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김웅준 ( Woong Jun Kim ),은창수 ( Chang Soo Eun ),이민규 ( Min Kyu Lee ),민승연 ( Seung Yeon Min ),유연화 ( Yeon Hwa Yoo ),한동수 ( Dong Soo Han ),전용철 ( Yong Cheol Jeon ),손주현 ( Joo Hyun Sohn ) 대한장연구학회 2012 Intestinal Research Vol.10 No.2
A chronic intestinal pseudo-obstruction is a rare disorder and a severe digestive syndrome. It is characterized by deranged gut propulsive motility that resembles a mechanical obstruction, but no obstructive process is present. An intestinal pseudo-obstruction may be classified as acute or chronic; the chronic form may also be classified as idiopathic or secondary to a variety of diseases. Treatment of intestinal pseudo-obstruction involves nutritional, pharmacological, and surgical therapies. Surgery should be limited to patients who are refractory to medical therapy and show a deteriorating course. Despite available medical and surgical interventions, the outcome remains poor. Here, we describe a case of a 54-year-old female with chronic constipation and abdominal distension, who was subsequently found to have segmental aganglionosis. The patient was treated with a subtotal colectomy and ileosigmoidostomy without sequelae. (Intest Res 2012;10:210-214)
성인에서 발생한 대장의 Aganglionosis 1 예
박수철(Su Cheol Park),김병관(Byeong Gwan Kim),김 원(won Kim),우열근(Yeol Keun Woo),이종열(Jong Yeul Lee),이상협(Sang Hyub Lee),예병덕(Byong Duk Ye),김주성(Joo Sung Kim),박규주(Kyu Joo Park),김우호(Woo Ho Kim),정현채(Hyun Chae Jung 대한소화기학회 2001 대한소화기학회지 Vol.37 No.6
Aganglionosis is a rare form of neuronal intestinal malformation, which includes hypoganglionosis, neuronal intestinal dysplasia A (NID type A), B (NID type B) or combined type. Classic aganglionosis, or Hirschsprung’s disease is a neurogenic form of neonatal bowel obstruction characterized by a congenital absence of ganglionic cells in the submucosal and myenteric plexus, and presents in childhood with symptoms of constipation, colonic obstruction or sepsis due to enterocolitis. However, aganglionosis can be diagnosed first in adult due to short segment Hirschsprung`s disease with mild symptom and maybe also due to acquired process. We experienced a case of adult type aganglionosis assumed to be acquired type without malignancy or other neurological involvement. (Korean J Gastroenterol 2001;37:470-474)
이석구,이우용,김현학,Lee, Suk-Koo,Lee, Woo-Yong,Kim, Hyun-Hahk 대한소아외과학회 1998 소아외과 Vol.4 No.1
Early recognition and surgical treatment of Hirschsprung's disease prevents serious mortality and morbidity from enterocolitis and obstruction. Usually this disease is characterized by a single aganglionic segment of the colon extending distally to the anal margin. In surgical treatment, the surgeon performs a frozen section biopsy to confirm whether there are ganglion cells. If there are intervening ganglionic sites in aganglionic bowel, there may be confusion in diagnosis and treatment. The authors experienced one case of total colonic aganglionosis with skip area. A transverse loop colostomy was performed on a 7 day-old male baby with colon perforation due to Hirschsprung's disease. But intestinal obstruction persisted and required two more operations to find the true nature of the disease. There were aganglionic segments from the anal margin to the terminal ileum 3.7cm proximal to the ileocecal valve. The entire transverse colon and appendix were normally ganglionated.
김연수 ( Yeon Soo Kim ),이준성 ( Joon Seong Lee ),김경민 ( Kyung Min Kim ),이보영 ( Bo Young Lee ),김영대 ( Young Dae Kim ),정인섭 ( In Seop Jung ),고봉민 ( Bong Min Ko ),홍수진 ( Su Jin Hong ),유창범 ( Chang Beom Ryu ),김진오 ( J 대한소화기기능성질환·운동학회 2006 Journal of Neurogastroenterology and Motility (JNM Vol.12 No.2
Hirschsprung`s disease is associated with congenital megacolon of the colon proximal to a segment that lacks ganglion cells in the myenteric plexus and submucosal plexus. Hirschsprung`s disease is usually considered as a pediatric disease entity. In the typical cases, the aganglionosis arises from the anal verge and this extends proximally for varying distances; the lesion mainly involves the rectum or rectosigmoid colon. Zonal segmental involvement is very rare even in children. We experienced a 45-year-old male who suffered from acute intestinal obstruction with a collapsed luminal portion in the distal descending colon and proximal sigmoid colon. The patient underwent surgical resection for segmental constriction. We present here a case of zonal adult Hirschsprung`s disease along with a review of the literature. (Kor J Neuro-gastroenterol Motil 2006;12:170-176)
선천성 거대결장증 수술 후 문합부에 발생한 점액성 대장선암: 증례 보고
김형수,윤권하,Kim, Hyung-Soo,Yoon, Kwon-Ha 대한영상의학회 2004 대한영상의학회지 Vol.50 No.1
선천성 거대결장증으로 수술한 후의 장기 추적에서 문합부에 악성종양이 생긴 경우는 보고된 바가 없다. 저자들은 선천성 거대결장증으로 26년전에 Soave 수술을 받은 환자의 문합부에생긴 점액성 대장선암를 경험하였기에 이를 보고하는 바이다. To our knowledge, rectal cancer arising at the anastomotic site after surgery for Hirschsprung's disease has not been reported. We report a case of mucinous adenocarcinoma arising at the anastomotic site after Soave operation 26 years ago.