The pyloric atresia-junctional epidermolysis bullosa syndrome. Report of a case and review of the literature.

BACKGROUND AND METHODS: The concomitant occurrence of the two rare conditions of pyloric atresia (PA) and inherited epidermolysis bullosa (EB) is not as rare as would be expected. We collected 41 case reports in the world literature and add a personal case in which EB was investigated with modern methods and found to be a GB3-positive/non-Herlitz junctional variant. OBSERVATIONS: Our review of the PA-EB association discloses that it is an autosomal recessive inherited entity in which EB is of the junctional EB (JEB) subtype and PA is a primary manifestation rather than a scarring process secondary to JEB. The disease is thus better called "PA-JEB." Patients with the PA-JEB syndrome present, not uncommonly, with erosions and/or subepithelial cleavage in the respiratory, gastrointestinal, and urinary tracts. In addition, certain facultative features are unique to PA-JEB, ie, obstruction of the ureterovesical junction and high incidence of a peculiar form of aplasia cutis congenita. CONCLUSION: The GB3 monoclonal antibody was found normally expressed in three of three cases, excluding the Gravis-Herlitz variant, in spite of an unmatching EB phenotype in one case. Further studies are needed to assess which of the JEB varieties are present in the PA-JEB syndrome.

Hydatidosis in childhood.

A series of 45 children treated during the period 1959-1978 at the American University of Beirut Hospital is reported. Their ages ranged from 3-15 years. The organs affected were the lungs (67% of cases), liver (40% of cases), brain (9% of cases), kidney, neck and heart (two patients each). Multiple organ affection was noted in nine patients (20%). Because of this distribution and the presence of multiple cysts in the same organ, these patients required 64 surgical procedures for their treatment. The frequency of nonreactors to CF and IHA tests done on 33 patients was 36%, of whom 2 patients had multiple organ involvement. A false preoperative diagnosis was made in 11% of cases. Endocystectomy is the procedure of choice in most cases, combined either with marsupialization of the pericyst to the body cavity, or with closure of the pericyst if the cyst is not infected. Primary pulmonary lobectomy was resorted to in 23% of patients with lung cysts. Aqueous cetrimide solution (1/1000) has been used exclusively as a scolicidal agent during the last 10 years. The transdiaphragmatic approach to cysts in the dome of the liver is recommended and was used in four patients. Selective endobronchial intubation of the contralateral safe lung was used in six patients with lung cysts to prevent aspiration pneumonia and to make the dissection technically simpler. Three deaths were recorded early in this series giving a mortality rate of 6.7%. Methods for reducing complications and mortality are described.

Repair of major defect of the upper extremity with a latissimus dorsi myocutaneous flap: a case report.

The latissimus dorsi myocutaneous flap was first used by Tansini in 1896 and in recent years has been increasingly used for a wide range of surgical reconstructions, particularly as a free flap transfer. We report a case in which an extensive defect of the left arm and shoulder was resurfaced with a latissimus dorsi myocutaneous flap prior to orthopaedic reconstruction of a large defect in the humeral shaft.

Traumatic injuries of the alimentary tract in children.

Traumatic alimentary tract (AT) perforations in children secondary to instrumentation, though rare, can occur at any age, especially in neonates and young infants. Awareness of such a possibility is essential for prompt management to be initiated. Over a 10-year period (1986-1995), eight pediatric patients with AT perforations secondary to instrumentation were managed at the American University of Beirut Medical Center. Five had upper AT injuries that included three hypopharyngeal, one esophageal, and one gastric lesion. The remaining three patients had lower AT injuries involving the transverse colon in one and the rectum in two. All the patients but two (one with a hypopharyngeal and one with an esophageal injury) were managed surgically and all survived. These rare injuries are discussed in addition to the preventive measures to be taken.