Wilbur L. Smith

Tumors of the chest wall in infants and children.

Chest wall tumors of children may arise from subcutaneous tissues, the bony thorax, or extrapleural region. Review of experience at our institution and the literature indicates that primary bony tumors are most often malignant; Ewings sarcoma is the most frequent in this category. Extrapleural tumors are rare but also usually malignant. Clinical and roentgen features of these lesions are discussed.

The VATER Association

The VATER association is a group of congenital anomalies with a nonrandom tendency for concurrence. Defects include vertebral, vascular, anorectal malformation, tracheoesophageal fistula with esophageal atresia, radial-limb, and renal abnormalities. The critical period of organogenesis is at or before the sixth or seventh week of gestation. The VATER assoication is important in the evaluation of newborns with major congenital anomalies.

Early onset group B streptococcal disease: clinical, roentgenographic, and pathologic features.

Thirty-one neonates with early onset of serious group B streptococcal infections were observed in a four-year period. The mortality was 52%. Premature infants with clinical signs of respiratory distress syndrome were at highest risk of death; clinical signs of RDS were typical until apnea, shock, respiratory failure, and worsening of the radiographic pattern unexpectedly intervened. Pathologic material from infants with radiographic evidence either of RDS or of pneumonia showed both typical hyaline membrane disease and pneumonia in most instances. Factors which may be helpful in recognizing premature infants at risk for GBS disease in the much larger group of premature infants with uncomplicated RDS include: history of artificial, premature, or prolonged rupture of membranes; localized pulmonary infiltrates on chest roentgenogram; low absolute neutrophil count; and an unusually rapid progression of RDS.

In utero diagnosis of achondrogenesis, type I

A series of three infants with achondrogenesis, all born to the same mother, are reported. The third case was diagnosed in utero.

Respiratory distress following elective repeat cesarean section

The clinical course and chest radiographs of 47 infants with respiratory distress after elective cesarean section were reviewed. The mean difference between the gestational age determined prenatally and that postnatally was 2.6 +/- 1.6 weeks. However, 14 of the infants were delivered at term. All 47 infants required more than 40% oxygen, and 18 infants required a respirator. Fifteen infants developed a pneumothorax; one, a pneumopericardium; one, bronchopulmonary dysplasia; and one infant died. Chest radiographs and the clinical course were consistent with hyaline membrane disease in 17 patients; respiratory distress syndrome type II in 24; and in three the radiographic findings were normal. These data suggest that some of the respiratory morbidity subsequent to elective repeat cesarean section is not secondary to iatrogenic delivery of a premature infant, and that much of it is not due to hyaline membrane disease. These data emphasize that respiratory distress in an infant delivered by elective cesarean section does not necessarily suggest poor prenatal care in regard to the timing of delivery.

The use of metrizamide (amipaque) to visualise the gastrointestinal tract in children: A preliminary report

Metrizamide (amipaque) has not been used previously as a diagnostic contrast agent in the gastrointestinal tract. Metrizamide is a water-soluble isotonic contrast material having many advantages over barium and existing hypertonic water-soluble agents. There are many clinical situations in children in which metrizamide should be the contrast agent of choice for investigating the gastrointestinal tract. Four neonates are presented in whom barium or gastrografin were absolutely contraindicated. In each case metrizamide gave excellent visualization of the gastrointestinal tract. It could be followed through be bowel giving excellent visualisation even up to 120 h after ingestion. No harmful effects were noted in the four cases studied.

A Practical Method for Monitoring Diagnostic Radiation Dosage in the Newborn Nursery

Diagnostic radiation exposure in 133 consecutive newborns was studied using a TLD monitoring system. Eighty-eight per cent of dosimeters received less than 1 mSv (100 mrem) total exposure. The mean exposure per chest radiograph was 0.044 +/- 0.023 mSv (4.4 +/- 2.3 mrem). Abdominal surface exposure was 0.053 +/- 0.03 mSv (5.3 +/- 3.0 mrem) for boys and 0.044 +/- 0.021 mSv (4.4 +/- 2.1 mrem) for girls. The gonadal exposure, which was calculated from the abdominal exposure data, was 0.053 +/- 0.030 mSv (5.3 +/- 3.0 mrem) for boys and 0.026 +/- 0.012 mSv (2.6 +/- 1.2 mrem) for girls. The correction factor for beam attenuation over gonads in girls was 0.58. In general, the radiation received by these infants did not exceed the currently published protection limits.

Bone scanning in management of metastatic osteogenic sarcoma.

Bone scan findings are presented from five consecutive cases of metastatic osteogenic sarcoma. Every patient had pulmonary metastases which concentrated the bone imaging radiopharmaceutical to some degree. In one patient, the diagnosis of pulmonary metastasis was made prior to our seeing any radiographic abnormalities. The mechanism by which skeletal imaging agents localize in pulmonary lesions is not clear. Unsuspected skeletal metastases were also discovered on scans in two patients, both of whom had normal radiographs. Diagnosis of hypertrophic pulmonary osteoarthropathy was made in one patient with normal radiographs. These findings indicate that bone scans as well as radiographs should be performed routinely in preoperative staging and followup of patients with osteogenic sarcoma.

Dominant inheritance of velopharyngeal incompetence

A family is described in which a girl, two boys and their father showed speech hypernasality. A half‐sib, a grandfather, a great‐aunt, and a cousin of these sibs also showed a similar speech defect. Analysis of recorded speech of the father and three sibs revealed articulation deficiencies in addition to hypernasality. Intra‐oral examination and neurologic evaluation of the cranial nerves failed to reveal any abnormality in palate morphology or cranial nerve function. Psychometric and audiometric studies of affecteds did not contribute to an explanation of the defect. Speech cinefluoroscopy and cephalometric radiographs confirmed that the speech defect involved velopharyngeal incompetence resulting from an anatomic disproportion of the velopharyngeal structures. The family pedigree supports the concept that this type of nasal speech, resulting from disproportion of velopharyngeal structures, is transmitted as an autosomal dominant trait.

Paralysis of the small bowel resembling mechanical intestinal obstruction

In some instances, particularly in the neonate, paralytic ileus affects predominantly the small bowel, thus resembling intestinal obstruction. Localized paralysis of the small bowel occurs secondary to regional events in a segment of gut and resembles mechanical obstruction even more closely. In localized paralysis the bowel lumen is usually dilated without abrupt distal transition to collapsed intestines. If there is radiographic and clinical recognition of localized, transitory bowel paralysis, surgery is usually unnecessary.