenocarcinoma), a rare pancreatic neoplasm of childhood, are described. Fine needle aspiration (FNA) under ultrasound guidance produced a hypercellular specimen consisting of numerous oval-to-cuboidal cells that had a moderate amount of granular cytoplasm. Spindle-shaped, elongated and triangular-shaped epithelial cells were also seen, along with smaller cells that had a higher nuclear/cytoplasmic ratio and a denser cytoplasm.

Thoracic neuroblastoma presenting as a cystic hygroma.

A case of thoracic neuroblastoma with extension into the supraclavicular fossa is described. By physical examination and ultrasound the patient was thought to have a cystic hygroma with mediastinal extension. A 'cystic' neck mass may be the initial presentation of a mediastinal neuroblastoma.

Clinical utility of fine needle aspiration in the diagnosis and management of neuroblastoma.

Eleven fine needle aspiration (FNA) biopsies were performed in five children with neuroblastoma, including one patient with peripheral neuroectodermal tumor of the thoracopulmonary region (Askin tumor). Cytologic features in conjunction with immunocytochemistry and electron microscopy on the aspirated material enabled us to make a primary diagnosis in four of the five patients and diagnose local recurrence and metastatic disease in three patients. There were no false-positive or false-negative cytologic diagnoses; therefore, diagnostic accuracy was 100%.

Failure of systemic thrombolytic and heparin therapy in the treatment of neonatal aortic thrombosis.

An unsuccessful attempt was made to lyse a large aortic thrombus in a newborn using systemic high-dose streptokinase and urokinase therapy and subsequently the use of heparin failed to prevent the propagation of thrombus. The patient was a seven-day old premature, sick neonate in whom an aortic thrombosis developed following umbilical artery catheterization. Surgical thrombectomy could not be performed in this patient, and local thrombolytic therapy was not technically feasible.

Chest radiographic findings in neonates on extracorporeal membrane oxygenation.

Eight full-term neonates with pulmonary failure were managed with extracorporeal membrane oxygenation (ECMO). Chest radiographs obtained before and during ECMO support were reviewed. During periods of increased flow requirements, the chest radiographs were difficult to evaluate because of increasing pulmonary opacity.

Adrenal abscess in the neonate.

Adrenal abscess in the neonate is a rare complication of adrenal hemorrhage. The radiographic and clinical findings of 12 previously published cases and two new cases of adrenal abscess in the newborn are presented. Sonography was the most helpful examination in distinguishing a suprarenal lesion from an intrarenal lesion and in demonstrating the morphology of the abscess.

Ultrasound detection of focal bacterial nephritis (lobar nephronia) and its evolution into a renal abscess.

A case of acute focal bacterial nephritis (lobar nephronia) which evolved into a renal abscess is reported. This case is unusual for 2 reasons: the focal nephritis was isoechoic (not previously reported), and it progressed to an abscess despite antibiotic therapy. Ultrasound initially detected the focal nephritis and later confirmed its progression to an abscess.

Renal tubular acidosis type 4 in neonatal unilateral kidney diseases.

Three neonates, two with unilateral renal vein thrombosis and one with unilateral dysplastic kidney, developed type 4 renal tubular acidosis, manifested by nonazotemic hyperkalemic metabolic acidosis with alkaline urine pH and reduced potassium excretion. Normal plasma concentrations of sodium, aldosterone, and renin activity, together with normal renal fractional excretion of sodium, supported the diagnosis of renal tubular acidosis type 4, subtype 5. Arginine HCl loading studies showed that despite their ability to bring the urine pH to less than 5.

Chronic granulomatous disease of childhood. Probable diffuse gastric involvement.

Diffuse gastric infiltration in a child with CGD is described as a cause for gastric outlet obstruction.

Short rib-polydactyly syndrome type II (Majewski syndrome): a case report.

The Majewski type of Short Rib-Polydactyly syndrome is a rare lethal dwarfism syndrome that has recently been recognized as a distinct entity. The full range of associated anomalies remains to be described. This case report details the clinical and autopsy findings in this condition and reviews the differential diagnosis of polydactylous dwarfing syndromes.

Ultrasonographic diagnosis of gangrenous bowel in neonatal necrotizing enterocolitis.

The identification of gangrenous bowel in neonates with enterocolitis is essential for proper timing of surgical intervention. The use of sonography to identify the presence of gangrenous bowel is illustrated.

Colitis cystica profunda: a pediatric case report.

A case of colitis cystica profunda in a 14-year-old male is presented. The clinical and pathologic management are reviewed. Differentiation from adenocarcinoma by careful histologic evaluation of the epithelium is fundamental in avoiding overtreatment.

Midline cerebral arterio-venous anomalies: ultrasound diagnosis.

Ultrasound features of three cases of midline cerebral arterio-venous anomalies of neonates presenting with congestive heart failure are presented. Two cases have CT and angiographic correlation. The third case has post-mortem correlation.

Cheirolumbar dysostosis: a phenotype of pseudohypoparathyroidism.

Cheirolumbar dysostosis is a skeletal dysplasia characterized by brachydactyly and stenosis of the lumbar vertebral canal. Our recent experience with a patient having these clinical features indicates that this condition may be a phenotype of pseudohypoparathyroidism, rather than a distinct entity.

Effects of 1,25-dihydroxyvitamin-D3 on renal function, mineral balance, and growth in children with severe chronic renal failure.

To confirm and extend previous observations of enhanced linear growth in children with chronic renal disease being treated with 1,25-dihydroxyvitamin-D3 and to characterize further the calcium, phosphorus, magnesium, and zinc disorders in renal failure, 11 children (mean age 8 +/- 5 years) with chronic renal insufficiency (glomerular filtration rate 18% +/- 13% of normal) were evaluated on the basis of their reciprocal serum creatinine concentrations, height-velocity curves, mineral balances, and radiologic findings. Reciprocal serum creatinine concentrations analyzed retrospectively and prospectively during 32 months of 1,25-dihydroxyvitamin-D3 therapy showed progression of renal failure at rates linearly identical with those before treatment, thus suggesting that the treatment did not accelerate the rate of deterioration of glomerular filtration rate in chronic anal insufficiency. Indeed, one patient manifested a lesser decline in renal function (P less than .

Spondylocostal dysostosis associated with anal and urogenital anomalies in a Mennonite sibship.

Spondylocostal dysostosis, anal atresia, and urogenital anomalies were observed in two male infants of a consanguineous Mennonite couple. A careful review of previously reported syndromes of severe vertebral abnormalities and/or imperforate anus suggests that our patients have a previously undescribed recessive disorder that should be included in the differential diagnosis of rib and vertebral anomaly syndromes.

Management of congenital microgastria with a jejunal reservoir pouch.

A 3-mo-old female presented with growth retardation, vomiting, reflux esophagitis, recurrent aspiration pneumonias, and was found to have megaesophagus and microgastria. After the failure of conservative therapy a double-lumen jejunal (Hunt-Lawrence) pouch with distal Roux-en-Y anastomosis was anastomosed to the stomach to increase the gastric reservoir. One year later, there has been progressive weight gain, the megaesophagus and gastroesophageal reflux have lessened significantly, pneumonia has not recurred, and the tracheobronchitis and esophagitis have resolved.

Focal nodular hyperplasia of the liver in children: a report of three new cases.

Three cases of focal nodular hyperplasia of the liver in children are presented. The angiographic findings of a hypervascular mass with dense, delayed capillary stain in combination with increased uptake of 99mTc sulfur colloid on radionuclide scans were present in two cases. Computed tomography, utilized in one case, demonstrated a well-circumscribed mass with an irregular stellate area of low density which corresponded to the central collagenous scar described pathologically.

Grand round series: hypoglycemia and unilateral pulmonary edema in a newborn.

A case of cardiomegaly with unilateral pulmonary edema is presented. The predisposing etiologies were severe hypoglycemia and a hypertonic glucose infusion into a branch of the left lower lobe pulmonary vein. Resolution resulted following maintenance of adequate glucose levels, vigorous cardiopulmonary support, and repositioning of the catheter tip in the inferior vena cava.