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. We present a case in which displacement of neck contents by mass effect resulted in a carotid artery course as close as 2mm from the skin surface. Radiographic and ultrasonographic documentation is included.

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. In addition, there were abundant fragments of stroma present, including some surrounded by epithelial cells. Immunoperoxidase studies performed on the aspirated material revealed positive staining of the epithelial cells for cytokeratin (AE1/3), including high and low molecular weight cytokeratin, carcinoembryonic antigen, neuron-specific enolase and alpha-1-antitrypsin. Ultrastructural examination demonstrated epithelial cells containing either large electron-dense zymogen granules in the range of 400 nm to 600 nm or small dense neuroendocrine granules measuring from 100 nm to 200 nm. This finding, in concert with the immunocytochemical studies, supported a "blastemal" cell origin with bidirectional differentiation for this unusual pancreatic neoplasm and enabled a specific preoperative diagnosis of pancreatoblastoma to be made. The differential diagnosis of pancreatoblastoma from other pediatric neoplasms involving the pancreas, including neuroendocrine tumors and neoplasms of acinar cell derivation, is presented. We believe that the FNA cytologic findings can lead to a correct diagnosis of pancreatoblastoma, especially when coupled with immunocytochemical and ultrastructural studies performed on the aspirated material.

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%. FNA is an extremely useful technique for the primary diagnosis and management of neuroblastoma and excludes other small cell malignancies of children. The results of this study and literature review demonstrate that FNA cytology coupled with ancillary techniques of immunocytochemistry and electron microscopy is a rapid, safe, minimally invasive procedure which can aid in the diagnosis and management of patients with neuroblastoma without resorting to more aggressive diagnostic procedures in selective cases.

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. During periods of the infants' clinical improvement, their chest radiographs tended to improve as well. A direct cause-and-effect relationship between the pulmonary density and the ECMO flow requirements has not yet been established. The chest radiographs were reliable in confirming correct catheter positions. Routine daily examinations did not demonstrate unsuspected abnormality. Chest radiography during periods of clinical instability, however, added confirmatory evidence to some clinical diagnoses and assisted us in making the diagnosis of an unexpected pneumothorax.

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. Successful treatment was obtained with sonographically guided percutaneous catheter drainage in conjunction with systemic antibiotics.

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 5.8, net acid excretion was inadequate relative to the corresponding plasma bicarbonate concentration. Treatment with oral bicarbonate resulted in sustained normalization of blood acid-base status and accelerated linear growth in the first two infants, in whom spontaneous recovery occurred by ages 8 and 15 months, respectively. At that time, the affected kidneys were extremely small with distorted collecting systems; the contralateral kidneys showed compensatory hypertrophy. In the third infant, persistent acidosis and growth failure resulted from medical noncompliance; the removal of the dysplastic kidney at 7 months of age was followed by the return to normal blood acid-base status and normalized tubular hydrogen and potassium excretion. We conclude that neonatal unilateral kidney disease can result in renal tubular subtype 5. Spontaneous recovery can be expected, presumably because of " autonephrectomy " of the affected kidney plus the compensatory hypertrophy of the contralateral kidney.

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.

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.

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.

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.

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. Ultrasound easily detected the dilated midline vascular channels in all three cases. Angiography was needed to further define the abnormal anatomy. We suggest a role for ultrasound to screen for midline arteriovenous malformations in neonates with congestive heart failure of undetermined etiology.

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 .05). The height velocity of six of the children (75%) less than 12 years of age improved markedly over that expected for chronologic and bone ages after one year of treatment with orally administered 1,25-dihydroxyvitamin-D3, 15 to 35 ng/kg/day. All other medications except vitamin D2 were continued at their pretreatment dosage levels throughout the study. Growth velocity was unimproved in two of three children older than 12 years at the initiation of 1,25-dihydroxyvitamin-D3 therapy. Mineral balance data showed significant retention of calcium, phosphorus, magnesium, and zinc (357 +/- 32 mg/sq m/day, 250 +/- 82 mg/sq m/day, 38 +/- 32 mg/sq m/day, and 1,157 +/- 283 microgram/sq m/day, respectively), after treatment for 12 months. In addition, serum calcium, alkaline phosphatase, and parathyroid hormone concentrations returned toward normal. Finally, healing of renal osteodystrophy was radiologically evident after six months of therapy.

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. This suggests that the gastroesophageal reflux and megaesophagus were due to an inadequate reservoir with a secondary gastric overflow as the esophagus dilated to enlarge the reservoir capacity of the upper gastrointestinal tract. Utilization of a jejunal pouch increased the size of the gastric reservoir, allowed resolution of the secondary esophageal changes, and permitted normal growth to proceed.