Tovar J A, Eizaguirre I, Albert A, Jimenez J
J Pediatr Surg. 1983 Feb;18(1):1-6. doi: 10.1016/s0022-3468(83)80262-0.
In this article, we report two new cases of Peutz-Jeghers syndrome in children and review the literature over the past twenty years. This series of 70 cases demonstrates that the clinical pictures observed in children are similar to those of adults. Rectal prolapse or extrusion of polyps can be the clue to the diagnosis at an early age, even in the absence of pigmentation, which can appear later. Gastroduodenal polyps were strikingly frequent in the less than or equal to 16-yr-old group (62%) a circumstance that can create operative difficulties. Five out of the 70 patients (7.14%) had tumors during childhood (two gastrointestinal adenocarcinomas, two ovarian and one testicular neoplasms). A higher risk of tumor development in these patients does exist either as a result of degeneration of the polyps or of a genetic predisposition. Whenever operation becomes necessary, a very cautious approach must be advised in order to preserve as much intestinal length as possible in these patients, who have a lifelong disease which may require repeated operations.
在本文中，我们报告了两例儿童黑斑息肉综合征新病例，并回顾了过去二十年的相关文献。这一系列70例病例表明，儿童观察到的临床表现与成人相似。直肠脱垂或息肉脱出可能是早期诊断的线索，即使在没有色素沉着的情况下也是如此，色素沉着可能会在以后出现。在小于或等于16岁的组中，胃十二指肠息肉非常常见（62%），这种情况可能会造成手术困难。70例患者中有5例（7.14%）在儿童期发生肿瘤（2例胃肠道腺癌、2例卵巢肿瘤和1例睾丸肿瘤）。由于息肉退变或遗传易感性，这些患者确实存在较高的肿瘤发生风险。每当需要手术时，必须建议采取非常谨慎的方法，以便在这些患有终身疾病且可能需要反复手术的患者中尽可能保留肠道长度。
