Aplicación de los colgajos fasciocutáneos vulvoperineales en cirugía pediátrica: a propósito de un caso / Application of vulvoperineal fasciocutaneous flaps in pediatric surgery: case report

La incidencia de agenesia de vagina es de 1/4000-10000 recién nacidas vivas. La causa más frecuente es el síndrome de Mayer-Rokitansky-Küster-Hauser. Presentamos un caso de reconstrucción vaginal con colgajos fasciocutáneos vulvoperineales de Málaga en una paciente pediátrica afecta de este síndrome, describiendo los pasos del procedimiento y las bases anatómicas en las que se fundamenta. Esta técnica permite la creación de una neovagina sensible, con una cobertura de excelente calidad, con un ángulo de inclinación fisiológico y natural y unaxis anatómico correcto para la relación sexual; se realiza en un solo tiempo quirúrgico y con mínima morbilidad para la paciente. Su principal inconveniente es el crecimiento de pelo dentro de la vagina, aunque se atenúa con el tiempo debido a un fenómeno de atrofia folicular y metaplasia cutánea (AU)

The incidence of vaginal agenesis is 1/4000-10000female alive newborns. The most frequent cause is Mayer-Rokitansky-Küster-Hauser syndrome. A technique for vaginal reconstruction using the vulvoperineal fasciocutaneous flaps (Malaga flaps) in a pediatric patient affected of this syndrome is presented, describing the steps of the procedure and its anatomic basis. This technique allows the reconstruction of an excellent quality and sensible neovagina, with aphysiologic inclination angle which allows the sexua lintercourse, in one single stage operation and with minimal morbidity. The main inconvenient of this procedure is the hair growth inside the vagina, although it diminishes with the passage of time due to follicular atrophy and cutaneous metaplasy (AU)

[Puberal gynaecomastia. Review of nine cases]. / Ginecomastia puberal. Revisión de nueve casos.

Gynaecomastia is more frequent during the adolescence. In this paper, a retrospective study of nine cases diagnosed and treated on a period of six years is presented. The reports of nine patients are reviewed. The mean age at diagnosis was 10.4 years (range 9-12). In all cases a bilateral mammary hypertrophy was detected, symmetrical in 6 cases (67%) and asymmetric in 3 (33%). After a mean follow-up period of 24 months, surgical treatment was indicated: a subtotal subcutaneous mastectomy was performed in all nine cases. Only two patients developed early complications: one wound infection and one haematoma. The long-term plastic results were satisfactory after a mean follow-up postoperative period of 11 months. The general management of this kind of pathologic process is presented. In the authors experience the elective surgical procedure is the subtotal subcutaneous mastectomy as simple procedure with a very short morbidity and excellent plastic results.

[Oculocerebrorenal sydrome of Lowe. Apropos of three cases]. / Síndrome oculocerebrorrenal de Lowe. A propósito de tres observaciones.

We report clinical features and course of Lowe's syndrome with regard to three cases. All of them are males and clear inherited transmission was demonstrated in patients 2 and 3 and was suggested in patient 1. Age at the moment of diagnosis oscillated between 7 and 18 years. The three cases showed weight and height percentiles under p 3. Congenital bilateral cataract and search nystagmus were found in all of them. Profound mental retardation, muscular hypotonicity and diminished or absent tendon reflexes constituted distinctive findings in the neurological area. Among renal manifestations stood out proteinuria, generalized hyperaminoaciduria and tubular renal acidosis, they carried from rickets and growth failure. Cases 1 and 2 has characteristic facies. Patient 1 died after series of recurrent bronchial and pulmonary infections: death happened during Fanconi's syndrome evolution. Cases 2 and 3 are in a stabilized period, with a longer life expectation, although they suffer from residual moderate renal failure.

Bilateral multifocal Wilms' tumor.

A three-year-old boy who presented with symptoms of peritonitis was found to have four Wilms' tumors affecting both kidneys. Individual enucleation of three tumors in the right kidney plus left lower nephrectomy were performed. Chemotherapy was administered for one year. The diagnosis of Wilms' tumor was confirmed on each specimen by the histologic studies. The child remains asymptomatic and developing normally six years after the initial surgical treatment. Bilateral partial nephrectomies is the most conservative of the surgical treatments available for bilateral Wilms' tumor.

[Cortical renal microcysts in Lowe's syndrome]. / Microquistes renales corticales en el síndrome de Lowe.

We report a familial case of Lowe's syndrome with histological and ultrastructural examination of the renal biopsy. The patient was an eleven years old boy with operated congenital bilateral cataracts, mental and psychomotor retardation, hyperexcitability, muscular hypotonia, proteinuria, generalised aminoaciduria, proximal tubular acidosis and reduced glomerular filtrate. The renal biopsy showed, in addition to the alterations in the glomerular corpuscle (mesangial proliferation), proximal tubules (atrophy, dilatation, hyalinous or calcerous cylindres and mitochondrial abnormalities) and interstitium (fibrosis, lymphocytic infiltrate), large number of cortical microcysts, many of with corresponded to Bowman's cystic capsules with small glomeruloid projections.

Ureteral triplication and duplicated opposite kidney with refluxing ureterocele.

A case is reported of ureteral triplication with ectopia of two of the ureters and contralateral duplication with a ureterocele. This patient is the youngest that we have found reported with this type of anomaly and the only one presenting with abdominal distention and an intact but refluxing ureterocele.

[Venous aneurysms of the neck in childhood (author's transl)]. / Aneurismas fusiformes venosos cervicales en la infancia.

Three children with four fusiform venous aneurysms in the neck are described. A correct referral clinical diagnosis had not been established in any of these children. The diagnosis should be made generally on the basis of the physical examination, including the test of the "digital pressure", which is particularly useful to rule out a laringocele. Three of the aneurysms have been treated by surgical removal. Histological examination showed microscopic changes which were related to the time elapsed since the mass was first noticed. Areas of thinning out and sclerosis of the venous wall were evident in one case. The patient with the remaining aneurysm is being followed closely.