Malformación Vascular Linfática en un Recién Nacido / Vascular Lymphatic Malformation in a Newborn

Las malformaciones vasculares son enfermedades poco frecuentes que representan errores en el desarrollo de los vasos sanguíneos. Las malformaciones vasculares linfáticas (MVL) consisten en lesiones congénitas del sistema linfático compuestas por canales y cavidades de diferente tamaño que resultan en la acumulación de linfa. Las malformaciones macroquísticas se manifiestan como tumoraciones palpables, de consistencia blanda pero no compresible. La localización más frecuente es en la cabeza y el cuello Se presenta el caso de un neonato con una malformación vascular linfática localizada en zona postero-lateral de cuello. Se arribó al diagnóstico en base a la clínica y las características ecográficas. El tratamiento fue muy satisfactorio luego de dos procedimientos de escleroterapias, presentando una excelente evolución

Vascular malformations are rare conditions associated to anomalies in blood vessel development. Lymphatic vascular malformations are congenital lesions consisting of channels and sacs of different size caused by lymph fluid acumulation. Macrocystic vascular malformations present as non-compresible soft tissue masses. They are generally found withinthe head and neck (70%-80%). We present the case of a newborn with a lymphatic vascular malformation in the posterior cervical área. The diagnosis was made on the basis of clinical and ultrasound findings. Sclerotherapy was succesful wihout signs of recurrence

Neurofibromatosis tipo 1. La necesidad de un reconocimiento clínico precoz / Type 1 neurofibromatosis. The need of an early diagnosis

La neurofibromatosis tipo 1 (NF-1) es una entidad claramente relacionada con la aparición de neoplasias en los niños. La NF-1 ha sido demostrada como factor pronóstico adverso ante neoplasias de nervios periféricos y del sistema nervioso central. El retraso diagnóstico y con ello la disminución en los porcentajes de resecabilidad, son factores que atentan contra la curación de estos pacientes. Por ello nos pareció oportuno revisar los criterios de diagnóstico, menejo y derivación para detectar en tiempo útil al portador de NF 1 e ingresarlo en seguimiento prospectivo, intentando de esa manera disminuir la morbimortalidad por tumores que ellos presentan.

Pólipos umbilicales y otras anomalías del conducto onfalomesentérico / Umbilical polyps and other abnormalities of the omphalomesenteric duct

El conducto onfalomesentérico es una estructura tubular que une el intestino primitivo con el saco vitelino del embrión. Normalmente esta estructura desaparece obliterándose completamente al final de la 5º o 6º semana de vida intrauterina, pero en ocasiones puede persistir ocasionando diferentes anomalías como fístulas, quistes vitelinos o pólipos umbilicales. Los pólipos son lesiones tumorales rojo brillante que se observan en la cicatriz umbilical tras la caída del cordón.Se presentan 10 casos de anomalías del cierre del conducto onfalomesentérico, de los cuales 7 fueron varones y 3 mujeres. 9 pacientes presentaron pólipos umbilicales, 2 de ellos con fístulas enterocutáneas, y una paciente un quiste vitelino. Las edades de consulta fueron desde los 4 días de vida hasta los 6 años de edad. En 8 pacientes se realizaron estudios histopatológicos, que evidenciaron mucosa gastrointestinal en contacto con epidermis, en 2 casos se realizaron estudios por imágenes contrastadas, encontrándose fístulas enterocutáneas, y en uno fue necesaria una laparotomía exploradora para llegar al diagnóstico. Es importante conocer estas anomalías umbilicales ya que por su embriogénesis, pueden asociarse con alguna de las otras alteraciones derivadas de la falla del cierre del conducto onfalomesentérico, las cuales pueden ocasionar consecuencias graves para el paciente, de no realizarse el diagnóstico oportuno y el tratamiento correcto (AU)

Congenital self-healing histiocytosis (Hashimoto-Pritzker).

BACKGROUND: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition, initially seen at birth or in the neonatal period, with generalized papules, vesicles, or nodules. Affected infants are otherwise well and the skin lesions tend to involute spontaneously within weeks to months. METHODS: Twelve patients with CSHLCH were seen from 1989 to 1998. RESULTS: Eight patients were girls and four were boys and all presented with lesions at birth which disappeared 1-3 months later. The lesions consisted of numerous brownish-red papules, papulovesicles, crusts, and nodules distributed on the face, limbs, palms, and soles. Two patients had oral mucosal lesions, and one had ulcerated lesions that evolved leaving hypochromic macules. Light microscopy showed a histiocytic infiltrate in the papillary dermis with epidermotrophism. Two cases were studied by electron microscopy: the Langerhans cells showed Birbeck granules and laminated corpus in their cytoplasm. Immunomarking with S100 protein was performed in all 12 patients and was positive. CD1 was also tested in four cases and was positive. CONCLUSIONS: Because CSHLCH is a rare condition, we emphasize that, although it is usually a benign, self-limited entity, careful evaluation for systemic disease must be performed and long-term follow-up must be carried out to detect evidence of relapse or progression of the disease; this is essential when treating these patients.

Congenital hemangioma with milialike structures: a case report.

A 4-month-old infant had two 3 cm x 4 cm hemangiomatous lesions on the scalp and back, present since birth, which contained peculiar white-yellowish small nodules. Histologically the lesions proved to have a hemangiomatous (capillary-type) component together with small keratin-containing epidermal cysts (milia-like) which progressively extruded their contents. The lesions also contained embryonic-like hair structures. The hemangiomas resolved spontaneously. We were unable to find any reference in the literature describing this peculiar combination of features.

Disseminated cutaneous eruption after BCG vaccination.

We present nine infants (3 to 10 months of age) with numerous small, papular, papular-lichenoid, and papulo-pustular lesions predominantly on the upper and lower limbs associated with local (axillary) lymphadenopathy which appeared after BCG vaccination. Histopathology of the lesions showed small tuberculoid granulomas mainly in the papillary dermis. The presence of BCG bacillus was demonstrated in five out of seven samples from the lymph nodes after culture and in one skin biopsy specimen. All cases, whether treated or not, evolved to complete resolution of the skin lesions. We believe that this peculiar association results from hematogenous spread of the bacillus, which regresses after an adequate immune system reaction.

Fox-Fordyce disease in two prepubertal girls: histopathologic demonstration of eccrine sweat gland involvement.

This report presents two prepubertal girls with Fox-Fordyce disease. The pruritic papules extensively affected the areas where apocrine glands are distributed (axillae, periareolar and intermammary zones, pubes, infraumbilical midline), and also extended to the neck and face near the external angle of the eyes in one child. Analyses of several biopsy specimens showed that the main lesion was a spongiotic vesicle containing inflammatory cells and keratinocytes affecting the hair infundibula and acrosyringia, together, with hyperkeratosis of both adnexa. The cause of the disease remains elusive, but the microscopic findings may explain the good results obtained with keratolytic agents.

Congenital hemangioma of eccrine sweat glands.

We present six patients with congenital hemangioma of eccrine sweat glands. In every one the lesion was congenital, clinically angiomatous, painless, and nonsweating, with progressive involution over months. Histologically all specimens showed many dilated capillaries with prominent endothelial cells associated with the eccrine sweat gland coils.