A perspective for pediatric surgeons: chronic idiopathic intestinal pseudoobstruction

Chronic idiopathic intestinal pseudoobstruction (CIIP) is a disorder of alimentary tract motility with onset of symptoms in adolescence or young adulthood, characterized by recurring attacks of abdominal pain, distention, vomiting, and constipation often leading to malnutrition and death. Starting with an adolescent patient, treated for a succession of life-threatening attacks of pseudoobstruction, the authors have identified CIIP in four generations of a Virginia family. Studies show characteristic dilation and diminished motility of the esophagus and intestine. Surgery's role in CIIP is palliative, and operation should only be offered when medical measures have failed to control symptoms.     

Visceral myopathy causing chronic intestinal pseudoobstruction and intestinal failure in a child with Sanjad-Sakati syndrome

Sanjad-Sakati syndrome is a rare autosomal recessive disorder mainly occurring in the Arab Peninsula. This condition is associated with metabolic and septic complications starting in the neonatal period. Chronic intestinal pseudoobstruction owing to visceral myopathy is a rare disabling condition. We report a rare concurrence of Sanjad-Sakati syndrome and chronic intestinal pseudoobstruction in a Saudi child complicated by intestinal failure, sepsis, and early mortality.     

肝硬化大鼠小肠Cajal间质细胞超微结构及平滑肌细胞线粒体膜电位的变化

目的研究肝硬化大鼠小肠Cajal间质细胞超微结构的改变及平滑肌细胞线粒体膜电位的变化,探讨肝硬化胃肠动力障碍的相关机制。方法 20只Wister大鼠随机分为肝硬化模型组和对照组,每组10只,采用CCl4溶剂大腿根部皮下注射法制作肝硬化模型,用葡聚糖蓝-2000作为胃肠内标记物,观察大鼠肠道传输,透射电镜观察小肠Cajal间质细胞超微结构的改变,激光共聚焦显微镜检测小肠平滑肌细胞线粒体膜电位的改变。结果与对照组比较,肝硬化组大鼠小肠动力明显减弱(P0.01),透射电镜观察显示空肠Cajal间质细胞的超微结构发生明显改变,线粒体等细胞器明显减少(P0.01)。小肠平滑肌线粒体膜电位显著低于对照组(P0.01)。结论肝硬化大鼠肠道传输减弱与肠道Cajal间质细胞超微结构改变,细胞器减少及线粒体膜电位的降低有关。     

Chronic intestinal pseudoobstruction syndrome in adults

The aim of this paper is to debate, based on medical literature review, the chronic intestinal pseudoobstruction syndrome in adults, from the surgical point of view. The beginning of the paper emphasizes the difficulties in pseudoobstruction syndrome definition and etiology, and then we discuss the importance of intestinal histological assessment (whenever it is possible) and intestinal structure modifications. A very important part of this paper is reserved to the diagnostic difficulties, especially to those between intestinal pseudoobstruction syndrome and mechanical intestinal obstructive syndrome (similar etiologic circumstances, similar clinical and radiological manifestations). Finally, this paper debates the therapeutic difficulties, emphasizing the importance of surgical methods and techniques useful in treatment of the patient with chronic intestinal pseudoobstruction syndrome.     

Isolated congenital megacystis without intestinal obstruction: a mild variant of chronic intestinal pseudoobstruction syndrome?

Megacystis is frequently involved with chronic intestinal pseudoobstruction syndrome; however, isolated megacystis without intestinal obstruction is extremely rare. We present the case of a female patient with isolated congenital megacystis without severe intestinal obstruction. In this case, barium enema did not reveal any significant findings; however, histologic evaluation of her rectum showed hypoganglionosis of the submucous and myenteric plexuses. These findings indicate that this case may be a mild variant of chronic intestinal pseudoobstruction syndrome. The presence of megacystis should alert the physician to the possibility of chronic intestinal pseudoobstruction syndrome.     

Intestinal and multivisceral transplantation in children with severe gastrointestinal dysmotility

Background/Purpose

Severe gastrointestinal dysmotility (GID) impairs patients' quality of life and is almost uniformly fatal after complications of parenteral nutrition. Intestinal and multivisceral transplants have been used as alternative treatment of these disorders. We studied patients with GID treated with transplantation in our center, and reviewed their outcome to determine the therapeutic efficacy of multivisceral transplants.

Methods

The transplant database was searched for patients with GID from 1994 to 2001. We excluded patients with Hirschsprung disease, scleroderma, and diabetic enteropathy. We reviewed explanted organs, histochemistry, and immunohistochemistry and classified cases by etiology.

Results

We selected 12 children with GID from 124 patients transplanted. Nine presented before 1 year and 3 started with symptoms between 2 and 8 years. By combined clinical and histopathological features, 6 were classified as megacystis microcolon intestinal hypoperistalsis syndrome, 4 as chronic idiopathic intestinal pseudoobstruction, and 2 as intestinal neuronal dysplasias. Six patients died during the follow-up from 21 to 546 days after transplant. The Kaplan-Meier actuarial survival rates were 66.7% at 1 year and 50% at 3 years.

Conclusions

Multivisceral transplantation is a valuable therapeutic alternative for children with severe GID who cannot be adequately managed with parenteral nutrition.     

Stomal prolapse in children with chronic intestinal pseudoobstruction: a frequent complication?

Aim

The aim of the study was to evaluate the morbidity rate of stoma in children diagnosed with chronic intestinal pseudoobstruction (CIPO) and try to determine risk factors.

Material and Methods

Twenty-two children (65%) of 34 referred to our center between 1988 and 2008 had a stoma. They were compared with 22 other children referred for another pathology necessitating a stoma.

Results

The incidence of stomal prolapse in CIPO children was 45% vs 9% in non-CIPO children (P = .01). Prolapse occurred between the first postoperative day and the 10th postoperative month, with a median of 2 months. Surgical management was required in 60%, with an intestinal necrosis rate of 20% leading to intestinal resection. No mortality was noted. No risk factors favoring prolapse in CIPO children were identified.

Conclusion

Children with CIPO have a high rate of stomal prolapse with an increased risk of intestinal necrosis. Careful management of the stoma is necessary to avoid the risk of intestinal resection, which may aggravate the underlying intestinal disorder.     

Akute intestinale Kolonpseudoobstruktion 2 Tage nach Versorgung einer pertrochantären Femurfraktur

Acute colonic pseudoobstruction, following traumatic injuries, is a rare diagnosis. Nevertheless it is life threatening, if it is not recognized and treated promptly. We report one case of this so-called Ogilvie's syndrome, which followed fixation of a trochanteric fracture by intramedullary nailing within 2 days. Due to massive acute colonic distension, the patient suffered from respiratory failure. We excluded other intestinal diseases by CT scanning. After conservative colonic decompression, he recovered after 2 days in the intensive care unit. We describe a variable treatment depending on the severity of the colonic atony. Knowledge of Ogilvie's syndrome, or acute colonic pseudoobstruction, is a must for trauma surgeons, since it can occur within a few hours and can lead to dramatic situations.     

Ninety-five cases of intestinal transplantation at the university of Miami

Intestinal failure requiring total parenteral nutrition (TPN) is associated with significant morbidity and mortality. Intestinal transplantation can be a lifesaving option for patients with intestinal failure who develop serious TPN-related complications. The aim of this study was to evaluate survival, surgical technique, and patient care in patients treated with intestinal transplantation. We reviewed data collected from 95 consecutive intestinal transplants performed between December 1994 and November 2000 at the University of Miami. Fifty-four of the patients undergoing intestinal transplantation were children and 41 were adults. The series includes 49 male and 46 female patients. The causes of intestinal failure included mesenteric venous thrombosis (n = 12), necrotizing enterocolitis (n = 11), gastroschisis (n = 11), midgut volvulus (n = 9), desmoid tumor (n = 8), intestinal atresia (n = 6), trauma (n = 5), Hirschsprung’s disease (n = 5), Crohn’s disease (n = 5), intestinal pseudoobstruction (n = 4), and others (n = 19). The procedures performed included 27 isolated intestine transplants, 28 combined liver and intestine transplants, and 40 multivisceral transplants. Since 1998, we have been using daclizumab (Zenepax) for induction of immunosuppression and zoom videoendoscopy for graft surveillance. We began to use intense cytomegalovirus prophylaxis and systemic drainage of the portal vein. The 1-year patient survival rates for isolated intestinal, liver and intestinal, and multivisceral transplantations were 75%, 40%, and 48%, respectively. Since 1998, the 1-year patient and graft survival rates for isolated intestinal transplants have been 84% and 72%, respectively. The causes of death were as follows: sepsis after rejection (n = 14), respiratory failure (n = 8), sepsis (n = 6), multiple organ failure (n = 4), arterial graft infection (n = 3), aspergillosis (n = 2), post-transplantation lymphoproliferative disease (n = 2), intracranial hemorrhage (n = 2), and fungemia, chronic rejection, graft vs. host disease, necrotizing enterocolitis, pancreatitis, pulmonary embolism, and viral encephalitis (n = 1 case of each). Intestinal transplantation can be a lifesaving alternative for patients with intestinal failure. The prognosis after intestinal transplantation is better when it is performed before the onset of liver failure. Rejection monitoring with zoom videoendoscopy and new immunosuppressive therapy with sirolimus, daclizumab, and campath-1H have contributed to the improvement in patient survival. Presented at the Forty-Second Annual Meeting of The Society for Surgery of the Alimentary Tract, Atlanta, Georgia, May 20–23, 2001 (oral presentation).     

内脏肌病致急性假性结肠梗阻

内脏肌病是一种由内脏平滑肌萎缩、缺失和纤维化引起的罕见疾病。病变常累及消化道,临床表现随病变部位和程度不同而异。小肠病变可表现为腹胀、腹泻及呕吐等消化不良症状,累及结肠则表现为急性假性肠梗阻。本病可继发营养不良、低蛋白血症等。常与麻痹性肠梗阻,慢性便秘,进行性全身硬化等疾病相混淆,诊断上存在一定困难。本病病程进展通常较缓慢,远期预后不良。本文回顾性分析吉林大学白求恩第一医院收治的l例内脏肌病致急性假性结肠梗阻患者的临床资料,总结该疾病的诊断和治疗经验。     

The clinical significance of pathological studies of congenital intestinal atresia

Objective

The purpose of this study was to explore the mechanisms of postoperative intestinal motility disorders in intestinal atresia patients by investigating the expression profiles of proteins, including calretinin (CR), glial-derived neurotrophic factor (GDNF), bone morphogenetic protein 2 (BMP-2), c-kit, α-smooth muscle actin (α-SMA), and S-100 protein; to decipher the correlation between the area of the pathological segment and the alteration of the above 6 proteins; and thereby to provide a clinical specific reference values to determine the removal length for intestinal tract resection.

Methods

Immunohistochemistry technique was applied to detect the CR, c-kit, GDNF, BMP-2, α-SMA, and S-100 protein in specimens of atretic, proximal, and distal intestine from 25 cases of intestinal atresia and samples of intestinal walls from 10 non-atresia control specimens. The alteration of the enteric nervous system, nerve growth and its regulatory factors, the interstitial cells of Cajal (ICCs), and the enteric muscle system were examined, with particular attention being paid to pathological changes and the lesion area.

Results

The expression of all of the abovementioned 6 proteins in the proximal side of the atresia was significantly lower than in control group. The expression of the abovementioned proteins tended to be higher farther away from the atresia site. The expressions of both GDNF and BMP-2 had returned to normal level at 10 cm proximal to the atresia site, whereas the expressions of CR, c-kit, α-SMA, and S-100 protein only returned to normal at 15 cm proximal to the atresia site. On the distal side, the expression of all 6 markers at 3 cm distal to the atresia site was normal.

Conclusion

Pathological deterioration of the myenteric ganglia, nerve growth factor, and ICCs are the causes of intestinal motility disorders after the surgical repair of intestinal atresia. Our data support resecting an intestinal segment extending from 15 cm proximal to 3 cm distal to the atretic segment. In proximal jejunal atresia, when it is not possible to resect 15 cm, we suggest resecting as much of the hypertrophic proximal intestine as possible. Based on our data, we believe this surgical practice could improve postoperative dysmotility in these patients.     

Familial visceral myopathy: the role of surgery

Familial visceral myopathy is a form of chronic intestinal pseudoobstruction in which fibrosis of the smooth muscle of the alimentary tract causes protean symptoms of disordered visceral motility. Based on our 8 year observation of six members of one family with familial visceral myopathy and review of the few well-documented case reports of this disease, we have confirmed a role for surgeons in tissue diagnosis, case identification, and palliation of incapacitating symptoms of familial visceral myopathy after obtaining dynamic radiographic assessment of the entire alimentary canal. Because the duodenum and colon are the usual sources of major dysmotility symptoms, patients incapacitated by duodenal or colonic pseudoobstruction may benefit from bypass or resective operations.     

Congenital interstitial cell of cajal hyperplasia with neuronal intestinal dysplasia

Interstitial cells of Cajal (ICCs) are intestinal pacemaker cells that initiate peristalsis in the stomach and intestine, and are considered to be precursors of gastrointestinal stromal tumors (GISTs). We report a 2-year-old girl who suffered from scanty stool passage since birth. On barium enema, the distal colon was rigid with narrow lumen, whereas the proximal colon was dilated and atonic. She received right hemicolectomy and ileostomy. Histopathologically, there was continuous proliferation of spindle cells located between the layers of the muscularis propria throughout the right colon. These spindle cells were positive for c-kit and CD34 but negative for myogenic or neurogenic markers, indicating they are ICCs. No germline or somatic mutation of the juxtamembrane domain of c-kit gene was detected. In addition, the changes of the submucosal plexus fulfilled the histologic criteria of neuronal intestinal dysplasia type B. To our knowledge, this is the first reported case of congenital ICC hyperplasia. Further studies of ICC development may contribute to better understanding of the pathogenesis of this congenital malformation and the tumorigenesis of GIST.     

Mechanical compression with secondary ischemia as a possible cause of atresias associated with omphalocele

We report 2 cases of omphalocele associated with intestinal atresia. In the context of this unusual sequence, we discuss the etiopathogenesis of intestinal atresias in association with tight abdominal wall defects.     

Ingestion of magnets: innocent in solitude, harmful in groups

Foreign body ingestion is frequent in children and generally associated with little morbidity. However, some foreign bodies are innocent when ingested as a single object, but may have harmful effect if numerous. We report a 9-year-old girl who swallowed 5 magnets, causing acute intestinal obstruction. At laparotomy, 2 magnets were found in the cecum and 3 in the transverse colon, attracting each other and clasping a segment of ileum in between, causing a complete obstruction of the small intestine. If numerous magnets are ingested, particular concern is advised, and if signs of intestinal distress develop, prompt laparotomy to prevent serious gastrointestinal complications should be performed.     

Colonoscopic decompression for acute pseudoobstruction of the colon (Ogilvie's syndrome). Report of 22 cases and review of the literature

This report has described a series of 22 patients who underwent colonoscopic decompression for acute pseudoobstruction of the colon and summarizes those cases previously reported in the literature. Twenty of the 22 patients (91 percent) were successfully treated by decompression initially. Fifteen patients (68 percent) were cured with the initial procedure, and 4 patients (18 percent) experienced recurrence. Overall, in 17 patients (77 percent), the pseudoobstruction resolved completely with colonoscopic decompression. Three patients (14 percent) underwent operation because of cecal dilatation refractory to colonoscopic decompression, and in one patient (4.5 percent), the colonic dilatation resolved spontaneously after a failed colonoscopy. Complications resulted in the death of one patient (4.5 percent). Our data are similar to those in the literature and indicate that colonoscopic decompression is a safe and efficacious first line of treatment for acute pseudoobstruction of the colon.     

Acute colonic pseudoobstruction in a child with sickle cell disease treated with neostigmine

Sickle cell disease is a disorder that produces significant morbidity and mortality. Vaso-occlusive pain crises are the most common presenting symptom associated with sickle cell patients. A rare, yet important to recognize, complication of sickle cell disease is acute colonic pseudoobstruction, also known as Ogilvie's syndrome. These patients may present with symptoms that are difficult to distinguish from other etiologies of abdominal pain, but a thorough diagnostic workup can provide important clues. Furthermore, there is no agreement on optimal treatment of pseudoobstruction. We report the first pediatric case of acute pseudoobstruction secondary to sickle cell disease that was treated successfully with neostigmine. Early recognition of this phenomenon is important as it alters patient management, can be treated medically, and may avoid unnecessary surgical intervention.     

Diffuse Intestinal Diverticulosis: a Case Report

We report an asymptomatic case of diffuse intestinal diverticulosis simultaneously affecting duodenum, jejunum and colon. The patient presented signs of progressive systemic sclerosis, which supports the hypothesis in the literature of an aetiological link.     

Intestinal malrotation associated with colon cancer in an adult: Report of a case

Intestinal malrotation is a congenital anomaly of intestinal rotation and fixation that generally becomes symptomatic during the neonatal period. However, it rarely occurs in adulthood, and synchronous colon cancer is even rarer. This report presents the case of a 45-year old man who suffered from intestinal malrotation associated with colon cancer.     

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目的 探讨小肠内置管排列术的临床疗效。方法 对1996～2003年采用改良的White手术，即小肠内置管排列术治疗病情较为复杂的肠梗阻35例，包括19例急症手术者。主要原发病为肠粘连和小肠炎性肠病等。结果 随访时间为1个月至3年，远期有效率为96．7％(29／30)，主要并发症为肠漏(25．7％)、低血容量性休克(5．7％)和膀胱损伤(5．7％)。结论 小肠内置管排列术是治疗复杂性肠梗阻的有效方法，正确选择适应证和积极的营养支持有助于减少并发症。肠功能衰竭的机制和诊治方法有待于深入研究。