Midgut Malrotation Research Articles

Sub–Hepatic Caecum

Malrotation of gut is an intestinal gestational disorder which not only affects the positioning of the midgut, but also its vascular supply. It usually presents with the syndrome of bouts of colic and vomiting with little distention of abdomen and diarrhea. Though midgut malrotation is a common cause of intestinal obstruction in newborn, scant attention is given to this developmental anomaly as a cause of symptoms in adults. The development of midgut portion of alimentary canal suffers a large variety of variations. The surgical importance of abdominal situation of the portions of intestine derived from the midgut loop requires little attention. Sub-hepatic anatomical location of the appendix makes it more difficult to diagnose acute appendicitis at any age, including in older adults. Failure to recognize the nature and characteristic features of these misplacements may lead to grave errors in procedure, or to injurious prolongation of the operation. Here in we found a rare abnormality of caecum, which was present in the sub hepatic region with the absence of ascending colon during routine dissection classes for undergraduate students.

Rare small bowel obstruction: Right paraduodenal hernia. Case report

INTRODUCTIONParaduodenal hernia (paramesocolic hernia), a rare congenital anomaly due to a midgut malrotation during fetal development, is recognized as the most frequent internal hernias. Two variants have been described: left and right, the latter less common than the first one. PRESENTATION OF CASEWe report a right paraduodenal hernia case in a 86 years old female patient who developed an acute bowel obstruction syndrome. Final diagnosis was achieved by imaging techniques as abdomen X-ray and CT and confirmed only after surgical operation. DISCUSSIONSurgical approach was via median laparotomy, consisting in hernia reduction, replacement and stitching of the bowel in its anatomical orientation, and fixing of the posterior wall defect. At 15 months follow-up from surgical procedure the patient is asymptomatic. CONCLUSIONParaduodenal hernia is a rare pathology but its involvement in bowel obstruction syndrome should be always taken into account during diagnostic process.

A rare presentation of midgut malrotation as an acute intestinal obstruction in an adult: Two case reports and literature review

INTRODUCTIONMidgut malrotation is a congenital anomaly presenting mainly in the childhood. Its presentation as an acute intestinal obstruction is extremely rare in adults usually recognized intra-operatively, therefore a high index of suspicion is always required when dealing with any case of acute intestinal obstruction. PRESENTATION OF CASEWe report two cases of young adults who presented with symptoms of acute intestinal obstruction and were diagnosed intra-operatively as cecal volvulus and paraduodenal hernia, respectively, caused by midgut malrotation. Post-operative CT scan confirmed these findings. DISCUSSIONMalrotation of the intestinal tract is a product of an aberrant embryology. The presentation of intestinal malrotation in adults is rare (0.2–0.5%). Contrast enhanced CT can show the abnormal anatomic location of a right sided small bowel, a left-sided colon and an abnormal relationship of the superior mesenteric vein (SMV) situated to the left of the superior mesenteric artery (SMA) instead of to the right. CONCLUSIONAnomalies like midgut malrotation can present as an operative surprise and awareness regarding these anomalies can help surgeons deal with these conditions.

Congenital midgut malrotation causing intestinal obstruction in midpregnancy managed by prolonged total parenteral nutrition: case report and review of the literature

Abstract Background: A review of the English literature from 1980 to 2010 revealed only six gravidas managed by total parenteral nutrition (TPN) for at least 16 weeks of pregnancy. We found only two gravidas with obstruction due to malrotation, both managed surgically. Case: We report a unique case of bowel obstruction presenting in the second trimester due to congenital malrotation of the small intestine that was conservatively managed by prolonged TPN, with the delivery of a healthy term baby girl. Surgical correction was delayed until the patient was not pregnant. Conclusion: Intestinal obstruction in pregnancy is rare, and malrotation of the small bowel as the cause is extremely rare. This is the first case of intestinal obstruction in midtrimester in which prolonged TPN was employed when surgery was contraindicated. TPN may be considered if surgery cannot be performed, with an excellent outcome likely.

Midgut volvulus induced extensive bowel infarction.

Midgut volvulus, mostly occurs due to congenital midgut malrotation, has been reported as a rare but lethal complication of some acquired medical conditions, such as postoperative adhesion bands, tumors, and mesenteric cysts. It is a surgical emergency to cause extensive bowel ischemia resulted from torsion of superior mesenteric artery. Early diagnosis and intervention is the only manner to prevent extended bowel necrosis. Here, we report a case of midgut volvulus with typical computed tomography features-the whirl sign, the transposition of the superior mesenteric artery and vein, and the ischemic change of bowel supplied by superior mesenteric artery. Early operation prevented the fate of extended bowel resection.

Midgut malrotation with chronic abdominal pain

Abnormalities in midgut rotation occur during the physiological herniation of midgut between the 5th and 10th week of gestation. The most significant abnormality is narrow small bowel mesentery which is prone to volvulus. This occurs most frequently in the neonatal period, less commonly midgut malrotation presents in adulthood with either acute volvulus or chronic abdominal symptoms. It is the latter group that represents a diagnostic challenge. We report a case of a 17-year-old male patient who presented with 10-year history of nonspecific gastro-intestinal symptoms. After extensive investigation the patient was diagnosed with midgut malrotation following computed tomography of abdomen. The patient was treated with a laparoscopic Ladd's procedure and at 3 months he was gaining weight and had stopped vomiting. A laparoscopic Ladd's procedure is an acceptable alternative to the open technique in treating symptomatic malrotation in adults. Midgut malrotation is a rare congenital anomaly which may present as chronic abdominal pain. Abdominal CT is helpful for diagnosis.

Acute Intestinal Obstruction: A Rare Aetiology

Internal herniation of small intestine is a very rare entity, and it poses a real diagnostic challenge clinically. Recurrent entrapment of the bowel may lead to partial to complete intestinal obstruction and eventually strangulation of the small bowel. Of this rare clinical entity, left paraduodenal hernia is more common. High index of suspicion with prompt management may prevent bowel strangulation and gangrene. We present a case of acute intestinal obstruction due to left paraduodenal hernia with malrotation of midgut in a 55-year-old male patient.

Type 3B malrotation presented with acute appendicitis as left renal colic

Midgut malrotation is a rare anatomic anomaly that complicates the diagnosis and managemant of acute abdominal pain. It is a congenital anomaly that arises from incomplete rotation or abnormal position of the midgut during embryonic development. We report a case of a patient who have very rare form (Nonrotation of the proximal loop associated with partial rotation of the distal loop) of malrotation with ruptured appendicitis. Left-sided acute appendicitis should be considered in the differential diagnosis of patients with pain with localized in the left lower quadrant.

Diagnostic value of CT in adults with midgut malrotation

Objective To evaluate the diagnostic value of CT in adults with midgut malrotation.Methods The CT and clinical findings of 13 cases confirmed by surgery were analyzed retrospectively.Results CT found “whirl sign” in 9 cases,“transposition sign” in 5 cases,and the superior mesenteric vein located in right lower abdomen in 4 cases.At the same time,duodenum and upper jejunum located in right upper abdomen.Conclusion “Whirl sign” and “transposition sign” were the typical CT findings of midgut malrotation in adults.Ectopic superior mesenteric vein highly indicated slight midgut malrotation in adults. Key words: Midgut; Malrotation; CT; Diagnosis; Adults

Pontocerebellar hypoplasia in association with de novo 19p13.11p13.12 microdeletion

The pontocerebellar hypoplasias (PCHs) are a group of clinically variable disorders characterized by abnormally small cerebellum and brainstem, generally inherited in an autosomal recessive pattern. While PCHs have been grouped into six subtypes, clinical diagnosis is equivocal until a genetic diagnosis is established. We report a patient with PCH, intrauterine growth restriction, ventricular septal defect, rib anomalies, midgut malrotation, and facial dysmorphic features. Using SNP analysis, we identified three de novo deletions of: 1.055 Mb at 6q24.3q25.1 (148174730-149229583); 169 kb at 16p13.2 (6565411-6733934); and 2.530 Mb at 19p13.11p13.12 (13857587-16387798), which were confirmed by FISH. 19p13 deletions are rare aberrations. Of patients previously described with overlapping 19p13.12 deletions and multiple anomalies, only one presented with PCH. Deleted in both that patient and the patient reported here, is DDX39, a DEAD-box RNA helicase. DDX39 is part of the homeostatic machinery that regulates the switch of cellular proliferation and differentiation. It is highly expressed in the developing central nervous system and optic cup of Xenopus laevis. The brain abnormalities in the patient reported here were more severe than the previously reported patient, which may be due to additional deletions or undetected point mutations in the nondeleted allele. Notably, the patient reported here also has a partial deletion of RBFOX1 (A2BP1), which lies within the autism susceptibility locus on 16p13.2. Our findings suggest chromosomal microarray analysis may be useful in determining etiology of syndromic PCH.

A Rare Case of Sigmoid Volvulus Secondary to Midgut Malrotation in the Setting of a Carcinoid Tumor in an Eight Year Old Girl

A Rare Case of Sigmoid Volvulus Secondary to Midgut Malrotation in the Setting of a Carcinoid Tumor in an Eight Year Old Girl

Right paraduodenal hernia leading to bowel strangulation

Up to half of all internal hernias are caused by paraduodenal hernia, a rare congenital midgut malrotation that accounts for less than 1% of all intestinal obstructions. The diagnosis may arise from an incidental finding on abdominal imaging or the patient may present with abdominal pain, vomiting, and obstipation. Early recognition and management of this disease entity are keys because serious complications such as bowel ischemia and infarction may result from a delay in diagnosis. We present a case involving a 14-year-old boy with gangrenous small bowel secondary to right paraduodenal hernia.

Midgut malrotation first presenting as acute bowel obstruction in adulthood: a case report and literature review

Malrotation of the midgut is generally regarded as paediatric pathology with the majority of patients presenting in childhood. The diagnosis is rare in adults, which sometimes leads to delay in diagnosis and treatment. A high index of suspicion is therefore required when dealing with patients of any age group with abdominal symptoms. We present a case of a 55-year old man who presented with an acute abdomen with preoperative computed tomography scan and operative findings confirming midgut rotation. The duodenum, small bowel, caecum and appendix were abnormally located, with the presence of classical Ladd's bands. There was no evidence of intestinal volvulus. The patient underwent an emergency laparotomy with an uneventful postoperative recovery.A review of the literature is presented to highlight the rarity of intestinal malrotation and the controversies surrounding its management in the adult population.

A case of midgut malrotation presenting as multiple afebrile seizures

Provoked seizures are a common pediatric problem and can be caused by multiple etiologies. However, there have been few reports of benign infantile seizures associated with gastrointestinal tract abnormality. We report on a 3-month-old male infant who presented with multiple afebrile seizures, EEG abnormalities and a midgut malrotation. Surgical treatment of the malrotation resulted in seizure resolution and EEG normalization. This case emphasizes that patients presenting with multiple seizures and symptoms of gastroenteritis should be evaluated for abnormalities of the gastrointestinal tract.

Malrotation—an unexpected finding at laparoscopic Roux-en- Y gastric bypass: a video case report

A 38-year-old woman with a long history of severe besity and a body mass index of 48 kg/m elected to undergo LRYGB. She had a medical history of pulmonary embolism and laparoscopic cholecystectomy for symptomatic cholelithiasis. She did not undergo any preoperative abdominal radiological studies, and no mention had been made of abnormalities at her laparoscopic cholecystectomy. After placement of the umbilical camera trocar, the initial inspection of the abdominal cavity revealed what appeared to be a mobile cecum and easily visualized appendix (see video). On retrospect, this should have alerted us to the possibility of midgut malrotation. Additional trocars were placed (Fig. 1), and the gastric pouch was created. The omentum was then grasped in an attempt to elevate the omentum and transverse colon cephalad. At first, it was thought that adhesions were tethering the transverse colon, but we then realized that the entire

Left-sided Acute Appendicitis: A Pitfall in the Emergency Department

BackgroundAcute appendicitis continues to be a condition at high risk for missed and delayed diagnosis. It characteristically presents with right lower quadrant pain after vague epigastric or periumbilical discomfort. Left-sided appendicitis is an atypical presentation and has been reported rarely. The majority of these cases have been described to be associated with congenital midgut malrotation, situs inversus, or an extremely long appendix. We report a case of left-sided acute appendicitis occurring in a patient with a redundant and hypermobile ascending colon. ObjectivesTo alert emergency physicians to an anatomical anomaly that could delay the diagnosis of appendicitis. Case ReportA 50-year-old man presented with fever and left lower abdominal pain. Physical examination revealed local tenderness over the left lower quadrant. Abdominal computed tomography scan revealed a redundant, floating, ascending colon and inflammatory appendix adhering to the descending colon over the left lower abdomen. Exploratory laparotomy was performed and perforated appendicitis with turbid ascites was found during the surgery. Appendectomy was performed and the patient recovered uneventfully. ConclusionThis case is presented to increase awareness among emergency physicians of this anatomical variant and atypical presentation of appendicitis.

Updates in neonatal gastrointestinal US

Sonography has come to play a significant role in imaging of the gastrointestinal (GI) tract in neonates. Although plain radiographs of the abdomen remain essential in some situations, the information obtained from radiographs regarding the bowel itself is often limited. US has several advantages over radiographs in that it can provide detailed information about the bowel wall regarding echogenicity (including intramural gas) and thickness; real-time images can provide information regarding peristalsis, and Doppler techniques can provide information regarding perfusion. US can also clarify whether a gasless area on a radiograph is the result of collapsed bowel, fluid-filled bowel, free fluid or a focal fluid collection. Separation of bowel gas on abdominal radiographs is a nonspecific appearance, and US might elucidate whether this is a result of true bowel wall thickening or fluid within the bowel lumen or in the peritoneal cavity. Furthermore, US can easily depict pneumoperitoneum caused by very small amounts of free gas at least as accurately as plain radiographs and probably even more accurately. To make the best use of US in the evaluation of the neonatal GI tract one has to optimize technique. The wider field of view obtained with vector or curved-array transducers is essential to evaluate the abdomen as a whole and to delineate the overall relationship of the bowel to other structures and free fluid or focal fluid collections. However, the features of the GI tract itself are often better depicted with the high-megahertz linear-array transducers. It is essential to magnify these images so as to depict only an individual or just a few bowel loops at any one time and to ensure that the focal zone is directed at the level of maximum interest in the GI tract. A relative limitation of US is the presence of large amounts of bowel gas but the technique of gentle graded compression can be useful in displacing the gas for better visualization. In neonates with severe abdominal tenderness, abdominal US might cause significant discomfort but the use of a large amount of gel on the abdominal wall can facilitate the examination by enabling images to be obtained without the transducer actually touching the abdomen. US should not be attempted in any neonate who is labile or unstable. There is a wide range of clinical situations in which abdominal US is invaluable for assessment of the neonate with known or suspected GI tract pathology. For example, in neonates with non-bilious vomiting, US can easily differentiate those with hypertrophic pyloric stenosis from those with gastric foveolar hyperplasia or other rarer causes of gastric outlet obstruction. In neonates with bilious vomiting US is the modality of choice to determine whether a midgut volvulus is present. In those neonates without a volvulus, US evaluation of the anatomical position of the entire duodenum (facilitated with intraluminal fluid) might elucidate the presence or absence of midgut malrotation. In the above examples US will obviate the necessity for an upper GI series. Another less common use of US is in the evaluation of the esophagus in neonates suspected of having an N-type tracheoesophageal fistula. Abdominal radiographs are still important in neonates suspected of having necrotizing enterocolitis (NEC). However, US can be invaluable in two clinical situations: first, in those neonates in whom the clinical and radiographic findings are nonspecific and the diagnosis remains indeterDisclaimer Dr. Daneman has no financial interests, investigational or off-label uses to disclose.

Endoscopic intragastric balloon: a bridge toward definitive bariatric surgical management of a morbidly obese patient with situs ambiguous and midgut malrotation (with videos)

d a m t g w i 2 m t o F w Situs ambiguous or heterotaxia, with polysplenia (leftsided isomerism), is a rare anomaly of organ arrangement.1 It is estimated to occur in about 4 per 1 million live births. Most affected persons die by the age of 5 years, mainly because of cardiac anomalies.2 Today, we have various surgical and endoscopic options for weight reduction in morbidly obese patients. Various surgeries in patients with situs inversus have been reported.3,4 We report this case for its extremely rare ocurrence and to emphasize the role of endoscopic balloon lacement as a bridge toward definitive bariatric surgical anagement in difficult cases of morbid obesity in which he laparoscopic approach remains challenging.

Acute presentation of intestinal malrotation in adults: a report of two cases

Intestinal malrotation is a rare developmental abnormality occurring as a result of incomplete rotation during fetal life. It usually presents in the first few weeks of life, but may persist unrecognised into adult life. We report two interesting cases in elderly patients both characterised by a significant diagnostic challenge due to atypical clinical and radiological signs and in one case an unusual complication following laparotomy. The first case was a 64-year-old man initially treated for diverticulitis but at laparotomy was found to have malrotation of the midgut and a perforated left-sided appendicitis. The second case was a 76-year-old woman admitted with multiple fractures and increasing abdominal distension following a fall. Ten days after admission, she underwent right hemicolectomy to treat faecal peritonitis due to multiple caecal perforations complicating volvulus in the presence of midgut malrotation. These cases illustrate challenges associated with managing patients with undiagnosed intestinal malrotation. Delayed diagnosis is a common feature in several case reports describing atypical presentation of appendicitis in patients with malrotation. While abdominal CT scan can remove much of the diagnostic uncertainty, the diagnosis of malrotation can be missed unless there is a high index of suspicion.

Laparoscopic Ladd's Procedure: Treatment of Choice for Midgut Malrotation in Infants and Children

Rotational anomalies of the midgut encompass a broad spectrum of incomplete rotational events with malfixation of the intestines during fetal development. Ladd's procedure, as a correction of these anomalies, has traditionally been performed by laparotomy. In our institution, the laparoscopic Ladd's (LL) procedure was introduced in May 2004 and soon became the standard approach. A retrospective analysis of all Ladd's procedures in children in our institution between September 1998 and June 2008 was performed. Outcomes between the open (OL) and LL procedures were compared. A total of 156 children underwent Ladd's procedure during the study period. There were 120 open and 36 laparoscopic procedures. Overall, 75% of patients in each group were symptomatic, most commonly with emesis and pain. Duration of surgery was similar in both groups. Time to starting feeds, and amount of time to attain full feeding, was significantly less in the LL group. Postoperative length of stay was significantly less in the patients having LL. Conversion rate to OL from LL was 8.3%. LL can be performed safely in selected patients with no increase in complications. Short-term results are superior to OL and can be achieved without any increase in operative duration.