Biomedical subjects
Publications and source records attributed to V Gilsanz.
Two children with pulmonary sequestration and gastroesophageal connections are described. Barium studies demonstrated the site of the connection and angiography showed the vascular connections enabling successful surgery to the performed.
Computed tomography in a 5-year-old girl revealed a central calcified retroperitoneal mass which was shown on subsequent arteriography to be an aortic aneurysm.
The clinical records of ten premature infants with nephrolithiasis were reviewed. The probable mechanism of stone formation was hypercalciuria due to furosemide therapy (nine patients) and congenital hyperparathyroidism (one patient). The relatively high rates of urinary excretion of calcium from immature kidneys predisposed these neonates to the development of calcium stones. Renal calculi in neonates are complications of distinct, treatable conditions, and premature babies receiving furosemide treatment should be screened by ultrasonography for renal calcifications.
The finding of extensive costochondral calcification in five adolescents, aged 14-16 years, with hyperthyroidism prompted a retrospective study of all young persons with this condition seen in the last 10 years. Thirty-six patients with hyperthyroidism (ages 13-18 years) were evaluated. Of 32 thyrotoxic teenage girls, 21 (66%) showed some degree of calcification, compared with 73 of 600 (12%) in the control group. Only four boys were evaluated, but one had grade 2 (mild) calcification that was not seen in any boy in the control group. Costochondral calcification increases with age and continues throughout life, reflecting the aging process. The much higher incidence of early costochondral calcification in thyrotoxic adolescents than in the general population is a radiographic indication of advanced bone maturation not previously recognized.
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This multicenter study was designed to determine if CT can assess operability in stage III neuroblastoma. Nineteen children (11 boys, eight girls), aged 2-51 months, considered to have, by conventional clinical and radiographic examinations, localized neuroblastoma that crossed the midline were examined by CT. After intravenous and oral contrast media enhancements, CT was able to show the relation of the tumor to the adjacent vital vessels: aorta, celiac axis, and superior mesenteric artery. In all 15 patients, tumors that encased the aorta or its major branches were unresectable. However, three of four tumors crossing the midline but not coming into contact with the aorta or adherent only to one side of the vessel could be resected. Because surgery currently plays the major role in the treatment of neuroblastoma, the relation of the tumor to the aorta and great vessels is a more reliable and important factor in predicting the outcome of these children than the extension and location of the tumor with reference to the midline. CT after intravenous contrast enhancement can establish this relation and assess resectability.
It has been well documented that children with severe neuromuscular disorders have tall vertebrae, presumably a consequence of altered mechanical forces. This finding was present in four neonates who were born with severe "floppy" hypotonia due to Werdnig-Hoffmann disease (two cases), nonspecific neonatal myopathy, and congenital muscular dystrophy. Fetal vertebral development is normally modified by intrauterine muscle tension and fetal activity.
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The authors conducted a blind retrospective survey of the clinical records and radiographic examinations of 100 patients with juvenile rheumatoid arthritis to ascertain the cause of the joint calcification observed in several cases. Review of the clinical histories showed that 39 patients (92 joints) had been treated with intrasynovial corticosteroids, while an independent study of the radiographs of the same 100 children revealed 20 cases of calcification involving 32 joints. All of the calcified joints had been injected with intrasynovial corticosteroids on one or more occasions. The frequency of calcification, its morphology, and the possible pathogenesis are discussed.
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Percutaneous drainage of intraabdominal fluid collections was performed in 13 children. After initial diagnosis with either sonography or computed tomography, a smallbore aspiration needle (22 gauge) was guided into the collection, usually by sonography. In five patients, complete evacuation was possible using the aspirating needle alone. In seven others, the fluid was successfully drained via a catheter introduced percutaneously. In one patient, surgery was required for complete evacuation of a hematoma containing large blood clots. There were no complications.
Three hundred seventeen patients with toxic oil syndrome caused by ingestion of denatured rapeseed oil were followed up after one year. Total mortality for the 317 patients was 4.4%. After 12 months, 3.2% of the surviving patients have a severe motor neuropathy, 52.9% are asymptomatic, and the remaining 39.4% have mild residual myalgia. The cause of death in seven patients during the acute phase of toxic oil syndrome was respiratory failure due to interstitial pneumonitis with cerebral edema. The death of five patients in the second phase was due to motor neuropathy that involved respiratory muscles and was complicated by aspiration pneumonia.
Two children with a congenital limb deficiency, involving the distal end of the femur and the femorotibial joint, are described. Both had associated ipsilateral limb anomalies; one had absence of the shaft of the femur and agenesis of the fibula and the other had a malformed tibia and dislocation of the knee. The similarities between distal focal femoral deficiency and the more common proximal femoral defects are stressed.
Four children with distal tibiofibular diastasis associated to talipes equinovarus deformity are described. Tibial hypoplasia resulting in tibiofibular diastasis is believed to be the underlying cause of the deformity. This complex deformity is usually associated with other congenital malformations including congenital cardiac defects and other limb anomalies. Surgical therapy has centered on establishment of an ankle mortise; however, several authors believe that the best treatment is early ankle disarticulation rather than reconstructive surgery. Radiographs should be made of all apparent clubfoot deformities in order to rule out the more serious distal tibiofibular diastasis deformity.
Progressive scoliosis was seen in eight children after thoracotomy for esophageal atresia. Postoperatively, all had had severe mediastinitis and empyema secondary to dehiscence of the esophageal anastomosis and required reoperation. Healing was accompanied by marked scarring and rib fusion; with growth, a scoliosis developed with the concavity toward the thoracotomy site. Most spinal curvatures appeared years after the thoracotomy and progressed rapidly at the time of adolescent growth spurt. Excision of fused ribs, spinal fusion, and instrumentation led to improvement in four patients.
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Forty-seven females with duplication of the uterus and cervix were retrospectively studied and their associated urinary tract malformations were analyzed. In 31 of the patients duplication of the müllerian ducts was diagnosed during the work-up for major genitourinary malformations that corresponded to three main complexes of anomalies: cloacal anomalies (16 patients), exstrophic deformities (4 patients), and renal malformation on the same side as an occluded müllerian duct (11 patients). Another five patients with normal kidneys had incomplete müllerian duct fusion discovered incidentally at autopsy, surgery, or during the work-up for urinary tract infections. None of the 11 women examined by excretory urography following the discovery of duplication of müllerian ducts by hysterosalpingography had urinary tract abnormalities. Therefore, although failure of fusion of the müllerian ducts frequently coexists with other genitourinary anomalies, the associated malformations manifest themselves clinically. Women who are found to have duplication of the reproductive tract during the work-up for infertility and who are otherwise asymptomatic and have a normal physical examination do not require urographic investigation.