Congenital Duodenal Anomalies in the Adult

Abstract

Background: Duodenal anomalies are defects in embryologic development and usually present as gastric outlet obstruction in infancy or early childhood. Occasionally, they remain asymptomatic until adulthood and, because they are unusual, may not be diagnosed. Hypothesis: Based on current experience and review of the literature, recognition of diagnosis and the preferred methods of treatment of duodenal anomalies can be recommended. Design: Retrospective study of congenital duodenal anomalies in adults. Setting: Tertiary care university medical center. Patients: Twenty-nine patients were observed and treated between 1983 and 1999 (19 women and 10 men; mean±SD age, 52 ± 16 years). Twenty patients had duodenal webs, 7 had annular pancreata, and 2 had both. Nausea, vomiting, abdominal pain, and weight loss were predominant symptoms in all groups. Peptic ulceration occurred in 13 of 20 patients with webs but in none of those with annular pancreata or combined anomaly. Main Outcome Measures: Surgical outcomes including postoperative complications, deaths, and resolution of preoperative symptoms. Results: The treatment for patients with duodenal webs was transduodenal web excision and duodenoplasty in 19 of 22. Patients with annular pancreata were treated by transection of the annulus and duodenoplasty (n=4) and proximal duodenal bypass (n=3). There were no operative deaths, but 44% of patients had some complications. No pancreatic fistulas occurred in patients who had division of an annular pancreas. Outcome was considered excellent or good in 17 of 20 patients with duodenal webs, 4 of 7 with annular pancreata, and 2 of 2 with the combined anomaly. Conclusions: Duodenal anomalies are rare in adults. Duodenal webs are best managed by transduodenal excision and duodenoplasty. Annular pancreas is generally best treated by duodenal bypass to the distal duodenum or the jejunum. Annulus division can be carried out if the annulus is extramural, without duodenal stenosis, and if access to the pancreaticobiliary sphincters is necessary.

Pâncreas anular: ressecção pancreática ou derivação duodenal

“…A origem embriológica do pâncreas anular tem início entre a quinta e sétima semana gestacional, quando os dois brotos pancreáticos, dorsal e ventral, acompanham o processo de rotação intestinal. Neste período, o duodeno sofre uma rotação da esquerda para a direita normalmente acompanhada pelo broto pancreá-tico ventral que migra posterior e inferiormente fundindo-se à porção mais caudal do broto dorsal 8 . Assim, o broto ventral dá origem a porção caudal da cabeça pancreática e ao processo uncinado, enquanto o broto dorsal se desenvolverá em corpo e cauda do pâncreas.…”

“…Embora suas manifes- 12 . Uma recente revisão da literatura mundial encontrou 160 casos diagnosticados na fase adulta 8 . Apesar do pâncreas anular produzir sintomas de obstrução duodenal ou biliar, as apresentações clínicas são variáveis.…”

“…A realização de uma laparotomia, em um paciente com sintomas obstrutivos, com identificação de uma banda de tecido pancreático que circunda a segunda porção do duodeno, reforça a hipótese diagnóstica confirmada com o exame da peça ressecada. O anel pancreático pode circular total ou parcialmente o duodeno, aderir-se fracamente à superfície serosa duodenal ou então se apresentar intensamente interligado com as fibras musculares da parede do duodeno 8,10 .…”

“…Ladd et al descrevem o relato de nove pacientes com pâncreas anular em que cinco deles foram submetidos à ressecção pancreática associada a duodenoplastia com bom resultado pós-operatório e sem desenvolvimento de fístula pancreática 8 . McGuinness 11 utilizou a sutura mecânica (stapler) para a ressecção de pâncreas anular em um paciente de 61 anos, o qual evoluiu favoravelmente e sem complicações, demonstrando a exeqüibilidade do procedimento e inclusive questionando a derivação duodenal como procedimento de escolha para o tratamento do pâncreas anular.…”

Duodenal Carcinoma in a Patient with an Annular Pancreas: Rarity Personified

“…It has been described 14 times in the medical archives (five pancreatic carcinomas, six ampullary carcinomas, two cholangiocarcinomas, and one in sulinoma [9][10][11][12][13][14][15][16][17][18][19][20]). …”