PDF(Pubmed)
Abstract：
BACKGROUND: Hirschsprung\'s disease is a congenital disorder identified by the absence of ganglion cells at the Meissner\'s plexus of the submucosa and Auerbach\'s plexus of the muscularis. This disease can be found in approximately 1 in 5000 live births. It is a congenital disorder that is rarely diagnosed in adults, where 95% of cases are diagnosed in infants aged under 1 year old. Here we present a rare case of adult Hirschsprung\'s disease to enrich the body of knowledge in diagnosing adult patients with chronic refractory constipation symptoms.
METHODS: An 18-year-old Indonesian woman came to the general surgery department of Unggul Karsa Medika Teaching Hospital with a defecating problem (constipation) since childhood. There was no history of her passage of meconium. A contrast enema study showed that the sigmoid colon was dilated and the rectum was narrowed, with rectosigmoid index < 1. With these findings, it was suspected that the patient may have ultra-short segment Hirschsprung\'s disease. The patient was then referred to the digestive surgery department of referral hospital for surgical treatment.
CONCLUSIONS: In adult patients presenting with history of constipation since childhood, it is necessary to consider the possibility of Hirschsprung\'s disease that was not diagnosed in early childhood. Hirschsprung\'s disease in adults is usually a short or ultra-short aganglionic segment because it shows relatively mild symptoms. Surgical removal of the aganglionic segment of the gut is the definitive treatment for Hirschsprung\'s disease.
METHODS: An 18-year-old Indonesian woman came to the general surgery department of Unggul Karsa Medika Teaching Hospital with a defecating problem (constipation) since childhood. There was no history of her passage of meconium. A contrast enema study showed that the sigmoid colon was dilated and the rectum was narrowed, with rectosigmoid index < 1. With these findings, it was suspected that the patient may have ultra-short segment Hirschsprung\'s disease. The patient was then referred to the digestive surgery department of referral hospital for surgical treatment.
CONCLUSIONS: In adult patients presenting with history of constipation since childhood, it is necessary to consider the possibility of Hirschsprung\'s disease that was not diagnosed in early childhood. Hirschsprung\'s disease in adults is usually a short or ultra-short aganglionic segment because it shows relatively mild symptoms. Surgical removal of the aganglionic segment of the gut is the definitive treatment for Hirschsprung\'s disease.
摘要：
背景：先天性巨结肠病是一种先天性疾病，由粘膜下层的Meissner神经丛和肌层的Auerbach神经丛缺乏神经节细胞而确定。这种疾病可以在大约5000名活产儿中发现。这是一种先天性疾病，很少在成人中被诊断出来，其中95％的病例被诊断为1岁以下的婴儿。在这里，我们介绍了一例罕见的成人先天性巨结肠病，以丰富诊断患有慢性难治性便秘症状的成人患者的知识。
方法：一名18岁的印尼妇女从小就因排便问题（便秘）来到UnggulKarsaMedika教学医院普外科。没有她通过胎粪的历史。对比灌肠研究显示乙状结肠扩张,直肠狭窄,直肠乙状结肠指数<1。有了这些发现,怀疑患者可能患有超短段先天性巨结肠症。然后将患者转诊至转诊医院消化外科进行手术治疗。
结论：在从小就有便秘史的成年患者中，有必要考虑在儿童早期未被诊断的先天性巨结肠的可能性。成人先天性巨结肠病通常是短或超短的神经节段，因为它表现出相对轻微的症状。手术切除肠的神经节段是Hirschsprung病的最终治疗方法。
方法：一名18岁的印尼妇女从小就因排便问题（便秘）来到UnggulKarsaMedika教学医院普外科。没有她通过胎粪的历史。对比灌肠研究显示乙状结肠扩张,直肠狭窄,直肠乙状结肠指数<1。有了这些发现,怀疑患者可能患有超短段先天性巨结肠症。然后将患者转诊至转诊医院消化外科进行手术治疗。
结论：在从小就有便秘史的成年患者中，有必要考虑在儿童早期未被诊断的先天性巨结肠的可能性。成人先天性巨结肠病通常是短或超短的神经节段，因为它表现出相对轻微的症状。手术切除肠的神经节段是Hirschsprung病的最终治疗方法。
