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We discuss 5 children with simple renal cysts, 2 of whom were diagnosed initially as having a Wilms tumor or mesoblastic nephroma. The most common presenting feature of this lesion is a palpable mass, especially in the infant or younger child. In older children characteristic radiographic features can suggest a correct preoperative diagnosis. However, distinguishing a simple cyst and a solid tumor with necrotic degeneration may not be possible in the infant and younger child, and surgical exploration is indicated.

Two siblings with the same short limbed neonatal dwarfism are reported. The patients died from pulmonary hypoplasia.

In three patients the diagnosis of an anomalous left pulmonary artery was confirmed when tracheobronchography demonstrated a discrete extrinsic impression on the proximal right bronchus. The diagnosis was unsuspected clinically in one patient with a hyperlucent left lung on conventional chest radiographs. In the other two patients there was a suspicion of a pulmonary sling, but findings on plain chest or airway radiographs were atypical. The tracheobronchogram can be a valuable procedure for diagnosing a pulmonary sling, detecting additional developmental anomalies of the bronchi, and predicting the post-operative clinical course.

Two children are presented with similar appearing small urographic defects in the ureter and renal pelvis similar to pyeloureteritis cystica. In one patient with aplastic anemia who died the abnormalities represented areas of subepithelial hemorrhage. Another child had clinically diagnosed Henoch-Schonlein purpura and the defects were probably due to subepithelial hemorrhage.

Summary In a 3-yr period, eight infants among 43 survivors of acute NEC developed intestinal stricture. Four infants developed multiple stricture after proximal diversion procedures, and four had single strictures after medical therapy. Nineteen cases of intestinal stricture after NEC were collected from the literature. Radiographic examinations at the time of the acute disease were not predictive of the risk of subsequent stricture. Histologic examination showed various stages of wound healing, most prominently in the submucosa. Stricture should be considered as the cause of intestinal malfunction in any child who survives acute NEC.

Extracorporeal membrane oxygenation was used for cardiovascular support in 13 infants and children with complex congenital heart disease and 1 premature neonate treated in preparation for pericardial patch trachenplasty for long-segment tracheal stenosis. Nine patients were weaned from extracorporeal membrane oxygenation These were five (36%) early deaths and four (29%) late deaths. Cannulation sites included right carotid/ jugular vessels, femoral artery and vein, and right atrium and aorta. In 4 patients, the neck vessels were repaired at decannulation. Five survivors had normal growth and neurodevelopmental evaluations at follow-up. Extracorporeal membrane oxygenation can be successfully used as biventricular support in patients with intractable low cardiac output syndrome after repair of congenital heart disease. Best results are obtained in patients who have several hours of stability after operation before initiation of support. Hemorrhagic complications are reduced and long-term neurodevelopmental outcomes appear promising with right neck vessel cannulation and repair. No bleeding complications were observed in patients cannulated through the neck vessels.

Ten patients with ectopic ureteroceles associated with segmental renal dysplasia are presented. The clinical, pathologic, and radiologic features are summarized. All patients have done well following either total or heminephrectomy.

A 3-year-old child is presented with a benign anterior mediastinal teratoma that was primarily located inferior to the left lung and confused with pleural fluid. The lesion was not apparent at 4 months of age. Computerized axial tomography of the specimen clearly established the diagnosis. This procedure may be of value in selected patients with apparent pleural disease.

Nine premature infants developed radiographic and biochemical rickets at a mean +/- SD of 12.6 +/- 2.8 weeks of age. Serum 25-hydroxyvitamin D concentrations were all low, with a mean of less than 3.6 +/- 2.1 ng/ml. The mean average daily intake of vitamin D since birth had been 300 +/- 181 IU, and the mean average daily intake during the week of diagnosis was 587 +/- 313 IU. All of the infants were extremely premature (mean weight 948 +/- 153 gm, mean gestation 27.7 +/- 1.1 weeks), and were being fed either a low-calcium "human milk-like" formula or a soy formula. It is postulated that low-calcium intake may have increased 25-OHD utilization in the face of a decreased ability of the extremely premature infant to produce 25-OHD. Because of multiple factors leading to both decreased production and possible increased utilization of 25-OHD, such infants have an increased requirement for vitamin D to maintain normal serum 25-OHD concentrations, and daily intakes of at least 400 IU vitamin D orally must be assured. Serum 25-OHD measurements and radiographs may be important in following infants at risk.

Three infants with fatal aspirations during gastroesophageal studies are reported because of the rarity of this catastrophe in the literature. Recommendations for avoiding this complication are suggested.

Congenital adenomatoid malformation (CAM) of the lung was diagnosed at 30 weeks gestation. The mother presented with preterm labor and polyhydramnios. A complex cystic mass was seen in the right lung of the fetus. Additional radiographic and sonographic investigations prior to delivery allowed differentiation of this rare lesion from other cystic thoracic pathology of the fetal period. Careful hospital obstetric management of the mother and fetus for over 3 weeks allowed the delivery of an infant with adequate pulmonary maturity to permit stabilization and surgery on the baby in the first days of life.

Enzyme replacement therapy for a severely affected 6-month-old girl with hypophosphatasia was attempted by repeated intravenous infusions of alkaline phosphatase-rich plasma, obtained by plasmapheresis, from two men with Paget bone disease. Circulating Paget AP activity was found to have a half-life (two days) similar to that reported in adults, which did not change during a five-week period of six AP infusions. Normalization of the patient's serum AP activity was followed by better control of her hypercalcemia and hypercalciuria. Sequential radiographic studies revealed arrest of worsening rickets with slight remineralization of metaphyses, although urinary excretion of the AP substrates phosphoethanolamine and inorganic pyrophosphate was unaltered by therapy. Our findings suggest that the infantile form of hypophosphatasia results from defective production of AP rather than from accelerated destruction of circulating enzyme, and that hydrolysis of AP substrates like PEA and PPi occurs primarily in tissues rather than blood. Study of additional cases of hypophosphatasia will be necessary to assess the clinical efficacy of this form of enzyme replacement therapy.

IBIDS is a syndrome characterized by ichthyosis, brittle hair, impaired intelligence, decreased fertility, and short stature, but unassociated with skeletal lesions. This condition is considered a form of trichothiodys-trophy because hair from several cases has been found to have a low sulfur content. We describe a 9-year and 10-month-old white boy whose clinical features resemble the IBIDS syndrome (ichthyosis, brittle hair, cataracts, and short stature), but who also has marked axial osteosclerosis and peripheral osteopenia. No abnormalities of mineral homeostasis were noted. Histopathologic assessment of nondecalcified bone specimens excluded osteopetrosis, but suggested slow skeletal remodeling. When subjected to polarized light microscopy, his hair exhibited the band-like pattern of birefringence described in trichothiodystrophy. Literature review disclosed 8 patients, 2 of whom had been diagnosed as trichothiodystrophy, with like clinical features including osteosclerosis. These skeletal abnormalities together with clinical features of the IBIDS/trichiodystrophy syndrome, we believe, reflect the prototype of a disorder that seems best described as central osteosclerosis with ectodermal dysplasia.

The 4p- syndrome results from a structural deficiency in chromosome 4. The clinical and radiologic findings in three patients with the 4p- syndrome are presented. Clinical, genetic, and radiologic findings in other reported cases are summarized. A widened interpubic distance with underdevelopment of the bony pelvis and deficiencies in ossification of the cervical spine are prominent radiologic features in our cases. Taken separately, these findings have limited differential diagnostic possibilities which are briefly discussed. The combination of these findings with characteristic clinical abnormalities is very suggestive of the 4p- syndrome and should prompt karyotyping for confirmation.

Pelvic pneumography was performed in 150 children, including those with precocious puberty, suspected pelvic masses, abdominal pain, virilization, ambiguous genitalia, gonadal dysgenesis, Stein-Leventhal syndrome, amenorrhea, and contralateral inguinal hernia detection. Pneumography proved safe, accurate, and easy to perform. However, advances in sonography have limited the use of pneumography primarily to the investigation of infant intersex problems and confirmation of idiopathic precocious puberty in the infant or very young girl.

The techniques of diagnostic radiology are generally safe, and most of the hazards can be avoided by observing simple precautions. The more invasive radiologic techniques should be avoided if the necessary information can be obtained with a procedure that carries less risk for the patient.

A three and one half-year-old female with Melnick-Needles osteodysplasia is presented. In addition to the characteristic bone roentgenograms and obstructive uropathy, the patient had inadequate formation of bone marrow elements and pulmonary artery hypertension.

Twenty-one infants and children with ovarian teratomas are reported. Calcification or ossification occurred in 68%, a higher figure than quoted in prior series. Teeth were recognized in 29% of these. A lucent fat-containing mass was appreciated in only two patients. In younger children the tumor was more frequently abdominal rather than pelvic. In two patients the tumor was malignant. A patient with an amputated freely mobile teratoma is reported in detail and the differential diagnosis of mobile abdominal calcifications discussed.

The clinical and radiographic features of 5 patients with congenital occipital dermal sinus are reviewed. This entity is frequently associated with a midline occipital bony defect visible on skull radiographs. Recognition and proper interpretation of this radiographic defect can be the first indication of the presence of a clinically unsuspected occipital dermal sinus. Alternatively, demonstration of the defect in patients being evaluated for midline occipital skin dimples, sinuses or masses can provide confirmatory evidence of congenital dermal sinus. Early recognition, particularly prior to development of infection, is of great importance.

Twenty-five children aged 8 months to 17 years were studied to determine the usefulness of computer-assisted ventilation-perfusion(V–P) scanning in analysis of regional pulmonary function in cystic fibrosis. Ventilation and perfusion scintiphotographs were compared with the patients' radiographs. Regional fractional exchanges of air and relative regional V–P ratios were obtained. Whole-lung air exchange was significantly correlated with standard pulmonary function tests and radiograph scores. Ventilation-perfusion ratio gradients were reversed in patients with predominantly upper zone disease, and regional V–P ratios were uneven in distribution. Quantitative assessment of regional ventilation and perfusion is a useful technique for assessing severity of pulmonary disease in children with cystic fibrosis.

Gastric outlet obstruction in infants and children may be due to a partial, prepyloric antral diaphragm. Twelve new patients are added to the 32 described previously. Onset of symptoms varied from shortly after birth to five years. Nonbilious vomiting was the most common presenting symptom. Radiographic evaluation requires specific technique for demonstration of the web and to differentiate this from pylorospasm and pyloric stenosis. Gastroscopy was employed in three patients. Repair usually consisted of incision of the web and construction of a patulous gastric outlet. All patients remained asymptomatic after operation. The etiology of the webs remains unknown, but they may result from an excessive local endodermal proliferation early in gastric development.

Seven patients with left-sided congenital diaphragmatic hernias at ages of 2--20 months are reported and five are described in detail. Radiographic findings were classic in one patient, but simulated inflammatory chest disease in two patients, gastric volvulus in three patients, and a pneumothorax in one patient. These misleading appearances if not recognized can lead to incorrect radiographic interpretation and in some cases inappropriate treatment.

Detailed cystoscopic grading of ureteral orifices has proved unnecessary in our experience for determining the prognosis of moderate, primary reflux in girls. Medially placed orifices strongly predict spontaneous resolution with preventive medication, while radiographic pyelonephritic scarring and/or grade III reflux strongly predict failure to resolve. While laterally placed ureteral orifices and low volume reflux suggest a need for operation, they are prognostically less useful, particularly when those with grade III reflux are excluded. Cystitis cystica and unrelated anomalies seem to be associated with the ultimate need for operation but are of uncertain significance.

Five children with intrathoracic metastases from Wilms tumor are presented. In two patients pneumothoraces developed, and in one patient pneumonitis developed secondary to a primary intrabronchial metastasis. Two other patients had paraspinal widening from metastases to paraaortic or posterior mediastinal lymph nodes. These are extremely rare complications of metastatic Wilms tumor.

The authors used the diagnosis of craniosynostosis to compare subjective evaluation of image quality with objective diagnostic utility. They studied in detail the responses of one observer, who read plain radiographs, computed tomographic (CT) scans, and three-dimensional reconstructions of CT scans (obtained with three different methods) for 82 patients with this diagnosis. The observer rated image quality and certainty in diagnosis made from each image. Subjective and objective performances were found to be strongly linked. High-quality images served as the basis for more accurate diagnoses than low-quality images. The increase in diagnostic performance results primarily from increased specificity, a fact that suggests that specificity and the concomitant diagnosis of normalcy are the focus of attention when image quality is evaluated.

Multilocular renal cysts are benign lesions with an unknown histogenesis. We report 3 children and summarize information currently known about these lesions. Capsular veins were seen in two patients during the early phases of intravenous urography. Intravenous urography with sonography and arteriography may suggest the diagnosis prior to surgery.

Twenty-two patients with Henoch-Schonlein syndrome are reviewed, and four unusual cases are reported in detail. Henoch-Schonlein syndrome represents a diagnostic problem when abdominal manifestations precede cutaneous lesions and suggest a surgical abdomen. Unusual radiographic features such as changes in the small bowel identical to Crohn disease or colonic involvement may further obscure the diagnosis. Intussusception and rarely bowel obstruction may complicate the clinical course. The spectrum of clinical and radiographic findings is described to increase the awareness of the radiologist to the possibility of the diagnosis of Henoch-Schonlein syndrome.

Analysis of airway radiographs of 20 children with proven acute epiglottitis revealed that five (25%) had, in addition to supraglottic edema, localized subglottic edema radiographically indistinguishable from that seen in croup. In all five patients the etiologic organism was Hemophilus influenzae type B.

Three adult sisters with osteopetrosis in infancy had spontaneous resolution of bone modeling defects and osteosclerosis. During adolescence, basal ganglia calcification developed in two. Renal tubular acidosis (type I) was diagnosed in each during early adulthood. The disorder was transmitted apparently as a recessive trait--the same mode of inheritance as for the "malignant" form of osteopetrosis which is usually fatal during childhood. Electron microscopy of bone suggested that osteoclasts failed to form "ruffled membranes" characteristic of active bone resorbing cells. Chronic systemic acidosis may have ameliorated the skeletal manifestations of this new syndrome.

The quantity of contrast agent that should be used for intravenous urography for neonates has been consistently higher than that for older children and adults. The authors wish to report an 18-day-old girl who developed pulmonary oedema shortly after receiving 4.7 ml/kg of Hypaque 50, a dose within the range recommended in some publications (Henderson and Torch, 1977; Standen et al., 1965). A 3 kg newborn girl who was the product of an uneventful pregnancy and delivery, was admitted to St. Louis Childdren's Hospital for exstrophy of the bladder, imperforate anus, and a small omphalocele. The omphalocele was repaired the first day of life and a colostomy performed. A chest roentgenogram demonstrated only slight dextroversion of the heart (Fig. 1a). She was clinically stable and well hydrated without unusual fluid loss from the colostomy. On day 17 the patient's serum chloride, sodium, potassium and blood urea nitrogen were normal.

Omental cyst is a rare lesion occurring primarily during childhood and young adulthood. It is closely related to mesenteric and retroperitoneal cysts, all conditions most likely being of congenital or benign neoplastic lymphatic origin. Omental cysts usually present as abdominal distention of long duration, less frequently as a palpable abdominal mass. Rarely they cause an acute abdominal surgical crisis clinically resembling appendicitis with peritonitis. Two cases are reported, one with acute and one with chronic symptoms. The clinical and radiologic features of omental cyst are reviewed in order to increase awareness of this entity.

• The clinical and radiographic features of five sick premature infants with idiopathic hypertrophic pyloric stenosis are presented. Clinical features were nonspecific, the common findings being recurrent nonbilious emesis, persistent abdominal distention, and the inability to place a nasojejunal feeding tube through the pylorus. Plain abdominal radiographs demonstrated persistent gastric dilatation in four of the infants. idiopathic hypertrophic pyloric stenosis should be considered in the differential diagnosis of premature infants with upper gastrointestinal tract symptoms. (AJDC1988;142:68-70)

Fourteen very low birthweight infants (mean +/- SD 1,070 +/- 180 g and 29.3 +/- 1.9 weeks gestation) fed their own mother's milk were clinically followed until 3-4 months of age with frequent measurements of serum calcium, phosphorus, magnesium, 25-hydroxyvitamin D (25-OHD), parathyroid hormone, alkaline phosphatase, and albumin, and urine calcium, phosphorus, and magnesium. These infants were matched for birthweight and gestation with 14 infants (1,075 +/- 152 g and 29.0 +/- 1.7 weeks) who had been similarly followed during concomitant studies of infants fed standard formula (Similac 20 cal/oz). Urine phosphorus was markedly lower in the breast milk-fed group from initiation of feedings, and serum phosphorus became significantly lower at and after 6 weeks of age. The fall in serum phosphorus was accompanied by a marked calciuria. Parathyroid hormone was suppressed in the breast milk-fed group, although serum calcium was not elevated and did not differ from formula-fed infants. A high incidence of moderate-severe hypomineralization on radiographs was seen in both breast milk- and formula-fed groups. Six of 14 breast-fed infants required phosphorus supplementation at 8-10 weeks of age because of significant hypophosphatemia, hypercalciuria, and hypomineralization. These infants differed from those not requiring phosphorus supplements by being smaller at birth but not of lower gestation, and having persistently low serum 25-OHD at and after 6 weeks of age.

After biochemical and radiographic studies, enzyme replacement therapy in three patients with the infantile form of hypophosphatasia was attempted by weekly intravenous infusions of bone alkaline phosphatase-rich (BAP) plasma from patients with Paget bone disease. Subsequently, circulating BAP activity was substantially increased in each patient, and in one was maintained in the normal range for nearly 2 months. Despite partial or complete correction of the deficiency of circulating BAP activity, we observed no radiographic evidence for arrest of progressive osteopenia or improvement in rachitic defects in any of the patients. Failure of infants with hypophosphatasia to show significant healing of rickets on correction of circulating BAP activity supports the hypothesis that this isoenzyme functions in situ during normal skeletal mineralization.

Congenital tracheal bronchus is an uncommon condition that may occur together with other, sometimes clinically significant anomalies of the tracheobronchial tree. Two such patients are reported. Both presented with respiratory distress, and one was initially diagnosed as having a pulmonary cyst. Radiographic demonstration of an upper-lobe cyst, particularly on the right, should suggest a tracheal bronchus.

A patient with a hyperalimentation catheter in the spinal canal is presented. This resulted in an extradural fluid mass and partial paraplegia that eventually cleared.

The axial projection of the spine is a useful view for evaluation of possible neural arch defects in children. The projection is easy to perform and allows visualization of the arches without confusing overlap by other structures.

Two brothers with chronic granulomatous disease of childhood are presented with circumferential gastric antral thickening demonstrated on sonography. Barium studies and sonography correlated well in one brother. The antral changes can resolve quickly without antibiotic therapy.

R E F E R E N C E S 1. Berdon, W. E., Baker, D. H., and James, L. S.: The ductus bump. A transient physiologic mass in chest roentgenograms of newborn infants, Am. J. Roentgenol. 95: 91, 1965. 2. Heikkinen, E. S., and Simila, S.: Aneurysm of the ductus arteriosus in infancy: Report of two surgically treated cases, J. Pediatr. Surg. 7: 392, 1972. 3. Heymann, M. A., and Rudolph, A. M.: Effects of congenital heart disease on fetal and neonatal circulation, Progr. Cardiovasc. Dis. 15: 115, 1972. 4. Moss, A. J., Emmanoulides, G., and Duffle, E. R.: Closure of the ductus arteriosus in the newborn infant, Pediatrics 32: 25, 1963.

We report 2 cases of ureteropelvic obstruction, 1 of which was congenital and the other was postoperative with asymptomatic deterioration of the involved kidney.

Cysto-Conray II 17.2% (81 mg l/ml), diluted Cysto-Conray 10% (47 mg l/ml), and Hypaque-Cysto 10% (47 mg l/ml) produced no histologic effects on rat bladders. Simulated ureters in water phantoms were well shown with lower concentrations of cystographic solutions including those of 5% (24 mg l/ml). In clinical studies there was little difference between Cysto-Conray II 17.2% and Hypaque-Cysto 10% for delineating refluxing ureters, bladder margins, and the urethra. Intraluminal filling defects in dog bladders were demonstrated best with cystographic concentrations of less than 10% (47 mg l/ml).

Three children with aneurysms of the thoracic aorta were studied. The clinical presentation, radiographic findings, and etiologic factors are discussed. These lesions are associated with a poor prognosis and only one patient survived. The radiographic appearance of an enlarging mediastinal mass close to the aorta should suggest an aneurysm of the thoracic aorta.

A young infant with vomiting associated with a gastric polyp is presented. The polyp proved to be focal foveolar hyperplasia. These non-neoplastic polyps of unknown etiology are usually found in adults.

A newborn with hemihypertrophy and bilateral palpable abdominal masses due to markedly enlarged kidneys is reported.The kidneys showed intrinsic distortion of the collecting systems by intravenous urography and a preoperative diagnosis of Wilms’ tumor was made. Open wedge biopsies of each kidney evinced normal tissue. Despite large kidneys the patient has remained well for 3 years.

Fifty-three children underwent permanent urinary diversion. Ileoureterostomy was performed in 43, ileocystostomy in 10. Fifty-eight per cent of the children have been followed for periods longer than 5 years. The best results were obtained when the upper urinary tracts were normal prior to surgery. When the upper tracts were dilated prior to surgery, the vast majority of the children improved on showed no change. There were 7 deaths in this group. The most common complications were calculus formation and stomal stenosis. The calculi usually appeared more than 5 years after surgery.

Twenty-seven children with total unilateral renal dysplasia were followed from 2 to 28 years with a mean duration of follow-up of 9.1 years.Only 1 instance of disease (a midureteral stricture) was found in the opposite kidney.In patients whose presentation is that of an abdominal mass which proves to be a totally dysplastic kidney, the prognosis is excellent.

A patient with atresia of the colon is reported in whom the distal colonic segment was perforated during a barium enema examination. The perforation occurred through an area devoid of muscle.

the pulmonary meniscus sign—a rounded mass in the lung capped by a crescent-shaped collection of air—has been typically applied to hydatid disease, but it can also be seen in other benign conditions. A patient with bronchogenic carcinoma presented with this sign. To our knowledge it has not been described in association with malignant lung lesions. Case Report A 55-year-old male was admitted to Barnes Hospital with a two-month history of malaise, fatigue, and the production of white-brown mucoid sputum. He lost 20 pounds during this interval but denied other symptoms. He had smoked at least one package of cigarettes daily during the past thirty-nine years. Physical examination disclosed occasional rales over the right posterior midlung field. White blood count was 13,500 with a normal differential. Hemoglobin and hematocrit were normal. Examination of the sputum revealed numerous Gram-positive cocci and an occasional Gram-negative rod. The purified protein derivative test was negative. The alkaline phosph...