[Distal pancreatectomy as surgical option in Pancreas divisum associated with Rubinstein-Taybi syndrome].

Abstract

INTRODUCTION:

The Rubinstein-Taybi syndrome and pancreas divisum are uncommon diseases in children. The Rubinstein-Taybi syndrome diagnosis is only clinical, and mental retardation and dysmorphic features mainly characterize it. The PD relates to idiopathic pancreatitis in 7.5% of patients suffering from this disorder. It has not been found any cases reported where these diseases are associated and the management of patients with pancreatitis associated with PD is still a challenge.

CLINICAL CASE:

A patient diagnosed with Rubinstein-Taybi syndrome has recurrent abdominal pain of six months and elevated pancreatic enzymes, he underwent a magnetic resonance imaging where pancreas divisum is suspected. Endoscopic drainage was foiled so he underwent intraoperative retrograde pancreatography where aberrant pancreas is confirmed and a distal pancreactectomy and yeyunopancreatic anastomosis is the chosen option.

COMMENTS:

The pancreatectomy with pancreato-jejunal anastomosis is an excellent therapeutic option surgical is proposed for a patient with difficulty endoscopic approach and chronic pancreatitis.

• Authors: H Delgado Bartra, F M Lapouble Ramírez, J Bonilla Pabón, S T Vega Centeno
• Journal: Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica (Cir Pediatr) Vol. 28 Issue 2 Pg. 84-87 (Apr 15 2015) ISSN: 0214-1221 [Print] Spain
• Vernacular Title: Pancreatectomía distal como opción quirúrgica en Páncreas divisum asociado a síndrome de Rubinstein-Taybi.
• PMID: 27775287 (Publication Type: Case Reports, English Abstract)