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Primary enteroliths associated with Crohn's disease have been considered to be rare and are most likely caused by severe ileal stenosis. Herein, we report the case of a primary enterolith possibly caused by mild jejunal stenosis in a Crohn's disease patient who received oral administration of ursodeoxycholic acid (UDCA). A 62-year-old woman with a 6-year history of Crohn's disease, currently in clinical remission, was on UDCA prescription for liver dysfunction. Magnetic resonance imaging and double-balloon endoscopy, which were performed to examine epigastric pain, revealed mild jejunal stenosis and an enterolith on the oral side. Since it was difficult to remove or crush the enterolith endoscopically, we decided to remove it surgically with the stenotic jejunum. Component analysis revealed that more than 98% of the enterolith was composed of UDCA; subsequently, oral administration of UDCA was discontinued. This case demonstrated that primary enterolith might develop in Crohn's disease patients with mild intestinal stenosis, and oral administration of UDCA can trigger an enterolith in such patients. Therefore, routine follow-up imaging is necessary for early detection. Oral UDCA should be administered with caution for Crohn's disease patients with stenosis of the proximal small intestine.

Objective To retrospectively analyze the imaging findings of fetal intestinal obstruction diagnosed by MRI and compare with postnatal surgery findings. Methods MRI data of 3346 pregnant women were retrospectively analyzed; we found 47 cases of suspected fetal small intestinal obstruction. Twenty-nine underwent postnatal surgery. Results We indentified one case of jejunal obstruction secondary to perforation, five annular pancreas, ten duodenal stenoses, four jejunal stenoses, five jejunal atresias, two ileal atresias, four intestinal volvulus, and four intestinal malrotations, We further found four cases of duodenal stenosis with intestinal malrotation (two cases also showed volvulus). On fetal MRI, annular pancreas and duodenal obstruction manifested as a "double bubble". Jejunal stenosis appeared as a "triple bubble". Jejunal and ileal atresia mainly manifested as proximal dilatation with high signal on T1WI. Intestinal volvulus showed a sausage-like intestinal distortion and mixed signals on T1WI and DWI sequences. Intestinal malrotations were characterized as abnormal duodenal morphology. We missed two malrotations, one jejunal obstruction was misdiagnosed. Conclusion Prenatal MRI can accurately determine the degree of intestinal obstruction and help qualitatively diagnose its possible etiology according to changes in MRI signals in multiple sequences, providing guidance for prenatal counseling.

International audience; Snyder-Robinson syndrome (OMIM #309583) is a rare X-linked condition, caused by mutation in the SMS gene (MIM *300105), characterized by a wide spectrum of clinical signs including developmental delay, epilepsy, asthenic habitus, dysmorphism, osteopenia, and renal or genital anomalies. Here we describe two maternal half-brothers who both presented with severe neurodevelopmental delay, seizures, hearing loss, facial dysmorphism, renal and ophthalmologic anomalies, failure to thrive and premature death. A novel p.(Gly203Asp) variant was found at the hemizygous state in the two boys, and an elevated Spermidine/Spermine ratio confirmed the diagnosis of Snyder-Robinson syndrome. One of the brothers presented with gastrointestinal symptoms, with jejunal stenosis, enteral feeding intolerance, failure to thrive due to a dysfunctional gastrointestinal system, cholestasis and exocrine pancreatic insufficiency. Although more studies will be needed to understand its mechanisms, this observation lends further support to the possibility of severe digestive involvement in Snyder Robinson syndrome.

Intestinal tuberculosis and Crohn's disease are a diagnostic challenge because of the clinical, radiological and endoscopic similarity. The histological and microbiological findings are positive in less than 50%, which delays the correct treatment, putting the patient at risk. We reported a 34-year-old immunocompetent patient with 4 years of malabsorptive diarrhea, weight loss, nocturnal diaphoresis, abdominal pain and an ulcer with stenosis in the jejunum was found; she mpirical antituberculosis treatment with clinical improvement. Later the culture was positive for M. tuberculosis.

This is a case report of a neonate who was antenatally diagnosed with jejunal atresia which turned out to be duodenal atresia with apple peel syndrome. A previous sibling, who also had apple peel but with jejunal atresia, succumbed to sepsis after surgery. The first sibling had jejunal stenosis and had died of sepsis following surgery. Combination of duodenal atresia with apple peel is extremely rare. This coupled with a familial condition is rarer still. This case was challenging due to the short length of the gut and prolonged need for total parenteral nutrition and sepsis in postoperative period.

Post-traumatic small-bowel obstructions are rare and late complications following blunt abdominal trauma. Timely diagnosis is frequently impeded because of nonspecific associated symptoms, and furthermore, the degree of urgency is frequently underestimated due to the accidental cause. During the last 6 years (2005-2011), we have observed only 2 cases (4 and 10 years old) with post-traumatic small-bowel obstruction. On admission the patients had a contusion on the abdominal wall, duodenal hematoma and a Chance fracture of the lumbar spine on the computed tomography (CT) scan. Although early clinical improvement was noted, progression of the disorder was observed for the following 15 days like an occlusive syndrome (abdominal distension, nausea, emesis). Abdominal ultrasound and computed tomography scan revealed small-bowel obstruction. At laparotomy, jejunal stenosis was found associated with mesenteric tears, which was resected with end-to-end anastomosis. Six to 8 months of follow-up ascertained the therapeutic efficacy of the procedure. Post-traumatic small-bowel obstructions are rare; the clinical presentation is unspecific and appears with a time lag following the trauma. Echography and computed tomography scan can establish both the diagnosis and therapeutic choice.

Gastric perforation (GP) of the newborn is a rare, serious, and life-threatening problem, and its etiology remains unclear. Although historically GP has often been described as “spontaneous’’, some cases are non-spontaneous. The aim of the present study was to review cases of GP and to discuss its etiology in a single prefecture in Japan over a period of 20 years. Eleven cases with GP that underwent surgery in 4 institutions in the Chiba Prefecture from 1991 to 2010 were reviewed and divided into 2 groups: the early (1991–2000, n = 7) and late (2001–2010, n = 4) groups. No factors were observed that could have caused GP other than malformations associated with distal obstruction (3 midgut volvulus, 1 jejunal stenosis, 1 diaphragm eventration). Distal obstruction was present in 1 case in the early group and all 4 cases in the late group (p = 0.015). While the incidence of GP did not change over the 20-year period reviewed, the incidence of GP without distal obstruction significantly decreased in the late group. The proportion of patients with GP and distal obstruction increased and true “spontaneous” cases of GP decreased over time. The possible presence of distal obstruction should be evaluated during surgery for GP.

We present a case of sonographically detected transient gas in the portal vein in a 4.5-month-old infant who had a history of two consecutive jejunectomies due to jejunal stenoses and was admitted to our hospital with clinical and laboratory findings consistent with a subacute small bowel obstruction and dehydration. Sonography excluded other pathologies and the patient was treated conservatively with success. The presence of gas in the portal vein could be a sign of an underlying mechanical obstacle, as another episode of small bowel obstruction 1 month later required surgical treatment of adhesive intestinal obstruction. © 2011 Wiley Periodicals, Inc. J Clin Ultrasound 2012

Jejunoileal atresia is a congenital anomaly that is characterized clinically by bilious vomiting and abdominal dis-tension. It has been associated with various other anomalies but its association with mesenteric cyst is rare. We are presenting a patient with antenatally diagnosed duplication cyst with multiple jejunoileal atresia and stenosis with possible etiology behind such an association.

Introduction Congenital jejunal stenosis and gastric duplication cysts are very rare congenital anomalies of the gastrointestinal tract in the newborn. We present a case of congenital membranous jejunal stenosis associated with gastric duplication cysts, which was diagnosed by ultrasonography. To the best of our knowledge, this is the first report of ultrasonographic diagnosis of congenital membranous jejunal stenosis associated with a gastric duplication cyst in a newborn. Case presentation A 1-month-old Chinese baby girl presented with projectile vomiting and hyperpyrexia for 3 days. An upper gastrointestinal contrast study showed incomplete duodenal obstruction; however, ultrasonography revealed congenital membranous jejunal stenosis associated with a gastric duplication cyst. After surgical excision of the jejunal membrane and gastric duplication cyst, she recovered well with no bilious vomiting at the 1-year follow-up. Conclusion Ultrasonography is a useful tool for the evaluation of membranous jejunal stenosis and the identification of small, asymptomatic gastrointestinal duplication cysts.

A major complication of portal and mesenteric vein thrombosis is acute bowel ischaemia resulting in infarction and requiring immediate resection of the involved segment. Sufficient collaterals can prevent acute haemorrhagic infarction, but bowel stenosis due to chronic ischaemia may develop. We report two cases of ischaemic jejunal stenosis occurring 4 weeks after successful treatment of portal and mesenteric vein thrombosis. Diagnosis of high-grade segmental stenosis of the jejunum was established by contrast medium radiography of the gastrointestinal tract. After laparotomy and resection of the stenosed jejunal segment, both patients recovered well from the operation and were released from hospital. Follow-up examinations revealed an unremarkable state of health. Ischaemic bowel stenosis should be considered in patients with recurring abdominal pain after mesenteric and portal vein thrombosis. A close follow-up of every patient after treatment for mesenteric and portal vein thrombosis should be carried out to ensure early diagnosis of this complication.

This report describes a case of symptomatic ectopic pancreas in the jejunum. A review of the literature revealed no other case of ectopic pancreas manifesting as jejunal stenosis during the neonatal period. Ectopic pancreas should be excised in consideration of the potential late complications.

Ischemic stenosis of the jejunum is rare. For technical, anatomical, and pathological reasons, ischemic stenosis of the jejunal segment used for the replacement of the esophagus or the stomach, or both, represents a special entity. The present study reports a case of balloon catheter dilation of ischemic strictures of the jejunal segment, used for substitution after gastrectomy. In this patient, an occlusion of the blood vessels supplying the affected segment was observed at its aortic origin, and a Wallstent was implanted. A rare late complication, aortoesophageal fistula, appeared one year after placement of the Wallstent. The case presented in this study suggests that using balloon catheters and implanting a Wallstent may be a useful approach to the management of postoperative ischemic strictures of the jejunum in selected cases. The minimally invasive technique with special indications used here has not previously been described. The rare complication mentioned, however, requires special attention.

We report a case of congenital jejunal stenosis in a 10-month-old boy. Upper gastrointestinal contrast study suggested congenital jejunal stenosis. Diagnostic laparoscopy was performed. A dilated jejunum starting from the ligament of Treitz was revealed and caliber change was identified. We made a small laparotomy at the left upper quadrant to pull out the caliber change. The proximal jejunum with the caliber change was pulled out through the tiny incision and resected, and end-to-end anastomosis was performed. We conclude that congenital jejunal stenosis could be identified even in a neonate, and that laparoscopic-assisted surgery for jejunal stenosis in an infant is an attractive alternative to the conventional open method.

Background recent advances in endoscopy have enabled us to explore the small intestine more efficiently, both with capsule endoscopy and double-balloon enteroscopy. However, these methods are not available in all hospitals. Therefore, when capsule endoscopy reveals proximal jejunal lesions, a possible alternative for treatment could involve push enteroscopy combined with colonoscopy. Lesions can thus be marked for subsequent monitoring. Objectives to describe the efficacy of pediatric colonoscopy for diagnosis, the marking of the area explored, and therapeutic options. Material and methods between October 2007 and September 2008 a total of 21 enteroscopies were performed using a pediatric colonoscope. Nine of these were used to take jejunal biopsy samples due to suspected disease of the mucosa. In 10 of the remaining 12, capsule endoscopy revealed lesions (vascular malformations or jejunal bleeding), and in 2 intestinal transit time was analyzed due to suspected jejunal stenosis. We used a PENTAX EC-3470-LK pediatric colonoscope, whose 11.6-mm sectional diameter and 3.8-mm working channel make it possible to administer all the usual endoscopic treatments. Results therapeutic endoscopy was performed on 7 men and 5 women (mean age 63.3 years). Jejunal lesions were observed in 10 cases (5 cases of angiodysplasia, 2 cases of jejunal stenosis, 1 case of nonmalignant thickened jejunal folds, 1 eroded submucosal tumor, and 1 case of duodenal and jejunal varices). The most distal area was marked with India ink (2), hemoclips (4), or both to help locate the lesions using simple abdominal radiography or capsule endoscopy. Conclusions jejunal enteroscopy enabled a firm diagnosis to be made in most of the patients studied. We were able to treat 58% of patients and mark the areas explored for subsequent follow-up. With hemoclips we were able to locate the most distal point explored using simple abdominal radiography.

We report herein a case of an 82 year old Japanese woman admitted to hospital after suddenly experiencing upper abdominal pain and vomiting. An upper gastrointestinal series showed a narrow segment with edematous adjacent loops in the proximal jejunum. On the twentieth day following her admission, a barium-meal radiograph revealed that the narrow segment had progressed to a firm tubular stenosis with effacement of the mucosal pattern. The stenotic segment, resected the following day, was 7 cm long with an internal circumference of only 0.5 cm at its narrowest portion. Histologic examination of the resected specimen showed ulceration and marked edema in the intestinal wall, accompanied by organizing and recanalizing thrombi in the subserosal arteries. These features were strongly suggestive of an ischemic process.

In cases where the papilla of Vater is unreachable because of pyloric/duodenal stenosis, or a catheter cannot be introduced into the papilla, or with recurrent tumor growth, or after previous gastrointestinal surgery, percutaneous transhepatic cholangiodrainage (PTCD) is considered to be the therapeutic alternative in cholestasis. The purpose of this report was to demonstrate that endoscopic ultrasound (EUS)-guided transesophageal cholangiodrainage is a feasible alternative in patients who decline to undergo PTCD. A 67-year-old female patient with recurrent tumor growth at the hepaticojejunostomy 17 months after a formerly resected cholangiocarcinoma (pT3, pN0 (0/2), M0, G2, R0; extended right hemihepatectomy), cholangitis, and peritoneal carcinomatosis underwent an EUS-guided transesophageal procedure to obtain cholangiodrainage by (i) puncture of a branch of the biliary tree at the left hepatic site, (ii) insertion of a guide wire into the bile duct and the anastomosed jejunum using the rendezvous technique with endoscopic retrograde cholangiopancreatography (ERCP)/conventional endoscopy, (iii) transesophagohepatic placement of an 8.5-Fr. double pigtail catheter, and (iv) transhepatic placement of a Wallstent through the jejunal stenosis, resulting in complete alleviation of the biliary and jejunal obstruction. There were no severe complications such as perforation or bleeding and no stent occlusion within the patient's lifetime of more than 3 months. Death was related to progressive tumor growth. EUS-guided transesophageal cholangiodrainage, here described in combination with Wallstent placement, is a reasonable, feasible, and encouraging treatment alternative in selected patients where conventional ERCP or PTCD is not an option.

Aim: to evaluate the utility of double-balloon enteroscopy for small-bowel disease. Design: a prospective study of 50 consecutive enteroscopies performed from December 2004 to July 2005 to analyze diag- noses and treatments. Patients: 44 patients (33 had undergone a previous capsule endoscopy) with indications for obscure digestive hemorrhage, an- giodysplasia, Peutz-Jeghers syndrome, ulcer, suspected Crohns disease, tumors, and refractory celiac disease. Results: we carried out enteroscopy studies in 44 patients by the oral route and, in 6 additional patients, by both the oral and anal routes. We reached the ileon with the oral route in all cases but one (jejunal stenosis), and in 4 cases out of 7 with the anal route, with an average duration of 73 minutes. We found an- giodysplasia in 19 cases, as well as NSAID-related enteropathy, Crohn's disease, diverticulosis, and Waldenstrom's disease. We performed biopsies in 31% of cases with diagnoses of adenocarci- noma, lymphangiectasia secondary to tumor in celiac disease, and Whipple's disease. We treated 19 patients with angiodysplasia (1 to 20 synchronous lesions) with argon, and 4 patients with polyps using polipectomy (sporadic polyps or Peutz-Jeghers syndrome). A retained capsule in one patient with stenosis was removed. Conclusions: double-balloon enteroscopy is a useful and ef- fective technique in the diagnosis and treatment of small intestine diseases, thus complementing capsule endoscopy. More studies are needed to analyze its impact on the management of this con- dition.

Wireless capsule enteroscopy, being a novel, painless investigative technique, is reported to be significantly superior to push enteroscopy in its ability to find bleeding abnormalities in the small intestine. Here we report a case of acute jejunal obstruction following wireless capsule endoscopy. The patient had a 1-month history of gastrointestinal bleeding of unknown source. Further evaluation including gastroscopy and colonoscopy, angiography and computed tomography (angio-CT), and radio-labeled erythrocytes scan failed to reveal a source of bleeding. Therefore, wireless capsule enteroscopy was performed. Before capsule endoscopy, there was no clinical or imaging evidence of strictures or stenosis. At readmission it could be shown that there were two inflamed strictures of the small intestine. The capsule was detected at a stricture of the small intestine detected by abdominal ultrasonography and conventional computed tomography. The patient underwent a medical treatment with steroidal and other anti-inflammatory drugs for a total of 23 days and was discharged without complaints. Acute laparotomy after readmission with jejunal ileus proofed the capsule occluding two highly inflamed jejunal stenosis caused by Crohn disease. The present case demonstrates the potential for complications when wireless capsule enteroscopy is performed in the presence of intestinal strictures. Any history of inflammatory bowel disease, abdominal irradiation, cancer, obstruction, and abdominal surgery must be elicited in detail and may exclude the use of wireless capsule enteroscopy.

Background and Aim: Stricturing is the most common complicated phenotype in children with Crohn’s disease (CD), but only few studies have described its course and there are no data on the efficacy of medical treatment. The purpose of this study was to retrospectively describe in pediatric stricturing CD the course and assess clinical and radiological response to medical therapy. Patients and Methods: 36 patients (pts) with stricturing CD (64% males, age range: 7.3–20.2 years, median 14.7), were identified by our department database. Records were reviewed for disease duration before detecting stenosis, location of strictures, type of medical treatment received, number of disease recurrences and hospitalizations. Pediatric Crohn’s disease Activity Index (PCDAI), need to change medical treatment or surgery, magnetic resonance imaging or small intestine contrast ultrasonography were used as outcomes and evaluated at 6, 12, 18 and 24 months after diagnosis of stenosis. Results: Strictures were ileal in 61% of pts, ileocolonic in 28% and colonic in 11%; 6 pts (17%) also had proximal jejunal stenosis. Thirteen pts (36%) had a stricturing disease at the time of CD diagnosis, while 64% developed it at the follow-up (2.48±4.12 years after CD diagnosis). Cumulative risk for developing stenosis was 22%, 27% and 28% at 12, 18 and 24 months, respectively. At baseline, 89% of pts underwent medical treatment, while 11% had surgical resection: in a multivariate analysis, only ileal stenosis and severe abdominal pain significantly differed between the two groups (p: 0.05 and p: 0.006, respectively). At 6, 12, 18, and 24 months, 53%, 50%, 42%, and 35% had a complete response to medical treatment, respectively; whereas 34%, 43%, 40%, and 34% had a partial response, defined as a radiological evidence of stenosis requiring a change of their medical therapy. Overall, 44% were unresponsive to medical therapy and required surgery during 24 months followup; responders and non-responders did not statistically differ for clinical variables such as duration of disease, location of stenosis, mean PCDAI at the beginning of the therapy and type of medical treatment. Conclusions: A stricturing phenotype is not uncommon at the diagnosis of CD in children. Medical therapy seems to be poorly effective in avoiding intestinal resection and common clinical variables are not of value in discriminating between responder and non responders to medical therapy. Prospective studies are needed to define the optimal management strategy of stricturing CD and to identify predictive factors of medical treatment failure.

tertiary IBD center were reviewed. Inclusion criteria: (1) Small bowel CD assessed by conventional techniques; (2) Detailed clinical history; (3) No stenosis/obstructions; (4) Small bowelCD assessed by entero-CT/-MRI, small intestinal contrast ultrasonography, barium follow through and/or ileocolonoscopy (IC) within 6mths from SBCE; (5) SBCE images reviewed by one single gastroenterologist. SBCE performed using the Given Pillcam SB (Given, Israel) after 2L PEG. Findings considered: (a) CD extent before vs after SBCE; (b) upper GI lesions (i.e. above the distal ileum proximal to the valve or to the anastomosis); (c) SBCE transit times; (d) impact rate. Results: A total of 40 CD pts (20M, median age 34 yrs, range 18 70) fulfilled the inclusion criteria. Previous ileo-colonic resection was shown by 29/40 (72.5%) pts (median interval 10mths, range 3 300). CD localization involved the ileum in 37/40 (92%) CD (recurrence in 26/37) and also the upper GI in 4/37 (10%), while 3 pts showed no recurrence. SBCE showed marked interindividual variations in terms of gastric (median 29min, range 3 360) and small bowel transit times (valve: median 350min, range 240 433 or anastomosis in resected CD: median 231 min, range 61 466). SBCE did not visualize the entire small bowel in 9/40 (22.5%) pts (5/11 CD not resected, 4/29 resected pts). Upper GI lesions were detected by SBCE in 23/40 (57.5%) pts, known before SBCE in only 4/40 (10%) pts. In one pt, SBCE visualized an ulcerated jejunal stenosis requiring surgery not detected by SBFT and IC. In 1/40 (2.5%) pts, SBCE impact requiring surgery within a “cul de sac” of an ileo-ileal anastomosis was observed. Conclusions: Upper GI lesions may be detected by SBCE in a high proportion of CD pts, although their clinical relevance needs to be determined. Before SBCE, CD pts need a careful selection due to possible incomplete small bowel visualization and to the impact risk occurring also in the absence of stenosis.

Reports on zonal aganglionosis of the bowel are very rare and the few cases reported concern colon and rectum. We present a case of segmental aganglionosis of the jejunum in a 2-months-old boy. He had to be operated upon twice because of an ileus of the short bowel. At the second operation, a 10 cm long narrow segment of the jejunum was resected. The histological examination revealed a segmental aganglionosis of the plexus myentericus. Possible causes of this rare zonal aganglionosis could be: 1. Local damage of the plexus myentericus through anoxia. 2. Primary abnormity in the gastrointestinal anlage. 3. An intrauterine inflammation. 4. A double migration and development gradient of the precursors of the neuroblasts, from each end to the middle of the gut. A defect in the microenvironment of the enteric neurons would result in their failure in differentiation. This possible explanation would challenge the accepted theory of pathogenesis of colonic aganglionosis.

INCREASING awareness of the varied clinical manifestations of chronic ischemia of the gastrointestinal tract1 , 2 prompts this report of an unusual case of segmental jejunal stenosis produced by th...

A small-for-gestational age white female infant was noted to have multiple minor anomalies and severe jejunal stenosis. Mild peripheral pulmonic stenosis, skeletal anomalies, and cholestasis with paucity of intrahepatic bile ducts were observed, and she was diagnosed as having arteriohepatic dysplasia. Chromosome analysis of peripheral blood leukocytes showed a 46,XX,del(20)(p11.2) chromosome constitution.

Sir .—Delayed complications of small-bowel injuries after automobile accidents are difficult to diagnose since the clinical and roentgenographic findings are often inconclusive. We report a late complication, jejunal stenosis. To our knowledge, only one similar case has been reported. 1 Patient Report .—A 15-year-old girl presented with severe abdominal and back pain after an automobile accident several hours earlier. She was a rear-seat passenger and was wearing a lap-type seat belt. The patient was alert, but a severe drop in her hematocrit value to 0.23, a systolic pressure of 80 mm Hg, and a pulse rate of 120 beats per minute were noted. A large hematoma over the lumbosacral area and a "seat belt" shaped bruise over the lower abdomen were present. There was severe abdominal tenderness to palpation, but the abdomen was not distended, with normal bowel sounds. The patient received several units of blood and fluid replacement. Following

A prospective study was undertaken to evaluate the significance of isolated bilious vomiting in the first 72 hours after birth. Over a 4-year period, 45 infants (2/1000 live birth)(mean ±S.D. B.W. 3.2±0.4 kg; G.A. 39.7±1.2 wks) were admitted to NICU because of bilious vomiting without other obvious signs of GI obstruction. The extent of work-up followed a protocol which included serum electrolytes, Ca, plasma glucose, CBC and plain abd. X-ray in all cases, upper or lower GI series and septic workup if indicated. Of the 45 infants, 9 (20%) required surgical intervention (5 malrotation, 1 jejunal atresia, 1 jejunal stenosis, 1 jejunal duplication cyst, 1 infantile myofibromatosis) and 36 (80%) associated with non-surgical conditions (31 idiopathic, 3 meconium plug; 2 left microcolon). There was no sign. diff. between the surg. and non-surg. cases at time of onset of bilious vomiting and onset of meconium passage. All infants with non-surg. condition resumed feeding by 1 week. Surgical cases had sign, higher (p