Multicenter trial of single-dose modified bovine surfactant extract (Survanta) for prevention of respiratory distress syndrome. Ross Collaborative Surfactant Prevention Study Group.

A multicenter, prospective randomized controlled trial was performed comparing the efficacy of a single intratracheal dose of modified bovine surfactant extract (Survanta, 100 mg/kg, Abbott Laboratory, North Chicago, IL) with air placebo in preventing respiratory distress syndrome. Infants were enrolled if they were estimated to be between 24 and 30 weeks' gestation, weighed between 750 and 1250 g, and were intubated and stabilized within 15 minutes after birth. A total of 160 infants were treated (79 with surfactant, 81 with air placebo) between 4 and 37 minutes after birth (median time 12 minutes). Of these, 5 infants were excluded from the final analysis. The 72-hour average values for the arterial-alveolar oxygen ratio, fraction of inspired oxygen, and mean airway pressure were calculated from the area under the curve of scheduled values measured throughout 72 hours. Clinical status was classified using five ordered categories (no supplemental oxygen or assisted ventilation, supplemental oxygen only, continuous positive airway pressure or assisted ventilation with intermittent mandatory ventilation less than or equal to 6 breaths/min, assisted ventilation with intermittent mandatory ventilation greater than 6 breaths/min, death). Chest radiographs at 24 hours were graded for severity of respiratory distress syndrome. Infants receiving Survanta had less severe radiographic changes at 24 hours of age and decreased average fraction of inspired oxygen (31% vs 42%, P = .002) compared with control infants. No differences were noted in the average arterial-alveolar oxygen ratio, mean airway pressure, or clinical status on days 7 and 28. A beneficial effect was noted in the incidence of pneumothorax (P = .057) and an increase was noted in the incidence of necrotizing enterocolitis (P = .052). No differences in incidence of patent ductus arteriosus, intraventricular hemorrhage, sepsis, or bronchopulmonary dysplasia were seen. According to results of a secondary analysis, there was improvement in the fraction of inspired oxygen and a greater number of survivors without bronchopulmonary dysplasia in the subgroup of infants weighing less than 1000 g who were treated with surfactant. It was concluded that a single dose of Survanta given shortly after birth resulted in decreased severity of chest radiographic findings 24 hours after treatment and improved oxygenation during 72 hours after treatment, but did not improve other acute measures of disease severity or clinical status later in the neonatal period. The group at highest risk for respiratory distress syndrome (infants with birth weights between 750 and 999 g) may benefit the most from preventive therapy.

Gonadal mosaicism in pseudoachondroplasia.

We report on a family in which a brother and sister have pseudoachondroplasia and normal parents. The brother married a normal woman, and they have 2 daughters; one of them has typical changes of pseudoachondroplasia, the other is normal. The most likely explanation in this family is gonadal (germinal cell) mosaicism in one of the grandparents. Other reports of possible autosomal recessive pseudoachondroplasia are reviewed. It is likely that gonadal mosaicism is responsible for a small percentage of cases with what appears to be a new mutation for pseudoachondroplasia.

Ectopic ureter and ureterocele: their varied sonographic manifestations.

The sonographic examinations of four patients with simple ectopic ureters and 11 with ectopic ureteroceles were reviewed to determine distinguishing characteristics. Ectopic ureters, in cases of extreme dilatation and tortuosity, sometimes mimic multiseptated, cystic abdominal masses. However, the proximal portions of some severely dilated ureters are surprisingly small. Ectopic ureters sometimes indent the lower vesical wall, simulating a ureterocele. Ectopic ureteroceles are dynamic structures, changing in shape and size according to intravesical pressure. The lower pole of a duplex kidney may be difficult to detect because of displacement by the dilated upper renal pelvis and ureter. The renal parenchyma associated with an ectopic ureter may be equally difficult or impossible to find because of diminutive dysplasia or, less commonly, acquired atrophy. Dysplasia is characterized sonographically by highly echogenic parenchyma, lack of corticomedullary differentiation, and occasionally massive enlargement by cysts. Ectopic ureters and ureteroceles can be identified by fetal sonography.

Osteogenesis imperfecta with unusual skeletal lesions: report of three families.

Thirteen individuals with osteogenesis imperfecta (OI) from three families were evaluated. All examined persons with OI had multilocular radiolucent, radiopaque, or radiolucent-radiopaque lesions of the maxilla and mandible. In most patients, the lesions involved the tooth bearing areas, but in two, the rami also were involved. Teeth were normal. Radiologic findings in the extragnathic skeleton included marked coarseness of trabeculae and diffuse osteopenia. It is proposed that these patients represent yet another dominantly inherited OI syndrome.

Polycarpaly and other abnormalities of the wrist in chondroectodermal dysplasia: the Ellis-van Creveld syndrome.

Radiographs of 45 wrists of 23 patients who had chondroectodermal dysplasia (CED) showed variable wrist malformations, which we grouped into eight categories. In each patient, the wrists tended to be similar but seldom identical; in six patients they were sufficiently dissimilar that we classified the malformations in their two wrists into separate categories. A ninth carpal bone was present in the wrists of all patients who were five years old or older (42 of 45 wrists). It was located in the distal row, medial to the hamate bone and proximal to the fifth and sixth metacarpals. A tenth carpal bone was found in six wrists. Unlike the ninth carpal bone, it varied in location. All of the carpal bones were deformed, and two or three separate ossification centers of the hamate were found in some individuals. Fusions between the capitate, the hamate, and the ninth carpal bone were present by the last available examination in 71% of the 45 hands: these included fusions between the capitate and hamate in 7%, between the hamate and the ninth carpal bone in 47%, and between all three bones in 18%.

The volume of the sella turcica in children: new standards.

Measurement of the sella turcica is valuable in the assessment of a variety of pituitary and other conditions. The volume, a more reliable measurement than sellar area, has been difficult to interpret for lack of adequate standards. This study presents mean and 10th, 50th, and 90th percentiles for sellar volume in normal children aged 6-16 years on the basis of measurements in 960 sets of skull radiographs made for orthodontic purposes. Sellar volumes are plotted against chronologic age, and against skeletal age for the 62% of measurements for which a simultaneous bone age determination had been made. As expected, the sellar volume increases with age: for example, mean volume for a boy 5 years 9 months to 6 years 8 months is 228 mm3, for a boy 15 years 9 months to 16 years 8 months is 640 mm3. For most age groups, sellar volume in males is greater than in females. Tenth and ninetieth percentiles for sellar volume are widely separated, giving a wide range of normal. Nevertheless they should be useful in assessing sellar volume in clinical situations.

Respiratory complications of achondroplasia.

Nine patients with achondroplasia who were seen over a three-year period developed significant respiratory complications. Eight had sleep-disordered breathing, including obstructive sleep apnea in five, for which two required tracheostomy. Of the seven patients with significant hypoxemia, five had clinical evidence of cor pulmonale and recurrent pulmonary infiltrates. Two patients died, one with autopsy findings of compression of the medulla at the level of the foramen magnum and one with respiratory and cardiac failure. Appropriate therapy for our patients depended on recognition of the mechanisms that led to the respiratory complications, including (1) chest deformity, (2) upper airway obstruction and sleep-disordered breathing, (3) neurologic complications, and (4) coincidental chronic pulmonary conditions such as asthma.

Sclerosteosis involving the temporal bone: clinical and radiologic aspects.

Sclerosteosis is one of the rare, potentially lethal, autosomal recessive, progressive, craniotubular sclerosing bone dysplasias. Syndactyly of the second and third or other fingers is evident at birth. Hyperostosis and sclerosis are most prominent in the skull and tubular bones, and are frequently associated with excessive height and weight. The typical facial deformity is apparent by the age of 5 years. The changes involving the temporal bone include a marked increase in overall dimensions, extreme sclerosis, and narrowing and constriction of the external ear canal, middle ear cleft, internal acoustic meatus, and falloppian canal. Impairment of hearing, as a rule bilateral, is a frequent presenting symptom which may manifest in early childhood. Initially it is an expression of interference with sound conduction; later it may become associated with a loss of sound perception. Impairment of facial nerve function is another salient feature which occasionally is present at birth. As a rule, it manifests initially as a unilateral, recurrent paresis, eventually progressing to a bilateral permanent partial loss of facial nerve function. Since impairments of hearing and facial nerve function are two of the salient features, present at birth or in early childhood, the responsibility for recognizing the disease often falls upon the otolaryngologist. The clinical and radiologic features permit not only early recognition of the disorder but also differentiation from similar bony dysplasias. Hyperosteosis and sclerosis of the skull lead to thickening and distortion of the calvaria, cranial base, and foramen magnum resulting in reduction of the intracranial volume, interference with the cerebral blood flow, resorption of cerebrospinal fluid, and gradual increase of intracranial pressure. Severe headaches resulting from this mechanism often develop in early adulthood, and several patients have died suddenly from impaction of the medulla oblongata in the foramen magnum. Decompression of the transverse sigmoid sinus and jugular bulb may be lifesaving, combined with a posterior, and if necessary, an anterior, craniectomy for decompression. Early decompression of the internal acoustic meatus and falloppian canal may help in the preservation of cochlear and facial nerve function.

Osteogenesis imperfecta lethal in infancy: case report and scanning electron microscopic studies of the deciduous teeth.

Radiologic evaluation of the skeleton and scanning electron microscopic studies of the teeth were performed on an infant boy with a lethal osteogenesis imperfecta (OI) syndrome who died at 10 mo of pneumonia. The skeletal findings included ribs that were focally expanded by fracture calluses, flat vertebral bodies, and wide limb bones. On fractured tooth surfaces, the enamel and dentin were normal as was the dentin calcification front. Although microscopic abnormalities have been noted in teeth from previously reported infants with lethal OI, a few studies also report infants with normal teeth. These differences in dental findings may indicate heterogeneity in OI lethal in infancy. Results of our study indicate that, until the primary biochemical defects in the OI syndromes are elucidated, examination of teeth from other infants with lethal OI and detailed evaluation of other clinical and skeletal features will aid in delineating heterogeneity and variation in expression in lethal OI.

Child abuse.

Child abuse is common in most, if not all, Western nations; it probably occurs worldwide. It may be a major factor in the increase in violence throughout much of the world. Radiologists who treat children should think of the possibility of abuse whenever they diagnose a fracture, intracranial bleed, or visceral injury, especially when the history is not compatible with their findings. Metaphyseal "corner" fractures in infants usually are caused by abuse. Less than 20% of abused children, however, present injuries that can be recognized by radiologic techniques. Consequently normal roentgenograms, nuclear medicine scans, ultrasound studies, and computed tomograms do not exclude child abuse.

Radiodensity of the proposed new penny.

In 1973 the United States Mint proposed replacing the bronze penny with a less expensive penny made of aluminum. Pediatricians and pediatric radiologists, by testifying that aluminum coins are difficult to see on radiographs, helped defeat that legislation. Now the Mint again proposes a less expensive penny, one made principally of zinc. This proposed penny is radiopaque and will be easily identified on radiographs.

The Drash syndrome: male pseudohermaphroditism, nephritis, and Wilms tumor.

We report the radiographic and clinical findings of five infants and children who had the combination of male pseudohermaphroditism, progressive nephritis, and Wilms tumor. The components of this syndrome are so striking that the radiologist can often suggest the diagnosis months to years before all three components are apparent, if proteinuria, hypertension, or an abdominal mass develop in any child with male pseudohermaphroditism. Since the progressive renal failure probably can be managed by dialysis or renal transplantation in some cases, prompt recognition and treatment of the Wilms tumor may permit the child to live a long and relatively normal life.

Oxandrolone therapy in patients with Turner syndrome.

Long-term, low-dosage androgen treatment of patients with Turner syndrome results in more rapid growth and significantly greater adult height than in control patients who receive only estrogen for pubertal development. Seventeen patients treated with oxandrolone for one year and ten treated for two years had significantly greater growth velocities during than before treatment. Mean adult height of 25 patients treated with oxandrolone, fluoxymesterone, or both was significantly taller than the height of adult patients with Turner syndrome treated with estrogen only. Excessive skeletal maturation was not generally observed.

N-type anorectal malformations.

Three cases of N-type anorectal malformations have been encountered. Two girls with anorectal-vestibular fistulas (one with a normal anus and the other with mild stenosis) were typical of 15 other such cases in the literature. A boy with an anorectal-urethral fistula and covered anus had a lesser variant than most of the other 8 males in the literature. The frequently accompanying esophageal, renal, and skeletal anomalies were not present, nor did he have anterior urethral hypoplasia. The constellation of other major associated anomalies in the boys, but not in the girls, follows the pattern of other types of anorectal malformations and suggests an earlier and/or more severe teratogenic insult in the boys. Anterior perineal resection of the fistula is appropriate for girls with N-type fistulas and for those boys with normal anterior urethras. An additional urethroplasty is necessary for boys with anterior urethral hypoplasia. Our experience and that from the literature suggest that N-type anorectal malformations constitute a rare but real entity, akin to N-type tracheoesophageal fistulas. A plea is made that they be included in classifications of anorectal anomalies.

Abnormal intestinal motility in gastroschisis.

The authors describe abnormal intestinal motility with a very slow transit time in eight children with gastroschisis. Other abnormal findings include malrotation, dilatation, and fixation of the loops of the bowel.

Left aortic arch with right descending aorta.

A left aortic arch with a right descending aorta is a rare congenital anomaly. The radiographic findings in 13 previously described cases and three additional cases are discussed. Characteristic findings on the plain radiograph and esophagram can usually obviate the need for aortography. The anomaly was associated with a vascular ring in at least four and probably five of the 16 patients. Additional and more complex studies may be needed in patients with suspected symptomatic vascular rings.

Peritoneographic demonstration of femoral hernia.

Four cases of congenital hernia demonstrated by positive-contrast peritoneography are reported. Its differentiation from indirect inguinal hernia is described.

Use and complications of high-dose disodium etidronate therapy in fibrodysplasia ossificans progressiva.

A boy with fibrodysplasia ossificans progressiva received nearly twice the usual therapeutic dose of disodium etidronate for 13 months in an attempt to prevent reossification following a second operation to correct severe torticollis. The operation was successful, but during therapy he developed weakness and distinctive bone lesions characterized by general osteopenia, widened physes, and unique radiolucent bands in the metaphyses. The osseous abnormalities were distinct from those of rickets and healed after withdrawal of the drug.