Dilated Small Bowel Research Articles

Perils of total colonic aganglionosis presenting in neonatal age.

The purpose of this study is to review the cases of total colonic aganglionosis seen in the span of ten years at a pediatric surgery unit of a tertiary care public hospital in New Delhi. Medical records of twelve patients with total colonic aganglionosis were retrieved. Ten out of the twelve patients were males; seven were of the Muslim community. Average recorded birth weight was 2.2 kg. Ten patients presented with features of intestinal obstruction, while two presented with perforation peritonitis. Among the cases of obstruction, Hirschsprung's disease was suspected in eight cases (one was associated with Shah-Waardenburg syndrome), one case each was preoperatively diagnosed as ileal atresia and meconium ileus. Abdominal X-rays at presentation of all the neonates except in one with Shah-Waardenburg syndrome showed multiple air fluid levels. Contrast enema was done in five patients. It showed micro-colon in two patients, and typical question mark sign, dilated small bowel with transition zone in hepatic flexure and normal caliber colon in one each. All the patients underwent exploratory laparotomy. Intra-operatively, the transition zone was seen at distal ileum in ten cases and at hepatic flexure and transverse colon in one each. Biopsies of all the twelve patients eventually showed absence of ganglion cells in entire colon. Ileostomy was done in nine cases, colostomy in two and primary Kimura's procedure in one (this patient was discharged and lost to follow up). Left colonic patch with Swenson's pull through with ileostomy was done for one patient on colostomy. His stoma was closed; he was eventually discharged and lost to follow up. In the other patient with colostomy, the stoma was closed and an ileostomy was created. Of all the patients on ileostomy, three expired in the immediate postoperative period. Four were lost to follow up. Two underwent Kimura's procedure; and expired few months later. One patient on ileostomy is awaiting further treatment. The outcomes for total colonic aganglionosis in those presenting in neonatal age tend to be unsatisfactory in the developing countries.

Sonographic diagnosis of intestinal anisakiasis presenting as small bowel obstruction.

To evaluate the accuracy of ultrasonography (US) in the diagnosis of intestinal anisakiasis presenting as small bowel obstruction. We retrospectively reviewed the clinical features, US examinations, and CT scans of seven patients with intestinal anisakiasis presenting as small bowel obstruction. We compared the performances of US and CT in demonstrating peritoneal fluid, small bowel dilatation, and segmental edema of Kerckring's folds. All patients presented with a history of ingesting raw saltwater fish or squid less than 2 days prior to presentation at the emergency department and had significant levels of Anisakis-specific IgE. Both US and CT revealed small bowel obstruction, with dilated small bowel in all patients and accumulations of free peritoneal fluid in six patients. CT showed segmental wall thickening and luminal narrowing with submucosal edema distal to the distended small bowel; US did so in four patients. Although US was inferior to CT in demonstrating the segmental intestinal edema causing small bowel obstruction, it can be applied in suspected cases of intestinal anisakiasis presenting as small bowel obstruction, particularly where CT is unavailable or there are concerns about radiation exposure.

Circular Versus Linear Versus Hand-Sewn Gastrojejunostomy in Roux-en-Y-Gastric Bypass Influence on Weight Loss and Amelioration of Comorbidities: Data Analysis from a Quality Assurance Study of the Surgical Treatment of Obesity in Germany.

Background: Since January 1 2005, the outcomes of bariatric surgeries have been recorded in Germany. All data are registered prospectively in cooperation with the Institute of Quality Assurance in Surgery at Otto-von-Guericke University Magdeburg.Methods: Data are collected in an online data bank. Data collection began in 2005 for the results of Roux-en-Y-Gastric Bypass (RYGB). In addition to primary bariatric operations, data regarding the complications and the amelioration of comorbidities have been analyzed. Participation in the quality assurance study is required for all certified centers in Germany.Results: Roux-en-Y Gastric Bypass is the most popular bariatric operation in Germany. There were 5115 operations performed from 2005 to 2010. A circular anastomosis was performed in 1587 patients, and a linear anastomosis was performed in 2734 patients. In 783 patients, the hand-sewn technique was used. The leakage rate for the linear technique is 1.6%, and the leakage rate is 1.2% for circular anastomosis, and 1.4% for hand-sewn technique.Conclusion: Roux-en-Y-Gastric Bypass is a popular procedure in Germany. The complication rate has decreased since 2005. The amelioration of comorbidities is not influenced by the anastomosis technique. Additional data are necessary to evaluate the long-term effect of linear versus hand-sewn and versus circular-stapled gastrojejunal anastomosis regarding pouch dilatation, small bowel dilatation, and consecutive weight loss.

Surgical options to enhance intestinal function in patients with short bowel syndrome.

This review will provide the practitioner with an understanding of the spectrum of nontransplant surgical options for managing patients with short bowel syndrome (SBS). Intestinal lengthening procedures are a promising therapy to allow autonomy from parenteral nutrition. The recently described serial transverse enteroplasty is an effective procedure that is easier to perform and has similar outcomes to the more standard longitudinal lengthening procedure described by Bianchi. There are several surgical options for management of the SBS, including construction of intestinal valves or reversed intestinal segments, interposition of segments of colon, or intestinal lengthening procedures. The choice of technique is dictated by the patient's underlying pathophysiology and includes such factors as intestinal transit time, length of remnant bowel, presence of intact colon, and degree of small bowel dilation. Nontransplant surgical interventions are important adjuncts to the elimination of parenteral nutrition dependence and need for intestinal transplantation.

English

Duplication of gut presenting as perforation, peritonitis in an adult is a very rare clinical presentation. Here we report a case of an adult female patient in generalized peritonitis was caused by perforation of tubular communicating, duplicated, Ileal loop which rotated at its attachment with main ileum. This condition was managed by performing laparotomy, excision of the duplicated gut segment followed by end to end ileoileal anastomosis. CASE REPORT: A 32 yrs. old female presented in surgical OPD with the history of generalized abdominal pain, gradual distension and vomiting off and on for the last 3-4 days. She had been suffering from episodes of these symptoms for past couple of months, suggestive of sub-acute intestinal obstruction. However for last few days, these symptoms were aggravated. Patient had been empirically put on ATT by clinicians whom she had been consulting before coming to our hospital. Patient had undergone laparotomy about 8 years ago for perforated appendix. Patient appeared quite ill, dehydrated with tachycardia tachypnea and mild fever. Physical examination, revealed distended abdomen with tenderness and guarding all over. Rebound tenderness was also present. No hepatosplenomegaly was detected. P/V and P/R examination were within normal limits. A right paramedian scar from previous surgery was present. Hematological profile revealed normal study. X-ray abdomen erect view showed multiple Air fluid levels. USG study of abdomen showed mesenteric thickening with mild fluid and dilated bowel loops. She was taken up for Exploratory Laparotomy after correcting her dehydration. Abdomen was opened through mid-line incision which revealed dilated small bowel loops with free fluid. Exploration revealed about one feet long tubular, dilated and duplicated gut which had rotated upon its own axis where it was joining with the main gut, communicating at both proximal and distal ends, causing obstruction and perforation. (Fig. 1, Fig. 2). The segment had no independent mesentery. It derived it's vascularity from the main ileal wall segment with which it was communicating (as shown by a hand inserted in the intervening empty space between the two segments in the Fig. 2). This segment was about 2.5 ft. away proximal to ileocecal junction. This duplicated gut segment was resected and an end to end ileoileal anastomosis was done. No evidence of abdominal Koch's was detected. Her post-operative period remained uneventful and she was discharged fifteen days after surgery.

Single-incision laparoscopic surgery for intersigmoid hernia.

Intersigmoid hernia is a rare form of internal hernia. Here, we report a case of intersigmoid hernia and provide a brief review of the 62 cases involving the mesosigmoid reported in Japan from 2000 to 2013. In the current case, a 26-year-old man with no previous history of abdominal surgery presented with abdominal pain and vomiting. Abdominal computed tomography revealed an extensively dilated small bowel and a closed loop of small bowel in the mesosigmoid. The patient was diagnosed with an intestinal obstruction due to an incarcerated internal hernia involving the mesosigmoid. There was no blood flow obstruction at the incarcerated bowel. An elective single-incision laparoscopic surgery was performed after decompression of the bowel using ileus tube. As the ileum herniated into the intersigmoid fossa, the patient was diagnosed with an intersigmoid hernia. The incarcerated small bowel was reduced in order to make it viable, and the hernial defect was closed with interrupted sutures. The patient had an uneventful recovery and was discharged on postoperative day five.

A spontaneous strangulated transomental hernia: Prospective and retrospective multi-detector computed tomography findings

Transomental hernias are among the rarest type of all internal hernias which overall account for less than 6% of small bowel obstructions. Most transomental hernias occurring in adults are either iatrogenic or post-traumatic. More rarely, a spontaneous herniation of small bowel loops may result from senile atrophy of the omentum. We report a case of an 86-year-old male who presented with signs and symptoms of small bowel obstruction but had no past surgical or traumatic abdominal history. At contrast-enhanced multi-detector row computed tomography (CT), a cluster of fluid-filled dilated small bowel loops could be appreciated in the left flank, with associated signs of bowel wall ischemia. Swirling of the mesenteric vessels could also be appreciated and CT findings were prospectively considered consistent with a strangulated small bowel volvulus. At laparotomy, no derotation had to be performed but up to 100 cm of gangrenous small bowel loops had to be resected because of a transomental hernia through a small defect in the left part of the greater omentum. Retrospective reading of CT images was performed and findings suggestive of transomental herniation could then be appreciated.

English

Colitis is the most common extraocular manifestation of cytomegalovirus (CMV) infection in human immunodeficiency virus (HIV) infected patients. CMV disease is usually seen in HIV-infected patients with CD4+ counts less than 50/ mm 3 . There are documented reports of CMV gastrointestinal disease in patients with CD4+ count greater than 50 cells/L. A 46-year-old man with HIV1 infection on irregular antiretroviral treatment presented with low grade fever, abdominal pain and vomitings. He is a known alcoholic. Physical examination revealed pallor, evidence of malnutrition and tenderness in the abdomen. Laboratory investigations revealed mild anaemia; CD4+ count was 240 cells/L. Fundus examination of the patient was normal. Contrast enhanced computed tomography (CECT) of the abdomen revealed dilated small bowel loops, thickening of wall of splenic flexure and thickening of caecal and terminal ileal wall with mild narrowing. As anti-CMV antibodies (IgM) and CMV real time-polymerase chain reaction (PCR) tested positive, patient was treated with intravenous ganciclovir for 14 days followed by oral valganciclovir and patient showed remarkable improvement. Our case highlights the fact that CMV colitis can also occur in patients with relatively preserved CD4+ counts especially if co-morbid conditions like alcoholism co-exist.

English

Caecal volvulus is a rare condition and caecal bascule, a variant of caecal volvulus is even rarer. Despite similar clinical presentation, initial vigil during clinical examination helps in furthering the diagnosis. The objective of treatment in case of caecal volvulus is to avoid any complication such as gangrene with resection being the only choice if gangrene has already developed. The present case of a 53 year old male, is a typical representation of a caecal volvulus complicated by presence of gangrene confirmed on laparotomy. Resection of 5.7 cm of involved gangrenous part averted any further complication. Follow-up and reversal of ileostomy was done after 6 weeks with an ileo-ascending anastomosis. No complication was reported during the follow up. INTRODUCTION: Cecal volvulus is a rare condition, and its incidence is reported to range from 2.8 to 7.1 per million people per year [1]. It is characterised anatomically by the axial twisting that occurs involving the caecum, terminal ileum, and ascending colon [2, 3]. Caecal bascule is a variant of this condition associated with the upward and anterior folding of the ascending colon and accounts for about 10% of all caecal volvulus case sp [3]. Despite anatomical differentiation, the clinical profile of caecal volvulus and caecal bascule is very similar and is often manifested in terms of intestinal obstruction and strangulation [2, 3]. Clinical presentation of volvulus cases is very diverse and is characterized by intermittent episodes of abdominal pain to unbearable pain leading to medical emergencies [3, 4]. Owing to its rare occurrence, it is difficult to diagnose. In this paper, we report a case of cecal volvulus with extremely rare caecal bascule variant seen in a 55-year-old man, diagnostic method, treatment planning and management of the case is discussed CASE REPORT: A 55 year old male presented with complaints of pain for the last 5 days followed by distension of abdomen for the last 2 days. He was also vomiting for the past 24-hours. On examination, generalized distension with tenderness was felt. Tympanic note over left upper quadrant was noticed. There were no bowel sounds and no evidence of sepsis. Erect X-Ray of abdomen showed dilated small bowel loops with a large gas-filled loop with haustrations placed obliquely which are characteristic of caecal bacsule. On exploratory laparotomy, caecum & appendix were found to be gangrenous and lying over splenic flexure of colon. Resection of gangrenous segment 5.7 cm in length was done with exteriorization of ileum & ascending colon. Patient recovered and was discharged with ileostomy in situ on day ___. Follow-up and reversal of ileostomy was done after 6 weeks with an ileo-ascending anastomosis.

P-156 Shear Wave Velocity Measurement in Bowel Wall Using ARFI Ultrasound for Prediction of Response to Medical Therapy in Crohnʼs Disease

Crohn’s disease (CD) is a chronic inflammatory disease necessitating surgical bowel resection in 60% of patients due to stricturing. CD stricturing is the result of bowel wall thickening composed of a mixture of inflammatory and fibrotic intestinal wall changes. Over time, cycles of inflammation and healing shift the stricture pathology from largely inflammatory to primarily fibrotic. Predominantly fibrotic strictures are unresponsive to anti-inflammatory therapies and require endoscopic dilation or surgery. Decisions between medical and surgical therapy are plagued by the difficulty of determining whether bowel wall thickening is the result of inflammation or fibrosis. The current practice of empirically using anti-inflammatory therapy in most patients with CD-related bowel wall thickening and obstructive symptoms in the hope of success leads to many unnecessary side effects, infections, worse surgical outcomes, and long delays of appropriate surgical intervention when significant fibrostenotic disease is already present. Ultrasound stiffness imaging, using acoustic radiation force impulse imaging (ARFI), provides real-time non-invasive bedside elastography of bowel wall reported as shear wave velocity (SWV). We hypothesize that AFRI SWV of luminal stricturing disease will stratify medically responsive (inflammatory) from non-responsive (predominantly fibrotic) ileal Crohn’s disease; we present the interim analysis. Subjects with established ileal Crohn’s disease hospitalized for small bowel obstruction (defined as CT evidence of small bowel dilation >3.5cm) and scheduled to receive methylprednisone were enrolled. Within 48 hours of admission subjects underwent bedside ultrasound using Acuson S3000 with ARFI Virtual-Touch software package (Siemens, USA). SWV of the lateral, anterior, and medial portions of diseased ileum at 0% and 10% strain were collected in triplicate at baseline and Day 3. Subject demographic data, medical history, laboratory values and Harvey Bradshaw Index (HBI) were assessed at both time points. Ultrasound SWV values were compared to a primary outcome of surgical bowel resection for obstructive disease within 90 days. In 10 subjects with complete follow up to date, 4/10 have undergone surgical bowel resection within 90 days. Patient demographics, BMI, anti-TNF and immunomodulator use, and prednisone use prior to hospitalization did not significantly differ between surgical and non-surgical groups. Mean SWV at 0% strain (no freehand force) did not discriminate those requiring surgery within 90 days (1.42 [1.28–1.53] versus 1.56 [1.44–1.73] m/s, P = 0.154). When applying 10% freehand strain to the affected segment, baseline SWV yielded improved discrimination between those requiring surgery within 90 days (2.20 [1.74–2.39] m/s) versus subjects avoiding surgery (1.81 [1.54–2.09] m/s), although statistical significance was not reached at this interim point of the study, P = 0.066. No significant difference in SWV change between baseline and day 3 of methylprednisone was observed between the surgical and non-surgical cohorts. Ultrasound stiffness imaging by ARFI elastography demonstrates a trend of increasing shear wave velocities in subjects with obstructive ileal Crohn's requiring near-term surgical resection, although at this interim analysis point trends have not reached statistical significance. The data presented suggests that SWV non-invasive bowel wall stiffness measurements at higher strain values (20%-30%) may better predict the success or futility of medical therapy in subjects with obstructive Crohn's disease.

Isolated Fever as a Rare Presentation of Bevacizumab-induced Colonic Perforation

Purpose: Bowel perforation is a rare complication of the vascular endothelial growth factor inhibitor, bevacizumab (Avastin), in the treatment of metastatic colon cancer. We report a case of colonic perforation due to bevacizumab with subsequent abscess formation, presenting as isolated fever. Case: A 75-year-old man with recurrent colon adenocarcinoma after a left hemicolectomy with primary anastomosis eight months earlier for Dukes C colon adenocarcinoma presented to the emergency department with a one-day history of fever. He also had complaints of fatigue and diarrhea for a week. He denied abdominal pain, nausea, vomiting, and constipation. Three weeks prior, he had completed his first cycle of capecitabine, oxaliplatin, and bevacizumab for recurrent disease with metastasis. On examination, he was febrile, with a temperature of 39C, tachycardic to 101, but normotensive. He was otherwise comfortable and in no acute distress. Mild right lower abdominal tenderness was noted; no guarding or rebound tenderness. WBC 10,200/μL, otherwise laboratory studies were unremarkable. Abdominal radiograph revealed mild-to-moderate dilated small bowel with air-fluid levels; no evidence of pneumoperitoneum. He was initiated on broad-spectrum antibiotics. CT abdomen revealed a large, irregular air and fluid collection adjacent to the right psoas muscle, extending into the right iliac fossa with contrast extravasation. He underwent exploratory laparotomy, which revealed perforated ileocolic anastomosis with large posterior abdominal abscess with local tumour recurrence. Resection and end ileostomy was performed. Postoperatively, he did well, completed antibiotics, and was subsequently discharged home. Discussion: Our experience indicates that fever may be a sign of a serious complication of bevacizumab treatment. Perforated bowel resulting in abscess formation is associated with significant morbidity and mortality, and requires prompt medical and surgical attention. Clinicians must consider bowel perforation and abscess formation in the presence of fever in the setting of recent bevacizumab administration for metastatic colonic adenocarcinoma.

Small Bowel Volvulus Following Upper Gastrointestinal Barium Radiography

Introduction: Small bowel volvulus is extremely rare in adults. Barium has been associated with obstructive symptoms related to baroliths, but these take several days to weeks to form. Small bowel volvulus has never been reported immediately following a barium study. Case: The patient is a 49-year-old man who presented with persistent gastroesophageal reflux despite treatment with dexlansoprazole. An upper GI series was performed to evaluate for hiatal hernia. The study revealed mid-esophageal reflux with small hiatal hernia and one area of nonspecific proximal small bowel prominence. One hour after the study, he ate lunch, and fifteen minutes later developed nausea, vomiting and diffuse abdominal pain. The pain progressed and his vomiting persisted requiring hospital admission. His past medical history includes bipolar disorder, hypertension and obstructive sleep apnea. He had no prior abdominal surgeries. His medications include depakote, trileptal, seroquel, lisinopril and dexlansoprazole. Exam revealed hypoactive bowel sounds and tenderness in the epigastrium and bilateral upper quadrants. Laboratories were notable for only mild neutrophilia. Abdominal computed topography (CT) scan showed small bowel volvulus and closed loop obstruction without free air. Contrast from the prior day was present in dilated proximal small bowel loops. Nasogastric tube was placed. Repeat abdominal x-ray showed persistent obstruction without any contrast in the colon. Given lack of improvement, laparoscopy was pursued. It revealed torsion of the jejunal mesentery that was corrected. No ischemic changes were noted. His symptoms resolved and he was discharged home without any further issues. Five months later, CT enterography with gastrograffin showed no evidence of intraluminal lesion or mesenteric pathology. Discussion: Small bowel volvulus is generally described in neonates with embryologic malrotation of the midgut. The etiology in this patient is a mystery. Contributing factors may have been slowing of colonic transit as side effect of bipolar medications as evidenced by excessive stool on CT. A large bolus of barium in setting of mild ileus may have caused the jejunal volvulus.

Severe Enteropathy in a Patient on Valsartan

Purpose: This is a first case report of severe enteropathy masquerading as refractory celiac disease that resolved after suspension of valsartan. A recent case series described 22 patients with olmesartan associated enteropathy. Valsartan and olmesartan are angiotensin II receptor blockers with shared properties. A 71-year-old female presented with a five-year history of malabsorption. Symptoms began with sudden onset of nausea, vomiting and diarrhea. Watery and malodorous diarrhea, nighttime fecal incontinence, and abdominal pain persisted for five years, resulting in severe weight loss (27 kg). Celiac disease was diagnosed. Duodenal biopsies showed villous atrophy, intraepithelial lymphocytosis and crypt hyperplasia. Celiac serologies were negative. Symptoms did not improve despite strict adherence to a glutenfree diet. Her exam was significant for low BMI of 19 mg/m2. Diffuse muscle wasting, minimal adipose tissue, hyperactive bowel sounds and bilateral pitting edema to the knees. Labs demonstrated anemia of chronic inflammation, Vitamin A, carotene, and zinc deficiencies, hypoalbuminemia and hypomagnesemia. Transaminases were mildly elevated. Inflammatory markers were normal. Celiac serologies were negative at multiple time points. HLA genotype was DQ8. Duodenal aspirate contained >100,000 CFU of gram negative bacteria. CT enterography showed diffuse small bowel dilatation and mesenteric adenopathy; idiopathic biliary dilatation and edema in subcutaneous fat. Esophagogastroduodenoscopy demonstrated duodenal scalloping and prominence of submucosal vasculature. Capsule endoscopy showed villous atrophy affecting 75-90% of small intestinal mucosa. Duodenal biopsies had a malabsorption pattern with partial villous atrophy, crypt hyperplasia and inflammation (100 intraepithelial lymphocytes per 100 epithelial cells). The patient was treated with a 10-day course of tetracycline for small intestinal bacterial overgrowth, and although diarrhea improved slightly, most symptoms persisted. Repeat biopsies at 1 and 2.5 years of follow-up were unchanged. Cessation of valsartan was advised and within days, bloating, diarrhea and abdominal pain resolved. Gluten challenge for several months was followed by duodenal biopsies at 1-year following valsartan cessation. Villous architecture had normalized. Correction of anemia, transaminases and nutrient deficiencies was documented. While causality cannot be proven in this case, there is suspicion of a medication induced enteropathy secondary to the use of valsartan. This case represents a unique observation that other angiotensin II receptor blockers may be associated with a similar celiac-mimicking enteropathy as described previously with olmesartan.

An Unusual Pathology of Cecal Dilation

Purpose: A 64-year-old male with a history of rheumatoid arthritis and alcohol abuse was started on plaquenil for the treatment of rheumatoid arthritis as an outpatient. Seven days after treatment initiation, papular eruption developed on his chest and progressed to diffuse erythematous eruption with symptoms of burning. He presented to the emergency room with these complaints, and was noted to have tachycardia and a prominent leukocytosis of 32,000 per microliter. He was subsequently admitted to the ICU, and developed mental status changes attributed to alcohol withdrawal. On day one, patient was noted to have significant abdominal distention, and an abdominal X-ray was obtained, which showed an isolated cecal dilation of 15 cm, with the cecum rotated centrally toward the midline and no evidence of any small bowel dilation. Conservative measures failed to improve his continued dilation. Gastroenterology consult service was then consulted, and after discussion with radiology and general surgery, it was felt that there was low probability of cecal volvulus, and the decision was made to attempt a colonic decompression. Colonoscopy showed stool in the ascending colon, and there was difficulty in intubating the cecum. A follow up X-ray showed a continued cecal dilation of 12 cm. Six hours later, and on follow up imaging, it was noted to be 16 cm, and at this time the patient was taken to the operating room. Intra-operatively a cecal bascule was noted. Cecal bascule is a rare type of intestinal obstruction that occurs when a mobile cecum folds upward over the ascending colon and obstructs the ascending colon. It is a rare diagnosis, and it is estimated that 0.2% of all intestinal obstructions are cecal bascules. Over the last 20 years, only a handful of cases have been reported (less than 10). It is frequently confused with cecal volvulus on x-rays, and plain films will frequently demonstrate a massively dilated cecum with or without evidence of small bowel obstruction. Computed tomography scan is much more sensitive and can clearly define the large bowel anatomy. Cecal bascule is defined as type 3 cecal volvulus, and is a rare diagnosis that should be considered in all patients who present with findings of cecal dilation and if there is concern for volvulus.Figure

Isolated Intestinal Involvement of Angioedema Induced by ACE Inhibitor

Purpose: 58-year-old obese African-American woman with metabolic syndrome presents to our hospital with progressively worsening acute on chronic non-radiating periumbilical pain with abdominal distension, nausea and non-bloody emesis, which has progressed over the past six months. Physical exam was notable for an uncomfortable afebrile black woman, normotensive, with tachycardia and tachypnea. She had no peripheral rash or edema, no mucosal edema, and had clear lung fields on auscultation. Her abdomen had no surgical scars, was obese and distended with hypoactive and high pitched bowel sounds, and was diffusely tender with guarding and rebound tenderness. Rectal exam revealed brown guaiac negative stool in an empty rectal vault. Laboratory investigation revealed a normal complete blood count, comprehensive metabolic panel, amylase, lipase, and slightly elevated lactate at 4.2 mmol/L. Contrast enhanced CT abdomen was revealed contiguous distal jejunal to ileal small bowel enhancement and thickening with associated small bowel dilation of the affected areas without a transition point of obstruction. In light of her presentation and risk factors for vascular disease, a CT angiogram was performed 6 hours after her initial CT abdomen to evaluate for mesenteric ischemia. Remarkably, this exam revealed complete resolution of bowel wall enhancement, thickening, and dilation, along with patent mesenteric arteries. During this time, patient's clinical symptoms also completely resolved. Upon reviewing patient's history, patient started having symptoms after initiation of lisinopril approximately 9 months prior to presentation. C1-esterase, C1q, and C3 complement levels were subsequently found to be normal. Our suspicion for ACE inhibitor induced intestinal angioedema was confirmed after patient remained asymptomatic with discontinuation of lisinopril. ACE inhibitor induced angioedema typically involves the extremities, face, and upper airways. It is caused by quantitative or qualitative deficiency of C1-esterase protein, which inhibits vasodilators such as bradykinin. Concomitant gastrointestinal involvement is common; however, it is rarely the sole manifestation of this disease. This disease carries a high mortality if untreated and may even lead to unnecessary surgical exploration if misdiagnosed. As such, clinical awareness of this entity as a cause of acute abdomen is paramount in providing the best care for these patients. Conclusion: Isolated intestinal involvement of ACE-inhibitor induced angioedema can also occur without typical features of facial or extremity swelling. Knowledge of such manifestation can improve patient outcomes.

Severe Iron Deficiency: Rare Etiology, Easy Treatment

A 23-year-old male with an over 10-year history of chronic iron-deficiency anemia and gastroschisis at birth was initially evaluated at an outpatient Gastroenterology clinic with complaints of generalized malaise and fatigue lasting for several days. Although he denied abdominal pain, he had experienced intermittent bloating and epigastric discomfort for several years. He also reported chronic diarrhea, which he described as foul smelling, loose stools that occurred three to six times per day for several years. He denied fevers, chills, nausea, vomiting, hematemesis, or rectal bleeding. Additionally, he denied dyspnea on exertion or chest discomfort. On examination, tachycardia and normotension were noted. A hemoglobin of 4.4 g/dL prompted admission for further evaluation. His past medical history included asthma. The patient had been hospitalized for 3 months after birth (at 36 weeks gestation), largely due to an inability to tolerate oral intake, having received total parenteral nutrition prior to being discharged tolerating oral feeds. Charts from that time noted that he ‘‘...had gastroschisis with intestine, spleen and stomach outside of the abdomen.’’ A few days after birth, he underwent non-resective surgical repair. Intraoperatively, there was no evidence of intestinal atresia. At the age of five, he had one episode of a small bowel obstruction that resolved with medical management. An upper gastrointestinal series (UGIS) with small bowel follow through (SBFT) at that time showed slightly dilated small bowel segments with normal transit time throughout. In 1994, at the age of six, his hemoglobin and mean corpuscular volume (MCV) were within normal limits. Nevertheless, even in 2001, the patient had a history of heme-positive stool, along with bloating, diarrhea, and anemia (hemoglobin 9.1 g/dL and MCV 60). His anemia was evaluated at that time with panendoscopy without abnormal findings. The duodenum was reported as being ‘‘patulous.’’ Biopsies revealed mild chronic active gastritis (antral biopsies were negative for Helicobacter pylori), mild reflux esophagitis, and a normal duodenum. Biopsies throughout the right and left colon were normal. Overall, his endoscopic evaluation did not reveal any abnormalities that explained his anemia. He was prescribed oral iron replacement therapy, which, however, was not tolerated and thus not taken regularly. Given his history of asthma and poor growth as a child, he was evaluated for cystic fibrosis (CF) with sweat chloride and genetic testing which were normal. In 2001 another UGIS with SBFT showed a somewhat dilated distal jejunum. Pancreatic enzyme supplements were prescribed for unclear reasons, which, however, initially improved his bloating and diarrhea. By 2004, the diarrhea recurred with non-bloody, loose stools reported two to three times a week. There was no clear explanation for the patient’s diarrhea, anemia, or hemepositive stools; he was lost to follow-up until being seen at our institution. The patient’s social history was unremarkable in that he denied any drug, alcohol, or tobacco use. He also denied foreign travel or unusual environmental exposures. He did not have any pertinent family history; his three siblings S. R. Sinha (&) G. Triadafilopoulos N. Shah Division of Gastroenterology and Hepatology, Department of Medicine, Stanford University Medical Center, 300 Pasteur Dr., Always Bldg. M211, Stanford, CA 94305-5187, USA e-mail: sidsinha@stanford.edu

Spiral intestinal lengthening and tailoring — first in vivo study

IntroductionSpiral Intestinal Lengthening and Tailoring (SILT) offers a new opportunity for the surgical treatment of short bowel syndrome. SILT requires less manipulation on the mesentery than the Bianchi procedure and does not alter the orientation of the muscle fibers like serial transverse enteroplasty (STEP). This study reports the first SILT results in a surviving animal model. Material and MethodsVietnamese minipigs (n=6) underwent interposition of a reversed intestinal segment to produce proximal small bowel dilation. Five weeks later the reversed segment was resected, and the wall of the dilated intestine was cut spirally at 45°–60° to its longitudinal axis. The bowel was lengthened longitudinally, and the spiral shaped intestinal wound was sutured. Five weeks later, the animals were explored, and the lengthened segments were measured. Haematoxylin and eosin, picrosirius, neuron specific enolase, S-100, C-kit, and immunohistochemistry were performed. ResultsMean lengthening was 74.8%±29.5% and mean tailoring (lumen reduction) was 56.25%±18.8%. No instances of necrosis, perforation, suture break down, or peritonitis were observed in 6/6 animals. Four of six animals recovered uneventfully with viable lengthened segments. Statistical analysis showed no significant difference in length (p=0,078) and width (p=0,182) after 5weeks. Two animals developed bowel obstruction due to narrowed lumen, adhesion, and strangulation after 14 and 24days of surgery. In both animals the lumen was tailored by more than 70% to less than 1.5cm diameter. The mucosa and the muscle layers in the operated segment had become hypertrophic, but the orientation of the circular and longitudinal muscle fibres remained normal after the SILT procedure. There were no signs of chronic ischemia or collagen accumulation after the SILT. The myenteric and submucosal plexuses and the Cajal cell network appeared normal. ConclusionThe bowel remained viable macroscopically and microscopically after SILT, such that SILT may be an alternative or an addition to the present technical repertoire of intestinal lengthening. However the limitations of tailoring should be kept in mind.

Intestinal pseudo-obstruction due to small bowel α-actin deficiency in a child with Ehlers–Danlos syndrome

Chronic intestinal pseudo-obstruction (CIPO) is a syndrome characterized by signs and symptoms of intestinal obstruction without a mechanical cause. In children, two main subtypes, the neuropathic and myopathic forms, are found [1]. A variety of pathological findings of the smooth muscle can result in clinical syndromes involving a motility disorder of the small or large intestine. Moreover, abnormalities of enteric innervation or intestinal muscle defects may be diffuse or involve limited tracts [1]. Deficiency of one of the isoforms of the cytoskeletal smooth muscle protein actin, aactin, involving the circular muscle of the jejunum, is known to induce CIPO both in adults and in children [2]. We report a unique case of a-actin deficiency associated with Ehlers– Danlos syndrome. A 14-year-old girl was urgently admitted because of shock with abdominal distension and severe dehydration. On plain abdominal X-rays, severe small and large bowel dilatation was noted. The patient’s history was negative for any previous medical or surgical condition. She had mandibular prognathism causing aerophagia and affecting speech and mastication. An urgent exploratory laparoscopy was carried out; dilatation of the ascending colon and terminal ileum were found. No perforation or necrosis was detected on inspection of the whole intestine. Two weeks later, persistent bowel dilatation necessitated ileostomy. The patient developed intestinal obstruction following ileostomy closure. Subsequently, several surgical intestinal deviation procedures were performed and after every attempt at reversal immediate reoperation was required. Ten months later, a definitive end ileostomy had to be fashioned 10 months later. Full-thickness biopsies of the ileum and colon were obtained. Histological examination with conventional (hematoxylin and eosin, H&E) staining appeared basically normal (Fig. 1a). Immunohistochemical assessment (NSE, S100, CD117) showed normal features of the enteric nervous system in both the small intestine and the colon, whereas immunohistochemistry for a-actin revealed decreased expression in the circular layer of the small bowel (Fig. 1b– d) and normal features in the colon. Skin biopsy performed after abdominal surgery, revealed hallmarks of disturbed fibrillogenesis and led to the diagnosis of Ehlers–Danlos syndrome, classical type (old type I) [3]. Abnormal intestinal findings often leading to intestinal perforation, especially in the colon, are observed in Ehlers– Danlos syndrome, although almost exclusively in patients with subtype IV [4]. As far as we know, no previous association has been described between Ehlers–Danlos syndrome and a-actin deficiency. The latter was probably the cause of CIPO in our patient (since the enteric nervous system appeared normal), causing impaired propulsion in the small bowel and pseudo-obstructive symptoms. The diagnosis of CIPO, in both adults and children, is often delayed, due a low index of suspicion and the fact G. Pelizzo ! G. Nakib ! V. Calcaterra Pediatric Surgery Unit, Department of Mother and Child Health, IRCSS Polyclinic S. Matteo Foundation, University of Pavia, Pavia, Italy

Intestinal obstruction due to congenital mesenteric band in an adult patient

We report the case of a 30-year-old male patient who was admitted to the Emergency Department with symptoms and signs of intestinal obstruction. There was no history of abdominal surgery or trauma. Abdomen was distended. Abdominal plain X-ray showed intestinal loops with air-fluid levels in the middle area of the abdomen. Computed tomography showed small bowel dilatation and free fluid in the abdomen. A laparotomy was subsequently performed and the intraoperative findings revealed a band extending from the root of the mesentery to the antimesenteric wall of the jejunum. The band was resected; the postoperative course was uneventful. Congenital bands are extremely rare and usually observed in childhood. This case, therefore, represents an unusual surgical problem in an older individual in whom the diagnosis was clinically unexpected.

Transmesosigmoid hernia with small bowel strangulation

Introduction: Internal herniation of the small bowel is difficult to diagnose before surgery. Delayed laparotomy is related to high morbidity and mortality. We present a rare case of a transmesosigmoid hernia to demonstrate the importance of high suspicion and early laparotomy. Case Report: A 44­year­old female patient had severe abdominal pain of acute onset. A CT scan at six hours after onset of the symptom showed a dilated small bowel loop of diminished attenuation. Her severe pain was not controlled even with narcotics. An emergency laparotomy eight hours after onset revealed about a 150 cm strangulated closed loop of the mid small intestine, herniated through a small mesenteric defect of the sigmoid colon. The defect in the mesosigmoid was about three cm in diameter without a sac, and functioned similarly to a napkin ring; therefore the reduction of the herniated closed intestine loop was difficult without division of the intestine. The patient recovered well after resection anastomosis and closure of the mesenteric defect. Conclusion: Congenital internal hernia is rare, but early development of incarceration and strangulation is associated with increased morbidity and mortality. Early