Carlos Enes

Hemangioendotelioma hepático infantil. Un caso multifocal y bilobular, asintomático, con regresión espontánea

El hemangioendotelioma hepatico infantil (HHI) es una enfermedad grave y con elevada mortalidad. A pesar de ello, estas lesiones vasculares pueden evolucionar espontaneamente para su resolucion completa entre 12 y 18 meses. La decision de intentar un tratamiento especifico y la eleccion entre las varias opciones terapeuticas continua siendo polemica, especialmente en los casos asintomaticos de afectacion multifocal o bilobular. Describimos un caso de HHI, multifocal y bilobular, asintomatico, no sometido a ningun tipo de terapia y con regresion de las lesiones antes de los 2 anos de edad.

Beckwith-Wiedemann Syndrome, Delayed Abdominal Wall Closure, and Neonatal Intussusception –Case Report and Literature Review

We present the extremely rare case of a male newborn with Beckwith-Wiedemann Syndrome (BWS) presenting as delayed abdominal wall closure and neonatal intussusception. Fetal ultrasound had shown omphalocele that resolved spontaneously. When feeding was attempted, he had various episodes of vomiting. An x-ray showed signs of high bowel obstruction. Jejunal intussusception was found on laparotomy. Enterectomy and primary jejuno-jejunal anastomosis was performed. During post-operative period subtle physical findings became prominent: plain hemangioma, posterior helical indentations, and macroglossia. Cardiac ultrasonography showed a patent foramen oval with small left-to-right shunt. Ultrasonography showed renal hyperplasia. Genetic study showed hypomethylation of DMR2 region of 11p15 chromosome.

Upper pole nephrectomy: a simplified technique using a retroperitoneal laparoscopic approach.

Objective. To describe a simplified technique already used in our institution for several years in the open heminephrectomy for duplication anomalies, now performed through a retroperitoneal laparoscopic approach. Methods. The technique begins with upper pole parenchyma incision since the demarcation between the affected upper moiety and the healthy lower pole is easily established. The dissection proceeds until the urothelium of the collecting system is entered, which will guide further excision, minimizing damage of the surrounding structures. The vascular supply is then identified since the upper pole is attached to the remaining renal parenchyma only by these structures that can be safely divided. Dissection and division of the ectopic ureter is carried next. Results. The operative time was 188 minutes. The blood loss was not significant, and there were no other complications during the procedure. The patient was discharged home 48 hours after the procedure, without any early or late postoperative complications. Conclusion. We believe this simplified technique allows a safer excision of nonfunctioning upper pole renal tissue by avoiding the initial dissection of the renal hilum, which associated with the known advantages of a laparoscopic approach makes us consider it the procedure of choice for upper pole nephrectomy in children.