Primary hepatic paraganglioma (PGL) is a rare neuroendocrine tumor characterized by clinical manifestations including paroxysmal hypertension, palpitation, abdominal pain and constipation. In the present study, the case of a 21-year-old woman with pathologically confirmed hepatic PGL with megacolon following surgery is reported. The patient initially visited Beijing Tiantan Hospital (Beijing, China) for hypoferric anemia. A triple-phase CT scan of the whole abdomen showed a large hypodense mass with a solid periphery and strong arterial enhancement of the peripheral solid portion of the liver. The sigmoid colon and rectum were obviously distended, filled with gas and intestinal contents. The patient was preoperatively diagnosed with iron deficiency anemia, liver injury and megacolon and then underwent partial hepatectomy, total colectomy and enterostomy. Microscopically, the liver cells exhibited an irregular zellballen pattern. In addition, immunohistochemical staining revealed that liver cells were positive for CD56, chromogranin A, vimentin, S-100, melan-A and neuron-specific enolase. Therefore, the diagnosis of primary PGL of the liver was confirmed. These findings suggested that primary hepatic PGL should not be excluded when megacolon occurs and comprehensive imaging evaluation is of great importance for its diagnosis.

暂无摘要。

OBJECTIVE: To summarize the experience of surgical treatment of children diagnosed with Currarino syndrome, with an emphasis on the selection of an optimal operative approach.
METHODS: The clinical materials of patients diagnosed with Currarino syndrome were recorded. Special attention was given to the operative management, particularly the different routes for operation. The type of ARM was the critical point. The Rintala score was used for the evaluation of bowel function.
RESULTS: The medical records of 26 patients were reviewed. Seven were male, and 19 were female, with a mean age of 19.38 ± 13.80 months. The standard posterior sagittal approach (SPS) group included three perineal fistulae, one anal stenosis, one retraction of the rectum after anoplasty for vestibular fistula, one ARM with no fistula, one rectourethral fistula, and one cloaca. In the limited posterior sagittal approach (LPS) group, there were 13 perineal fistulae, one displacement of the rectum, and one retraction of the rectum after anoplasty for the vestibular fistula. In addition, the transanal approach (TA) and anterior sagittal approach (AS) were also used. The mean follow-up time was 39.48 ± 26.84 m. The Rintala score was 16.74 ± 2.93.
CONCLUSIONS: For a perineal fistula, SPS or LPS should be used to reach anoplasty and remove the presacral mass. For a vestibular fistula, the AS or LPS should be chosen. For anal stenosis, SPS or LPS should be used.

Anal canal duplication is a rare gastrointestinal malformation characterized by extra anal orifices at 6 o\'clock in the lithotomy position. To date, there have been only 110 reported cases. The purpose of this study is to contribute two infant cases, one of which is associated with anorectal stenosis, which has never been described.

UNASSIGNED: To explore the treatments and short-term effects of different types of adult Hirschsprung\'s disease.
UNASSIGNED: 89 patients treated in Shanghai Changhai Hospital were retrospectively analyzed. According to the patient\'s medical history, clinical manifestations, auxiliary examination and postoperative pathological results, the patients were divided into adult congenital megacolon, adult idiopathic megacolon, ganglion cell deficiency (types I and II), toxic megacolon and iatrogenic megacolon, The Treatment methods and short-term prognosis of patients in each group were summarized.
UNASSIGNED: 41 cases of Hirschsprung\'s disease in adults and low anterior resection or pull-out low anterior resection was performed, and 35 patients with idiopathic Megacolon were treated with one-stage subtotal colon resection under the condition of adequate preoperative preparation. Some patients admitted for emergency intestinal obstruction received conservative treatment first or underwent elective surgery after colonoscopic decompression was improved; two patients with ganglion cell deficiency subtotal colectomy were performed to remove the dilated proximal bowel segment and the narrow distal bowel segment; three patients with toxic Hirschsprung\'s disease underwent colostomy in mild cases, while subtotal colorectal resection was required in severe cases; Iatrogenic megacolon was diagnosed in eight cases and the optimum operation should be selected according to the specific conditions of patients.
UNASSIGNED: Adult Hirschsprung\'s diseases were divided into adult congenital hirschsprung\'s disease, idiopathic Hirschsprung\'s disease, ganglion cell deficiency, toxic hirschsprung\'s disease, and iatrogenic Hirschsprung\'s disease. Different types of surgical treatments for Hirschsprung\'s disease in adults should be selected according to the specific diagnosis. All patients with adult Hirschsprung\'s diseases have good short-term outcomes after surgical treatment.

Adult megacolon is a rare disease with heterogeneneous etiology. The treatment schemes of megacolon caused by different causes are also different, but surgery is the final and the most effective method. Due to the lack of early understanding of the disease, many patients have not been clearly diagnosed as adult megacolon and have not been properly treated. This article classifies adult megacolon according to the etiology and summarizes its surgical options. For adult Hirschsprung\'s disease, modified Duhamel, the Jinling procedure, low anterior resection, or pull-through low anterior resection can be used. For patients with idiopathic megacolon, one-stage subtotal colorectal resection can be selected with adequate preoperative preparations. Some patients admitted to the hospital with emergency intestinal obstruction can be treated with conservative treatment or decompression under colonoscopy followed by selective surgery. For patients with aganglionosis, the procedure is subtotal colorectal resection, the same as that of idiopathic megacolon. The procedure is to remove both the dilated proximal intestine and the stenotic distal intestine, then an ileorectal anastomosis or ascending colon rectal anastomosis is performed. For toxic megacolon, colostomy can be done for mild cases, and for severe infections, subtotal colorectal resection is required. Latrogenic megacolon is mostly caused by segmental stenosis or lack of peristalsis, resulting in chronic dilatation of the proximal end and the formation of megacolon. It is necessary to choose a reasonable surgical procedure according to the specific conditions of the patient. The first choice for the treatment of acute colonic pseudo-obstruction syndrome is decompression under colonoscopy. For those with the secondary changes in the intestine, ostomy is still the most effective surgical procedure, but should be performed with caution.
成人巨结肠是一类少见疾病，而不同病因所导致的巨结肠，治疗方案有所不同，但手术是最终也是最有效的方法。有碍于早期对疾病的认识不足，很多患者并没有明确诊断为成人巨结肠，并未得到恰当的治疗。本文对成人巨结肠根据病因进行分类，并总结其术式选择。对于成人先天性巨结肠，可以选用改良Duhamel、金陵术、低位前切除术或者拖出式低位前切除术。对于特发性巨结肠患者可在术前准备充分的情况下，选择一期结直肠次全切除术治疗，部分以急诊肠梗阻入院的患者可先行保守治疗或者结肠镜减压好转后行择期手术治疗。对于神经节细胞缺乏症患者，手术方式采用和特发性巨结肠一致的结直肠次全切除术，将扩张的近端肠段和狭窄的远端肠段一并切除，行回肠直肠吻合或者升结肠直肠吻合。对于中毒性巨结肠，轻症者可行结肠造口术，存在严重感染者，需行结直肠次全切除术。医源性巨结肠多是因为医疗原因造成结肠局部或一段肠管发生狭窄或者无蠕动功能而导致近端慢性扩张而形成巨结肠，需要根据患者具体情况选择合理术式。急性假性结肠梗阻综合征的治疗首选方法为结肠镜减压，存在肠道继发性改变者，造口术仍是最有效的手术方案，但需要谨慎开展。.

OBJECTIVE: This study explored the clinical effects of WeChat-based peri-operative care on parents of children with congenital megacolon.
METHODS: Participants were randomly divided into WeChat group and telephone group. This study explored parents\' knowledge of the care of children with megacolon, the follow-up rate of children, post-operative defaecation function and complications.
RESULTS: WeChat group scored better in nursing knowledge than telephone group, and the difference was statistically significant. The lost follow-up rate in WeChat group was lower than that in telephone group, and the difference was statistically significant. Post-operative defaecation was also better in the WeChat group than in the phone group. Most complications in the phone group were significantly higher than those in the WeChat group.
CONCLUSIONS: Peri-operative care for parents of children with megacolon through WeChat can effectively enhance the level of parental care knowledge, improve defaecation, reduce the occurrence of certain complications and reduce lost follow-up.

暂无摘要。

Idiopathic megacolon (IMC) is an uncommon disease in adults. To date, only a few laparoscopic experiences and functional outcomes of IMC have been reported. This study was to retrospectively analyse our 12 year surgical experience and functional outcomes in adult patients with IMC.
A 12-year retrospective study from October 2006 to November 2018 was performed for patients with IMC who underwent surgical interventions. Patients who underwent laparoscopic-assisted colectomy and Duhamel procedure with ileorectal or colorectal anastomosis were collected. Clinical data of surgery and functional outcomes were analysed.
A total of 13 patients who underwent surgical interventions were included in the study. Seven patients underwent laparoscopic total colectomy with ileorectal anastomosis (Duhamel procedure), one patient underwent laparoscopic total colectomy with end ileostomy because of acute intestinal obstruction, while five other patients underwent laparoscopic segmental colectomy with colorectal anastomosis (Duhamel procedure). The mean operative time was 181.6 min (range 150-246). The mean estimated blood loss was 75.6 ml (range 40-200). The mean postoperative hospital stay was 8.2 days (range 6-13). There was no conversion to an open procedure and no surgical mortality. Postoperative diarrhoea was the most prominent complaint during the early period after total colectomy. All patients showed adaptation to the defaecation frequency 3-6 months postoperatively, and had a good quality of life in long-term follow-up.
Laparoscopic-assisted colectomy with Duhamel procedure is a safe and efficient technique for IMC in adults. The scope of colon resection and the type of anastomosis should be individually selected.

BACKGROUND: Intestinal hypoganglionosis most commonly presents in infancy or childhood, with only a few cases reported in adults. Those are mainly diagnosed after elective surgery for long-standing constipation and megacolon.
METHODS: We report a case of a 48-year-old female from China who presented with symptoms of discontinuation of bowel movements for 2 months. A hard, round mass could be felt in her right lower abdomen.
METHODS: The following examination methods diagnosed acquired segmental sigmoid hypoganglionosis. An abdominal computed tomography revealed a dilatation of the colon and suspicious wall thickening of the sigmoid colon. Anorectal manometry revealed relaxation of the anal sphincter. Histological examination revealed lower numbers and the degeneration of ganglion cells.
METHODS: Sigmoidectomy and transverse colostomy.
RESULTS: The patient recovered well from surgery. Three months after the surgery, barium enema revealed a recovery in colorectal dilatation.
CONCLUSIONS: This case could help raise awareness of acquired segmental hypoganglionosis. Resection of TZ and enterostomy presents an effective remission strategy for patients at risk of anastomotic leakage due to poor intestinal conditions.