Management of ampullary tumours in children: still a challenge.

This study describes the case of the youngest patient ever reported with ampullary adenoma. The incidence of ampullary adenomas in childhood is unknown. Endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound are used in adults to assess and treat these lesions, although there are no instruments designed specifically for use in young children. A six-year-old girl was admitted for abdominal pain, vomiting, pruritus and weight loss. Abdominal ultrasound showed biliary tree (8mm) and pancreatic duct dilatation (4mm). Magnetic resonance cholangiopancreatography and computed tomography confirmed these findings, and also showed displacement of the ampulla to the left upper quadrant. An upper endoscopy confirmed a large ampullary adenoma. A laparotomy was performed and a 5cm villous tumour arising from the ampulla was excised. The postoperative course was uneventful. The histology demonstrated adenoma of the ampulla (intestinal type) without low-grade dysplasia. all clinical and radiological parameters are normal at 20 months follow-up. We describe the case of the youngest patient ever reported with ampullary adenoma. Pancreaticoduodenectomy carries high morbidity and mortality rates, and therefore it should be avoided in absence of histologically proven malignancy. We believe that surgical ampullectomy is a safe and oncologically correct procedure until better endoscopic instruments for peadiatric use will be designed.

[Supernumerary intrascrotal ectopic spleen: discontinuous splenogonadal fusion]. / Bazo ectópico supernumerario intraescrotal: fusión esplenogonadal discontinua.

Splenogonadal fusion is a rare congenital malformation that results from abnormal connection of splenic tissue with the gonad or the mesonephric structures during embryonic development. It is usually detected in males and is classified into two types, continuous and discontinuous. We present the case of a 3-year-old boy with an asymptomatic scrotal mass that was suspicious for discontinuous splenogonadal fusion on Doppler ultrasonography. The diagnosis was confirmed by surgical excision and histological analysis. Splenogonadal fusion is a benign anomaly that has sometimes led to unnecessary orchidectomy because of suspicion that the mass represented a malignant tumor. Consequently, it is essential to include this malformation in the differential diagnosis of scrotal masses in children.

Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney.

Müllerian malformations include a broad range of anomalies, resulting from the incomplete formation and/or differentiation of the Müllerian ducts. The uterus didelphys with obstructed hemivagina represents the result of a lateral nonfusion of the Müllerian ducts with asymmetric obstruction, and it is almost always associated with renal agenesis. We report a case of incidental discovery of this anomaly in a 12-year-old girl during a routine renal ultrasound. A right multicystic dysplastic kidney had been detected in the prenatal and neonatal period with no evidence of uterine anomaly. Postnatal ultrasound examinations performed regularly had never detected either right renal tissue or the uterine anomaly before menarche. Hematocolpos after menarche made uterine anomaly detection easier. Magnetic resonance imaging performed to evaluate the uterine malformation detected a dysplastic right kidney. We review the present knowledge and recommendations for the diagnosis and treatment of this anomaly, and we emphasize the use of magnetic resonance imaging not only for the evaluation and classification of Müllerian malformations, but also for the detection of the persistence of renal tissue missed with other studies.