Azygous lobe presenting as a posterior mediastinal mass in a 2-year-old boy.

Posterior mediastinal masses encompass a varied group of surgical lesions that include malignant diseases as well as the anatomic malformations of four organ systems. The authors report on a 2-year-old child with a posterior mediastinal mass who was found at thoracotomy to have a chronically infected "hepatized" azygous lobe of the right lung. The authors review current literature on this topic as well as the embryogenesis of azygous lobes.

Leptomeningeal metastasis from a paraspinal rhabdomyosarcoma with a der(13)t(1;13)(q23;q32) in a 14-month-old boy.

Cytogenetic analysis performed on a 14-month-old boy with a primary retroperitoneal/paraspinal alveolar rhabdomyosarcoma showed the presence of a der(13)t(1;13)(q23;q32) resulting in partial trisomy of the 1q23-->qter region and loss of the 13q32-->qter region. The present case is discussed with reference to a similar case reported in the literature.

Gastroduodenal disorders associated with emesis in infants.

Emesis is a common occurrence in newborns and infants. The causes are ubiquitous and unfortunately nonspecific. However, clues exist with respect to the timing of the onset of emesis as well as to its character. Emesis can be bilious or nonbilious, projectile or nonprojectile, and in some patients it is effortless. Newborns and infants with emesis of gastric origin may display a paucity of visible external signs. Ultrasound, contrast radiographic studies, and endoscopy coupled with physical examination are invaluable in the assessment of the emesis. Important in the management is prompt correction of fluid and electrolyte losses. Surgical correction, if warranted, can then be undertaken with significant lessening of morbidity.

Familial Hirschsprung's disease: study of a Texas cohort.

OBJECTIVE: To better define the types of transmission of Hirschsprung's disease (HD) in order to allow for earlier diagnosis and treatment and reduction of attendant morbidity. METHODS: A proband with HD was identified. A detailed family history was obtained and a 54-member, five-generation family pedigree constructed and analyzed. RESULTS: Six members of the kindred presented with classic biopsy-proven HD. Two of the six members were a pair of dizygotic twins. Three additional members had symptoms reminiscent of HD or HD variant (neuronal intestinal dysplasia) and were managed nonoperatively. There was one death related to HD. Hirschsprung's disease was manifested in offspring of both affected parent(s) and unaffected parents. Symptom manifestation was noted to skip generations. CONCLUSIONS: Analysis of this kindred lends strong evidence to the possibility of autosomal dominant inheritance. This inheritance has a variable penetrance (expression) that may be a factor of mutation or of a spectrum of the disease, ie a type of neural crestopathy. Kindreds of this sort need to be identified to allow for a complete data collection and understanding of the vagaries of HD transmission. This detailed documentation will allow for earlier diagnosis and effective treatment and possible gene localization of the specific defect.

Observations of a pediatric surgeon in the Persian Gulf War.

In Third-World countries, infectious disease is the principal cause of childhood death and disability. During the Persian Gulf War trauma became the leading cause of death in children, prompting this review of experience with the delivery of pediatric trauma care to noncombatant children at a military hospital. Eight hundred seventy-seven patients were admitted to the 410th Evacuation Hospital from January to April 1991. Fifty of the patients (6%) were children, and 40 of the 50 were admitted for trauma. The mean age of the children was 9 years. Sixty-five percent of pediatric patients sustained penetrating injuries; mechanisms of injury included shrapnel wounds, gunshot wounds, burns, motor vehicle accidents, crush injuries, and falls. The overall mortality rate for children admitted to the hospital was 12%, but no injured child died as a result of trauma. Complications of dehydration or malnutrition in infants accounted for all the deaths.

A technique for documenting central placement of long term catheters in neonates.

The aforementioned technique of confirmation of the central catheter has been used in the placement of 50 consecutive catheters placed through the greater saphenous venous approach. The drop test has proved to be positive in 49 of 50 insertions (98 per cent). The single false-positive result was obtained in a catheter with a hairpin bend at the tip. This technique has markedly decreased the amount of time required to place the catheter by avoiding multiple roentgenograms and the associated waiting time and reduces the increased cost of multiple roentgenograms.

Penetrating trauma to the buttock.

Penetrating trauma to the buttock is an injury with potential impact on multiple body systems. The purpose of this study was to review our experience with penetrating trauma to the buttock, to establish the frequency of system injury and related morbidity and mortality, and to make recommendations for the evaluation and management of these injuries. Among the 56 system injuries, soft tissue injuries predominated. Of the 25 operative procedures done, eight were for wound care and debridement and seven for rectal injuries; three were orthopedic, two vascular, three genitourinary, and one neurosurgical. One patient had examination under anesthesia, and one had laparotomy for missile trajectory. There were no deaths in this series. Morbidity consisted of nerve injury/defect in three patients, stroke in one patient, and impotence in one. An understanding of the systems at risk in penetrating buttock trauma is necessary for prompt multisystem work-up.

Surgical management of proboscoid herniae.

The management of a large proboscoid umbilical hernia presents challenging problems to the surgeon. As children and parents are concerned with the appearance of the actual hernia they are also intensely interested in the postsurgical result. Presented is a simple one-stage umbilicoplasty that allows repair of the fascial defect and the management of the redundant skin in such a way as to produce a "scarless" and natural-appearing umbilicus.

Epidemiological survey of 6 years' federal health service experience of pyloric stenosis: case report of presentation in a 26-month male.

Congenital hypertrophic pyloric stenosis (CHPS) is primarily a surgical process of the neonate and infant. Diagnosis and treatment of CHPS in a 26-month-old male prompted an inquiry of the military health service database for age of onset, sex, and birth position data. Data were analyzed on 604 cases found in a 71-month time period. The incidence for first-born males was .43 percent, for other than first-born males .34 percent, and for females .091 percent, the peak age being 3 weeks and 1 month old. Children diagnosed at age 6 months and older, as well as adults, were identified. Utilization of the Federal Health Care System data base allowed the study of a large number of patients with a broad spectrum of social, racial, and geographic backgrounds. We feel the study of this data provides a unique understanding of the epidemiology of this entity (CHPS).

A practical approach to wound care in patients with complex enterocutaneous fistulas.

A practical, low cost method for controlling high output enterocutaneous fistulas is presented herein. The technique is advantageous because it provides for excellent protection of the skin, is low in cost and is constructed from readily available materials. It is applicable to all fistulas which do not readily accept prefabricated base plate appliances. Its components are so simple that minimal time for wound care on the part of the surgeon, nursing staff or stomal therapist is required.