Department of General, Visceral, Vascular and Pediatric Surgery, University of the Saarland, D-66421 Homburg/Saar, Germany.
J Pediatr Surg. 2011 Sep;46(9):e21-4. doi: 10.1016/j.jpedsurg.2011.06.005.
A 15-year-old adolescent boy with autosomal recessive agammaglobulinemia underwent endoscopy because of unexplained growth failure and malnutrition. Esophagogastroduodenoscopy revealed antropyloric stenosis, and a biopsy showed an invasive gastric adenocarcinoma. Chronic atrophic corpus gastritis type A and Helicobacter pylori were also identified. Abdominal magnetic resonance imaging confirmed the stenosis resulting from a semicircular intramural tumor without obvious local or distant metastatic spread. Gastrectomy with an extended lymphadenectomy was performed. Esophagoduodenal continuity was restored by an interposed jejunal parallel pouch developed from the first jejunal loop. Oral feeding was supplemented by parenteral nutrition via a Broviac catheter, and the patient is well 4 months later. Several cases of gastric cancer have been reported in children with hereditary agammaglobulinemia. Thus, endoscopy is mandatory in such patients with gastrointestinal symptoms to identify and treat tumors before metastasis occurs. Total gastrectomy, extended lymphadenectomy, and reconstruction using a jejunal reservoir with maintenance of duodenal continuity should be considered.
一名 15 岁的男性青少年,患有常染色体隐性遗传性低丙种球蛋白血症,因不明原因的生长发育迟缓及营养不良接受了内镜检查。食管胃十二指肠镜检查显示幽门狭窄,活检显示侵袭性胃腺癌。还发现慢性萎缩性胃体胃炎 A 型和幽门螺杆菌感染。腹部磁共振成像证实狭窄是由半圆形壁内肿瘤引起的,没有明显的局部或远处转移扩散。进行了胃切除术和扩大淋巴结清扫术。通过从第一空肠袢发展而来的间置空肠平行袋恢复食管十二指肠连续性。通过 Broviac 导管进行肠外营养补充口服喂养,4 个月后患者情况良好。遗传性低丙种球蛋白血症患儿已报告多例胃癌病例。因此,对于有胃肠道症状的此类患者,内镜检查是强制性的,以便在转移发生前识别和治疗肿瘤。应考虑全胃切除术、扩大淋巴结清扫术以及使用带有十二指肠连续性的空肠储袋进行重建。