Aplicación del sistema de presión negativa VAC® en dehiscencia postesternotomía media en pacientes neonatos / Use of vacuum-assisted closure therapy (VAC®) in poststernotomy dehiscence in neonates

El tricoepitelioma, descrito por Brooke en 1892 y también llamado epitelioma adenoideo quístico, es una genodermatosis autosómica dominante originada en el complejo pilosebáceo. Existen dos formas clínicas: la solitaria y la múltiple. Algunos autores recomiendan no tratarlo; sin embargo las lesiones múltiples tienden a deformar la anatomía y la resección parcial presenta recidivas así como cicatrices excesivas y en algunos casos tienen un comportamiento agresivo. Presentamos un caso clínico de tricoepitelioma con deformidad severa de la cara en el que se realizó tratamiento radical con restitución de unidades estéticas mediante colgajos locales. Consideramos que los casos agresivos o deformantes deben ser tratados de forma radical, eliminando todo el espesor de la piel para obtener resultados satisfactorios (AU)

Trichoepithelioma, first described by Brooke in 1892, also known as cystic adenoid epithelioma, is a dominantly inherited genodermatosis originated in the follicle bulb. There are two clinical forms, solitary and multiple. Some authors advocate not to treat them, but multiple trichoepithelioma presents a significant cosmetic problem and partial resection can lead to recurrence and excessive scarring, some cases with an aggressive behavior. We present a clinical case of multiple tricoepithelioma with severe deformity of the face in which we realized radical resection with restitution of anatomical units by local flaps. We consider that the multiple cases should be excised with total skin to avoid recurrence and obtain acceptable results (AU)

Adenomatosis hepática / Hepatic adenomatosis

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[Cholelithiasis in childhood. Proposals based on a multicentric study]. / Colelitiasis en la infancia. Propuestas de un estudio multicéntrico.

Fifty six cases of cholelithiasis in patients aged two months to 15 years (mean age 7.65 years) concerning to 11 hospitals are reviewed. The study protocol followed was the same in all medical records, although own criterions were considered on management performed in each center. From the cases, it follows: 1. Male/female rate is 1/1.5. 2. Symptomatology in infancy is relatively poor and pain localization is not orientative. 3. It was an incidentally finding in 41 per 100 of the cases. 4. Ultrasonography is the best examination procedure rendering diagnosis in the 51 cases it was underwent. 5. Hematologic study was abnormal in six of 46 cases. 6. Medical treatment was not performed in any hospital. 7. Existence of "lithogenic families" seems to be demonstrated. 8. The presence of four patients with Down syndrome in this series must be pointed out. 9. Among total 56 cases, 21 underwent surgical treatment, 29 were conservatively treated, two have died and four patients had spontaneous stone resolution. 10. In the face of these, we propose: A) Surgical treatment in symptomatic cases, porcelain gallbladder and nonfunctionating gallbladder. B) Expectant management and sonographic monitoring in asymptomatic cases. C) Carefully evaluation in patients with predisposing factors and patients with recurrent abdominal pain.

[Incidence of celiac disease in Vizcaya]. / Incidencia de la enfermedad celíaca en Vizcaya.

Celiac disease (CD) is a permanent gluten-sensitive enteropathy appearing in individuals genetcally predisposed. Its incidence varies according to the authors, but is situated about 1/1.500 alive newborn infants (ANI). Recently, a decreased in the incidence of the disease as well as a delay in the onset of symptoms have been reported in several countries. The incidence of the disease in Spain is unknown so we have studied it in our population. In the period 1976-1987, 117 patients were suspected to have CD in the different centers performing intestinal biopsies in Vizcaya. Diagnosis was confirmed in 87 cases, thus implying an incidence of 1/2.151 ANI. Age onset has been stable along the years, and most cases continue to be diagnosed during the first 2 years of life.