[Vascular malformations as syndromic markers]. / Malformaciones vasculares como marcadores sindrómicos.

Vascular malformations are static lesions, generally present at the moment of birth, formed by displasic vessels that grow in proportion to the growth of the child. They show normal cell replacements that constitute genuine mistakes of morphogenesis. The absence of regression of these malformations implies that they remain throughout the lifetime. The terminology describing this type of lesions gave rise to confusion in the medical literature until the International Society for the Study of Vascular Anomalies adopted a classification based on the dominant vessel of the malformation in 1996. This classification distinguishes between simple and complex forms depending on whether they affect one or several types of vessel. Vascular malformations can show themselves as isolated lesions or can be associated with other lesions, constituting the guide sign or being the principal marker of some syndromic complexes. This paper describes the principal signs and symptoms of those syndromes in which a vascular malformation is the key that raises suspicion about the existence of other associated lesions.

Malformaciones vasculares como marcadores sindrómicos / Vascular malformations as syndromic markers

Las malformaciones vasculares son lesiones estáticas, generalmente presentes en el momento del nacimiento, formadas por vasos displásicos que crecen de forma proporcional al crecimiento del niño. Presentan un recambio celular normal que constituyen verdaderos errores de la morfogénesis. La ausencia de regresión de estas malformaciones implica que permanezcan toda la vida. La terminología que describe este tipo de lesiones ha sido motivo de confusión en la literatura médica hasta que en 1996 la Sociedad Internacional para el Estudio de las Anomalías Vasculares adoptó una clasificación basada en el vaso dominante de la malformación. Esta clasificación distingue entre formas simples y complejas según se afecte un tipo de vaso o varios. Las malformaciones vasculares pueden presentarse como lesiones aisladas o bien asociarse a otras lesiones, constituyendo el signo guía o ser el marcador principal de algunos complejos sindrómicos. En este trabajo se describen los principales signos y síntomas de aquellos síndromes en los que una malformación vascular es la clave que hace sospechar la existencia de otras lesiones asociadas (AU)

Social behavior, cortisol, and sIgA levels in preschool children.

OBJECTIVE: This study aims to explore the possible existence of behavioral states in early stages of development, which are associated with specific cortisol production profiles and consequently with changes in the immune response [secreted immunoglobulin A (sIgA)]. METHODS: The subjects were 27 boys and 21 girls of preschool age whose behavior was videotaped in free play interactions. Their behavior was then evaluated using an ethogram of the social behavior of the child. Cortisol levels were measured using a radioimmunoassay in saliva samples, and sIgA levels were measured by means of an ELISA, also in saliva samples. RESULTS: No correlation between cortisol and sIgA levels was found. Variance analysis revealed that the only behavioral pattern, which showed significant differences with regard to cortisol levels, was isolation. CONCLUSIONS: There seems to be a connection between cortisol levels and isolation behavior; we found no relation between high cortisol levels and a lower sIgA secretion.

[The psoas bladder-hitch. An illustrative case]. / Vejiga psoica. Caso ilustrativo.

The psoas bladder hitch is an invaluable surgical procedure in the treatment of lesions of the lower ureteral third and transplantation with short ureters. We briefly review this procedure and describe an illustrative case of ureteroneocystostomy of the right lumbar ureter.

[Cytology positive for malignant cells in hydrocele secondary to testicular seminoma]. / Citología positiva a células malignas en hidrocele secundario a seminona testicular.

Finding tumor cells in the fluid of hydrocele secondary to testicular tumor is rare and may be due to the fact that hydrocele fluid cytology has not been studied systematically. The presence of malignant cells in the hydrocele fluid may be ascribed to two mechanisms: retrograde lymphatic flow from the infiltrated retroperitoneal lymph nodes or from tumor directly invading the tunica albuginea and vaginalis testis, with cells passing into the cavity of the vaginalis. Only two cases of hydrocele secondary to testicular seminoma with vaginalis fluid cytology positive for malignant seminomatous cells have been previously reported in the literature. To our knowledge, the case reported herein is the third. These three cases are briefly discussed.

[Fournier's gangrene. Presentation of 11 cases and review of the literature]. / Gangrena de Fournier. Presentación de 11 casos y revisión de la literatura.

Fournier's gangrene is an infectious necrotizing cellulitis of the penis and/or perineoscrotal region, which rapidly progresses and carries a high mortality. Eleven cases of Fournier's gangrene that had undergone an emergency procedure from 1985 to 1992 are described. Excellent results were achieved, with a survival rate of 91%. In our view, treatment of this disease entity is fundamentally based on early diagnosis, wide radical debridement of the affected area, frequent local treatment, debridement at the least sign of recurrence and early antibiotic therapy with three agents.

[Primary retroperitoneal hydatidosis]. / Hidatidosis retroperitoneal primaria.

The case of a primary retroperitoneal hydatidosis in a 62 year-old patient found accidentally during an haematuria study is presented. Both the possible extraperitonization mechanisms and efficacy of ultrasound scanning and percutaneous puncture in the etiological diagnosis in cases of retroperitoneal mass are evaluated.

[Bilateral renal adenocarcinoma: relationship with phakomatosis]. / Adenocarcinoma renal bilateral: relación con la facomatosis.

Von Hippel-Lindau disease is a very uncommon finding in our environment. A case of an incidental finding of that entity expressed through retinal angioma, bilateral renal cysts and bilateral renal carcinoma is described. A surgical course was followed performing a radical left nephrectomy, given the large size of the tumour, and right lower partial nephrectomy, as well as a review of the renal cysts considering the big tendency they have to degenerate in the disease.

[Leiomyosarcoma and liposarcoma: rare tumors of the kidney]. / Leiomiosarcoma y liposarcoma: tumores infrecuentes de riñón.

Sarcoma accounts for 2.8% of all renal tumours. We report a case of renal leiomyosarcoma (T2 N0 M0) and a case of renal liposarcoma (T3 N0 M0) submitted to surgery and chemo- and radiotherapy. Follow-up at nine months and 4 years, respectively, revealed the patients are tumor-free. The literature is reviewed highlighting the beneficial effect of chemo- and radiotherapy combined with surgery in the treatment of these tumors with a poor prognosis per se.

[Kidney hydatidosis]. / Hidatidosis renal.

Seven cases of hydatid renal cyst seen at our department over the past 8 years are described. The literature is reviewed and the importance of conservative surgery in the treatment of this disease is highlighted.

[Renal carcinoma presenting as a multilocular cyst]. / Carcinoma renal presentado como quiste multilocular.

Although it is not uncommon to find small foci of cystic degeneration in renal adenocarcinoma, it is uncommon to find these so extensive as to permit a preoperative diagnosis of multilocular cyst as in the present case. Several etiopathogenic mechanisms have been described. In the present case, the underlying mechanisms are likely to be tumor necrosis and intrinsic multilocular cystic growth. Arteriography is not the best diagnostic method and there are doubts as to the usefulness of punction aspiration. Prognosis remains unclear.