Nevo sebáceo de Jadassohn. Localización atípica y síndromes asociados / Nevus sebaceus of Jadassohn. Atypical location and associated syndromes

Hamartoma benigno que puede aparecer desde el nacimiento. Se localiza sobre todo en cuero cabelludo y cara; origina una placa alopécica, amarillenta, de superficie rugosa. Tiene tres etapas, una de hiperplasia epidérmica, luego una puberal con desarrollo de glándulas sebáceas y una tardía con desarrollo de tumores benignos o malignos, en especial siringocistadenoma papilífero y tricoblastomas. Puede presentarse asociado a síndromes genéticos. Su exéresis quirúrgica es objeto de controversia (AU)

Benign hamartoma that can appear from birth. It is located mainly on the scalp and face; It causes an alopecic, yellowish plaque with a rough surface. It has three stages, one of epidermal hyperplasia, then a pubertal stage with development of sebaceous glands, and a late stage with development of benign or malignant tumors, especially papillary syringocystadenoma and trichoblastoma. It can appear associated with genetic syndromes. Its surgical excision is controversial. (AU)

Severe ischaemic gangrene of scalp in an extreme preterm: a fatal case of combined aetiology.

A 24+5-week preterm neonate with a severe scalp lesion was admitted to the neonatal intensive care unit (NICU) after caesarean section due to maternal chorioamnionitis (MC). An Arabin pessary had been inserted in addition to a previous cervical cerclage due to cervix insufficiency at 21+5 weeks of pregnancy (wp). At 23+5 wp, preterm rupture of membranes was evidenced. Both devices were kept to provide fetal viability. On 24+4 wp, she developed MC. Urgent caesarean section was performed. Transvaginal manual manipulation was required during the procedure. On NICU, she presented severe shock which required high-dose vasopressors and blood products. Following surgical repair, a bilateral grade IV intracranial haemorrhage was evidenced. Subsequently, it was agreed to withdraw life support. We hypothesise that MC and local infection could have acted as predisposing factors, with the presence of a pessary in the setting causing uterine contractions and its manipulation acting as a precipitating factor.

Trombosis venosa cerebral como complicación de una otitis media aguda / Cerebral venous thrombosis as a complication of acute otitis media

La trombosis venosa cerebral es una enfermedad poco frecuente y de carácter excepcional. Se trata de una patología probablemente infradiagnosticada y debería incluirse siempre en el diagnóstico diferencial de la hipertensión intracraneal con focalidad neurológica o convulsiones, sobre todo si existen factores de riesgo conocidos. Las imágenes radiológicas son imprescindibles para su diagnóstico. Presentamos el caso de un paciente de 5 años con estrabismo convergente del ojo derecho de 3 días de evolución, en el contexto de otitis medias repetidas del oído derecho parcialmente tratadas con antibioterapia. Exploración patológica donde destacaba paresia del VI par derecho y un tímpano homolateral hiperémico en la otoscopia. Los exámenes complementarios fueron normales, salvo aumento del dímero-D. Las imágenes radiológicas obtenidas con resonancia magnética cerebral en fase venosa confirmaron la sospecha diagnóstica, pautándose anticoagulación, con evolución favorable

Cerebral venous thrombosis is a rare and exceptional disease. It is a pathology probably underdiagnosed and it should always be included in the differential diagnosis of intracranial hypertension with neurological focality and/or convulsions, especially if there are known risk factors. Radiological images are essential for diagnosis. We present the case of a 5-year-old patient with convergent right eye strabismus of 3 days evolution, in the context of repeated ear otitis partially treated with antibiotherapy. Pathological examination highlighting VI right paresis and a hyperemic homolateral eardrum in otoscopy was found. Complementary tests were normal, except for elevation of the D-dimer. Regarding the radiological images obtained with cerebral magnetic resonance with venous-phase, was confirmed, and had a favourable evolution after receiving prescribed anticoagulant treatment

Masa genital en un neonato; orientación diagnóstica / Genital mass in a neonate; diagnostic orientation

El quiste parauretral de Skene es una tumoración quística benigna dependiente de las glándulas de Skene y adyacente al meato uretral, que puede drenar contenido mucoide blanquecino al introito vaginal. El examen físico establece el diagnóstico, quedando el estudio ecográfico para aquellos casos dudosos y que se presentasen con obstrucción urinaria. El manejo debe ser conservador y una correcta aproximación diagnóstica es fundamental para descartar otros procesos que sí precisarían un tratamiento quirúrgico como, por ejemplo, himen imperforado, prolapso de uretra, prolapso de ureterocele, etc. A continuación, describimos el caso de una recién nacida a término en la que la exploración física de las primeras 24 horas de vida reveló una masa genital interlabial

Skene's paraurethral cyst is a benign cystic tumor dependent on the Skene glands and adjacent to the urethral meatus, which can drain whitish mucoid content to the vaginal introitus. The physical examination determines the diagnosis, leaving the ultrasound study for those doubtful cases and that presented with urinary obstruction. Management should be conservative and a correct diagnostic approach is essential to rule out other processes that would require surgical treatment such as imperforate hymen, urethral prolapse, ureterocele prolapse, etc. The case of a full-term newborn in whom an interlabial genital mass was discovered in her first 24 hours of life