Biomedical subjects
Publications and source records attributed to G Currarino.
We report on six children, from 2 to 19 months old, with a defect across the root of the acromion (basiacromion) diagnosed as a fracture in four and as an anatomical variant (separate ossification center for the lateral half of the basiacromion) in the other two.
This is the report of two newborn boys with imperforate anus associated with a long rectocutaneous fistula running deep into the scrotum and communicating, in its midportion, with the bulbar urethra. The findings are compared with those of a somewhat similar case in the literature, with some speculations as to the possible embryogenesis of the lesion.
PURPOSE: To describe our experience with congenital anomalies of the posterior arch of the atlas, with a review and classification of these defects and a note on their clinical significance. METHODS: We report six children and one adult, the mother of one of the children, with an anomalous posterior arch of the atlas. The diagnosis was made on lateral films of the neck. Three patients also had axial CT of the cervical spine. RESULTS: The anomalies encountered in the seven patients were absence of the posterior arch of the atlas (four patients), bilateral clefts (two patients), and unilateral cleft (one patient). In three patients the anomaly was discovered as an incidental asymptomatic finding; three other patients presented with transient neck pain or transient neurologic symptoms after head and neck trauma, and one patient (an adult woman) described neck symptoms of 1-year duration. CONCLUSIONS: On the basis of these seven cases we conclude that congenital defects of the posterior arch of the atlas may be discovered as incidental asymptomatic findings, but symptoms occurring after trauma to the head and neck or spontaneously also may be encountered.
The pars nuda is the most anterior segment of the male membranous urethra, extending from the urogenital diaphragm to the urethral bulb. It differs from adjacent urethral segments in being incompletely supported by corpus cavernosum and musculature. Usually, this part of the urethra is difficult to distinguish in a voiding urethrogram. Occasionally, however, it may stand out clearly in certain phases of urethral muscular activity, resulting in a roentgen picture which may mimic an abnormality.
This is the report of 9 infants in whom a lateral roentgenogram of the neck exposed in expiration showed an increase in the thickness of the retropharyngeal soft tissues with a small collection of air within these soft tissues simulating a retropharyngeal abscess. Both findings disappeared in inspiration consistent with normal variants.
Congenital double pylorus is a rare anomaly in which two pyloric openings connect the antrum of the stomach to the duodenal bulb. We report an unusual case of congenital double pylorus in which one of the channels led to an intraluminal cystic duplication of the duodenal bulb. An embryologic relation between congenital double pylorus and antroduodenal duplications is suggested.
Cystourethrography in 25 children 0 to 13 years old demonstrated reflux of contrast material from the urethra or bladder into 1 or both ejaculatory ducts. The main findings observed in these patients are discussed and the literature is reviewed.
Seven cases with the pathologic/autopsy diagnosis of caudal regression or sirenomelia in which antenatal sonography had been performed were reviewed. The three patients with caudal regression had similar findings on antenatal sonogram, including normal or increased amniotic fluid, mild dilation or normal urinary systems, nonfused extremities, and sacral agenesis. In the four patients with sirenomelia, common sonographic findings included marked oligohydramnios, suspected renal agenesis, and sacral agenesis. A history of maternal diabetes was elicited in all patients with caudal regression and in none of the patients with sirenomelia. Findings confirm recent articles in pediatric pathology suggesting that caudal regression is a separate entity, distinct from sirenomelia.
This is the third report of an anomalous umbilical vein draining ectopically in a left pelvic vein, probably the left internal iliac, and through the inferior vena cava into the right atrium. The anomaly was encountered in a newborn infant with nonimmune hydrops fetalis, hypertrophic cardiomyopathy, multiorgan failure and possibly Noonan Syndrome.
This is the report of 4 children, 2 with a vascular ring and 2 with an aberrant right subclavian artery, who presented with an esophageal foreign body in the proximal esophagus above the level of the vascular anomaly. A deep erosion of the esophagus at the site of the foreign body was demonstrated in 2 patients.
Three children with torsion and amputation of the right ovary are presented. The detached ovary resulted in a cystic mass containing necrotic material and a solid, partially calcified mural node. The cysts were attached to the omentum, to the mesentery of the transverse colon, or to the lower edge of the liver by a long twisted pedicle containing thin-walled vascular spaces. The radiographic and sonographic findings were quite similar and clearly reflected the operative and pathologic changes.
This report describes 3 children with a cleft in the pedicles of the second cervical vertebra of uncertain cause. One of these patients had pyknodysostosis. Previous instances of such defects in the literature are reviewed with some considerations on the possible nature of the lesion.
We present a case of a rare renal anomaly in which the 2 kidneys (separate or fused across the midline) are drained by a common renal pelvis and ureter. Previously reported cases have been reviewed and are classified in 3 groups according to their anatomical features. The lesion is associated with some frequency with imperforate anus, sacral and other vertebral defects, neurogenic bladder, vesicoureteral reflux, upper tract dilatation, and urinary tract infections.
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Infected bile lakes complicated orthotopic liver transplantation in two pediatric patients. Retransplantation was avoided by means of percutaneous transhepatic internal drainage of the bile lakes. Internal drainage maintained the enterohepatic cycle of cyclosporine and allowed the bile ducts to heal.
Congenital contractural arachnodactyly (CCA) is an uncommon condition characterized by arachnodactyly, dolichostenomelia, kyphoscoliosis, "crumpled" auricles, and flexion contractures at knees, elbows, fingers, and sometimes other joints. The long-term prognosis for affected individuals is usually relatively good. We report on two newborn infants with severe CCA, both of whom died in the first year of life. One child also had other anomalies not generally associated with this disorder. These observations are consistent with the possibility that CCA is an etiologically and clinically heterogeneous disorder.
The length of the thoracic spine was measured in chest roentgenograms of 331 children from birth to 16 years and standards for thoracic spine length in this age group were obtained.
This is the report of a boy who presented at birth with severe generalized platyspondyly, a vertebral "coronal cleft", and an abnormal configuration of the pelvis with short and broad iliac and ischial bones and horizontal acetabular roofs. The rest of the skeleton was normal. In the ensuing years the vertebral bodies and pelvis assumed a near normal configuration, but the patient developed changes of metaphyseal chondrodysplasia in the long bones of the lower limbs with progressive shortness of stature.