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Superior mesenteric arteriovenous fistula is an extremely rare vascular malformation with most cases occurring following abdominal trauma or surgery. They are often asymptomatic or present with various abdominal symptoms with or without features of portal hypertension. A 30-year-old man developed fistulising of the superior mesenteric artery into the superior mesenteric vein following bowel resection surgery for an acquired midgut volvulus. Although endovascular management remains the treatment of choice in such cases due to increased morbidity of a repeat abdominal surgery, definite risks remain, such as coil migration, which happened in this case. The open surgical approach remains the only option in such instances.

Aim: There are two types of intestinal volvulus: midgut (MGV) and segmental (SV). Patients with different types of intestinal volvulus are often included in the same case series, which may affect the perception of how severe "intestinal volvuli" are. We aimed to compare both types of intestinal volvulus. Methods: This is a retrospective observational study including all patients with MGV and SV up to 28 days of life admitted to a tertiary hospital in Spain over a 20-year-period (1999-2019). A comparison between groups and a logistic regression model for mortality were done. Results: We identified 32 patients: 23 MGV and 9 SV. Malrotation was exclusive of MGV. Prenatal diagnosis, cystic fibrosis, and intestinal resection were significantly more frequent in SV. Surgery was performed at a significantly lower age in SV. The mortality observed in acute MGV with intestinal compromise (41.7%) is four times higher than the mortality of SV (11.1%). The overall mortality of all MGV patients (21.7%) is almost twice that of SV. Mortality was best predicted by the presence of hemodynamic instability (OR 27.5 95% CI 2.50-302.17; p = 0.007). Conclusion: SV and MGV have a different clinical presentation. Hemodynamic instability is the major risk factor for death.

Mesentery is an uncommon location for occurrence of lymphatic malformations. Lymphatic malformations causing midgut volvulus are described in pediatric population; however, it is a rare presentation in adults. We present case of 20-year-old man with a large mesenteric lymphatic malformation who presented with acute abdominal complaints. On radiological work up, the lymphatic malformation was seen causing midgut volvulus and closed loop small bowel obstruction with the presence of classical whirlpool sign. The patient underwent emergency surgery and the lymphatic malformation was completely excised along with resection of a segment of small bowel loop.

Congenital hyperinsulinism (CHI) is a rare glucose metabolism disorder characterized by unregulated secretion of insulin that leads to hyperinsulinemic hypoglycemia (HH). Most cases are caused by mutations in the KATP-channel genes ABCC8 and KCNJ11. We report 2 patients that experienced severe HH from the first day of life. Patient 1 developed midgut volvulus after initiating diazoxide and required intestinal resection. He was subsequently managed with a high-dose octreotide and glucose-enriched diet. Consistent with diffuse type CHI by 18F-dihydroxyphenylalanine positron emission tomography-computed tomography, genetic testing revealed a homozygous ABCC8 variant, c.1801G>A, p.(Val601Ile). The rare variant was previously reported to be diazoxide-responsive, and the patient responded well to diazoxide monotherapy, with clinical remission at 2 years of age. Patient 2 responded to diazoxide with spontaneous clinical remission at 15 months of age. However, an oral glucose tolerance test at 7 years of age revealed hyperinsulinism. Genetic testing revealed that the proband and several seemingly healthy family members harbored a novel, heterozygous ABCC8 variant, c.1780T>C, p.(Ser594Pro). Genetic findings identified previously unrecognized HH in the proband's mother. The proband's uncle had been diagnosed with monogenic ABCC8-diabetes and was successfully transitioned from insulin to glibenclamide therapy. We report findings of intestinal malrotation and volvulus occurring 2 days after initiation of diazoxide treatment. We also report a novel, heterozygous ABCC8 variant in a family that exhibited cases of CHI in infancy and HH and monogenic diabetes in adult members. The cases demonstrate the importance and clinical utility of genetic analyses for informing and guiding treatment and care.

Highlights • Midgut volvulus related to adult intestinal malrotation found with weight loss is rare. • The development of collateral circulation from SMV to portal vein did not lead to intestinal necrosis. • It’s controversial whether it should be an emergency surgery or an elective surgery. • In the chronic course, there is a high degree of adhesions, and countermeasures against postoperative ileus are necessary. • If a patient complains of gastrointestinal symptoms, intestinal malrotation should be raised in the differential diagnosis., Introduction and importance The incidence of intestinal malrotation is 1 in 6000 births, and 90% of cases occur within the first year of life. Adult cases are rare, with a reported incidence of 0.2%–0.5% of all cases. The significance of reporting this case is to recognize that some adult-onset cases require surgery even in the absence of intestinal necrosis. Case presentation A 36-year-old man was infected with streptococcus and treated with antibiotics. He developed appetite loss and his weight decreased 12 kg in 4 months. His abdomen was flat and soft with no tenderness. A computed tomography scan showed that the horizontal duodenal leg was not anchored to the retroperitoneum. Rotation of the mesentery, which was wrapped around the superior mesenteric artery in a clockwise direction, was observed, suggesting midgut volvulus. We performed emergency surgery and Ladd’s procedure. Clinical discussion A previous study reported that the most common symptom in the chronic course of intestinal malrotation was abdominal pain in 41.2% of cases, and weight loss was observed in only 2.6% of patients. The high degree of intestinal adhesion suggests that repeated torsion and release and the development of collateral vessels may have contributed to the asymptomatic course. Conclusion Adult-onset intestinal malrotation should be considered as a differential diagnosis in the presence of weight loss and gastrointestinal symptoms. The timing of surgery is still controversial. In chronic cases, severe adhesion might be expected and laparoscopic surgery should be considered carefully.

Author(s): Fogam, Michael; Leigh, Natasha; She, Trent | Abstract: Introduction: Adult intestinal malrotation with midgut volvulus is rare and most often diagnosed on abdominal imaging. Once the diagnosis is made, prompt surgical intervention is necessary. A finding suggestive of malrotation with midgut volvulus on abdominal imaging is the “whirlpool” sign where the superior mesenteric vein and superior mesenteric artery twist at the root of the abdominal mesentery. This sign was once thought to be pathognomonic, but recent studies have shown that it can be seen in asymptomatic patients.Case Report: A 20-year-old female presented to our emergency department with diffuse abdominal pain. Computed tomography demonstrated the “whirlpool” sign with a concern for malrotation with midgut volvulus. Surgical consultation was obtained and the patient was rushed to the operating room for an exploratory laparotomy. Normal mesenteric attachments were seen and no significant pathology was identified during the laparotomy. The patient was eventually diagnosed with gastritis and discharged in stable condition.Conclusion: Emergency physicians and surgeons alike should be cautious in confirming malrotation with midgut volvulus solely due to the “whirlpool” sign on abdominal imaging. Premature diagnostic closure can lead to unnecessary procedures and interventions for patients as in the case we report here.

Heterotaxy syndrome is a spectrum of abnormalities that should be differentiated from situs inversus. Multiple spleens are the most common malformation seen in this syndrome. Intestinal rotation abnormalities should always be looked for, because of the risk of midgut volvulus that can be prevented by a correction procedure. We report the case of a 43-year-old patient with heterotaxy syndrome.

Highlights • Diverticuli are found throughout the gastrointestinal tract from esophagus to rectum. • In the jejunum diverticuli are most commonly proximal, multiple and asymptomatic. • A large solitary jejunal diverticulum caused life endangering midgut volvulus. • In the absence of timely surgical intervention the condition would have been fatal., Background Jejunal diverticula are a rare subtype of false diverticula found in the gastrointestinal tract. When present, they are usually multiple, in the proximal jejunum and asymptomatic. Rarely, they can cause acute complications that can develop into an acute abdomen requiring surgical intervention. We present the rare manifestation of a single jejunal diverticulum causing midgut volvulus and bowel ischemia. Early surgical intervention with small bowel derotation allowed complete recovery. Methods Data regarding the case, operative intervention, and patient follow-up was prospectively accumulated with permission of the patient at an academic institution. All patient identifiers were removed. All research steps were performed under guidance outlined in the SCARE criteria. Results We present the case of a 78-year-old man who presented to our institution with an acute abdomen. CT imaging demonstrated a whirlpool sign without an obvious lead point. The patient was without prior surgical intervention. Urgent exploratory laparotomy revealed a midgut volvulus with associated bowel ischemia and impending infarction secondary to adhesive disease from a large single jejunal diverticulum. Derotation resulted in gradual recovery of bowel vascularity. Resection of the portion of the jejunum containing the diverticulum resulted in full clinical recovery of the patient. Conclusions Complications of jejunal diverticula, although rare, should be considered as part of the differential diagnosis of an acute abdomen of unknown etiology. Urgent surgical intervention was required to avoid a potential catastrophic outcome.

The developing intestine rotates around the superior mesenteric vessels during physiological herniation from 6th to 10th weeks of embryogenesis. Intestinal malrotation leading to midgut volvulus and small bowel obstruction is a rare condition. Patients who develop this condition usually present in the first year of their life. Intestinal malrotation is characterized by right sided duodenojejunal junction, caecum in left iliac fossa and a narrow mesentery. Ladd’s procedure is the treatment of choice which is done to restore the normal anatomy. We report the case of an 18-year-old male patient, who presented with intestinal malrotation in emergency department of Nishtar Hospital, Multan. It is an uncommon age for presentation of this anomaly. The patient had fecal peritonitis due to caecal perforation. He underwent Ladd’s procedure in which ileocolostomy was made after right hemicolectomy. The patient had an uneventful recovery after the surgery and had no complaints on follow up visit one week after the discharge.

Aim: To compare clinical outcomes in infants and children with intestinal malrotation who were treated by laparoscopic or open Ladd's. Methods: PubMed, EMBASE, and Cochrane library were searched fo...

Highlights • Intestinal malrotation and midgut volvulus in adulthood are rare events. • Reports of recurrence among adults are very scarce. The rate of recurrence and optimal surgical management are yet to be determined. • Bowel fixation procedures may be considered in Ladd procedures for adult midgut volvulus in order to reduce recurrence., Introduction Intestinal malrotation is a rare condition, with an incidence estimated between 0,2 to 1%. Most cases are diagnosed and treated during childhood. Adult presentations are rare and most adults present with chronic nonspecific complaints. Midgut volvulus is the most feared complication of intestinal malrotation, far more common among the pediatric than the adult population. Presentation in adulthood with a midgut volvulus accounts for a minority of these patients (15%). The Ladd procedure is the standard surgical management of midgut volvulus and intestinal malrotation. Most evidence on the outcomes of the Ladd procedure originates from studies on pediatric population and the recurrence among children who have had a Ladd procedure is low (2–7%). Presentation of case We report an exceedingly rare case of a patient who presented in adulthood with a midgut volvulus and less than two years after undergoing Ladd procedure presented with a recurrence of the midgut volvulus. The recurrent midgut volvulus was successfully treated by a fixation procedure (cecopexy and duodenopexy). Conclusion Reports of midgut volvulus in adult patients are scarce and reports of recurrence even scarcer hence the rate of recurrence among adult patients has yet to be determined. The recurrence rate in some of the available adult series is much higher than the rate reported among children. Should the rate of recurrence among adult patients prove higher, it poses the question of whether the Ladd procedure should be modified to include bowel fixation when performed in adults.

Midgut volvulus is a rare cause of acute abdomen characterized by a twisting of the entire small intestine around its mesenteric pedicle. It is a well-known complication of intestinal malrotation usually occurring in infants and young children. We report a rare case of a midgut volvulus without any underlying condition occurring in a young adult.A 24-year-old male was admitted to our surgical department with symptoms and signs of small bowel obstruction. The abdominal computed tomography (CT) scan showed dilatation of the entire small intestine and a rotation of the mesentery. The patient underwent an urgent explorative laparoscopy with conversion to median laparotomy, revealing a midgut volvulus with marked intestinal ischemia. Mesenteric fixation was normal but the mesenteric pedicle was described as relatively narrow. The midgut volvulus was untwisted with subsequent recovery of the intestinal ischemia.The postoperative period was marked by prolonged ileus requiring nasogastric tube decompression that gradually resolved after 1 week. The patient was discharged on the tenth postoperative day. He had fully resumed his daily activities 1 month after surgery and had experienced no recurrent symptoms 6 months later.Primary midgut volvulus is an extremely rare condition that must be considered in all young adults presenting with intestinal obstruction without previous abdominal surgery. Early diagnosis and immediate surgical intervention are the key factors to a successful outcome.

We present a rare case of recurrent primary midgut volvulus in an elderly female with an interesting intraoperative finding of an abnormally elongated small bowel mesentery. This patient presented with symptoms of obstruction, including nausea, vomiting and obstipation, similar to previous episodes of volvulus for which she underwent exploratory laparotomies and reduction of the volvulus. We describe a novel use for enteropexy in which we effectively shortened the small bowel mesentery in an effort to eliminate the source of recurrent volvulus. The patient’s post-operative course was complicated by prolonged ileus requiring total parenteral nutrition. However, she had not developed signs or symptoms of bowel ischemia or recurrent volvulus at the time of this writing. Our findings suggest that enteropexy is an effective technique for preventing recurrent midgut volvulus primarily caused by abnormally elongated mesentery.

Chronic midgut volvulus is a rare condition and the diagnosis can pose a serious challenge. We present the case of an 8 year old girl, with a long-standing history of atypical gastrointestinal symptoms. Although extensive evaluation was performed, the diagnosis of intestinal malrotation with chronic volvulus was only established after 5 years. Her symptoms resolved after corrective surgery. This case report underlines the importance of considering malrotation in the differential diagnosis of patients that present beyond infancy with vague nonspecific gastrointestinal complaints, as a misdiagnosis can result in a catastrophic outcome.

Disorders of intestinal rotation arise during gestation and represent the failure of normal 270-degree counterclockwise rotation of the intestines around the superior mesenteric artery (SMA). Malrotation is associated with a narrow mesenteric base and intestines what are not fixed to the abdominal sidewall or retroperitoneum. This anatomy predisposes the gastrointestinal (GI) tract to volvulus, in which the bowel twists around the SMA, forming a closed loop obstruction of the entire small bowel with interruption of arterial inflow and venous drainage. Bilious emesis in an infant is the clinical finding that signals this life-threatening complication, and must be addressed with expedient operative exploration if the patient is in extremis versus rapid upper GI contrast study. The definitive surgical management of malrotation with midgut volvulus is Ladd’s procedure, which includes reduction of volvulus, lysis of adhesive bands, and placement of the small bowel in the right abdomen and large bowel in the left abdomen. This chapter discusses the embryology, presenting features, acute clinical management, and the role of laparoscopy and prophylactic Ladd’s procedure for children with malrotation.

Incomplete intestinal fixation or malrotation of gut with midgut volvulus is one of the important causes of bilious vomiting in neonates. The incidence of malrotation of gut in population is 4% and that of duplication cyst is 1:4500. Patients with malrotation are prone to develop midgut volvulus due to their narrow mesenteric base demanding urgent surgical intervention. Common associated anomalies are intrinsic duodenal obstruction, internal hernias, caecal volvulus, anorectal malformations and Hirschsprung’s disease. The present case refers to a 4-day-old neonate who presented with malrotation of gut with reverse volvulus and an associated gastrointestinal duplication cyst, which is a rare association with only few reported case reports. After imaging with ultrasound and contrast radiograph, the baby underwent prompt surgical intervention in the form of Ladd’s procedure with resection and anastomosis of jejunal duplication cyst.

De novo HCC following transplantation in a child is a rare occurrence. Even within the adult liver transplantation population, there are a limited number of published cases. In this report, we present a case of de novo HCC found in a child, post-multivisceral transplantation. A 19-year-old boy, at the age of one, received liver and small bowel transplantation due to short gut syndrome secondary to midgut volvulus and total parenteral nutrition-associated liver disease. Eighteen years later, he was found to have a large mass involving the right hepatic dome consistent with HCC. To the best of our knowledge, this is the second reported case after gut transplantation and the third case post-liver transplantation in the pediatric population.

Mesenteric Volvulus from Jejunal Diverticulosis A 75-year-old man presented with abdominal pain and vomiting, and imaging showed a “whirl sign” suggestive of midgut volvulus. Jejunal diverticula we...

Introduction: Since the first description by William Ladd, the Ladd's procedure has been the surgery of choice for the correction of malrotation. The laparoscopic Ladd's procedure is becoming popular with the advent of minimal access surgery and is described in the literature. Various techniques of the Ladd's procedure have been described but none of them describes the stepwise technique for derotation of volvulus which is the most difficult and confusing part of the surgery. We describe 'steering wheel' technique for easy derotation of volvulus associated with malrotation. Method: A total of 62 patients were diagnosed to have an intestinal malrotation between 2010 and 2017. All cases which had complete non-rotation with a midgut volvulus were reviewed. Out of these, 48 patients were operated with open technique and 14 patients were subjected to the laparoscopic correction. Technique: Using three-port technique, stepwise derotation of volvulus is done which simulates the rotation of steering of car at an acute turn and has been described in four simple steps. This technique also stresses the importance of the release of Ladd's band before derotation. Results: Of 62 patients diagnosed with malrotation, 14 (22.6%) patients underwent the laparoscopic Ladd's procedure. The mean age was 26 + 8 months, mean weight was 10 + 2 kg and included eight males (57%) and six females (43%). There was only one (7.14%) conversion to open technique, due to a huge dilatation of duodenum causing difficulty in dissection in a patient with malrotation without volvulus. The laparoscopic Ladd's procedure took an average time of 70 ± 15 min. Conclusion: The laparoscopic 'steering wheel' derotation technique is easy and provides a stepwise description of the laparoscopic derotation of volvulus associated with malrotation in children.

Intestinal malrotation is a congenital anomaly of intestinal rotation and fixation that is generally symptomatic in the neonatal population. However, malrotation in an important minority is diagnosed long after infancy and is not manifested by the typical clinical manifestation of acute bilious vomiting with or without abdominal distension in an otherwise normal newborn. Here, we present a case of mesenteric cyst as a presentation of chronic midgut volvulus in a 12 year old male patient with intestinal malrotation.

Highlights • Midgut volvulus secondary to intestinal malrotation is a rare cause of acute abdomen in adults. • There are only 92 reported cases in the literature. • Diagnosis of the midgut volvulus was predominantly made via CT (67%) but also by ultrasound (15%) and theatre (18%). • Midgut volvulus is associated with a high risk of ischaemia and necrosis of bowel supplied by the SMA (35). • There is a 5% associated mortality rate., Introduction Midgut volvulus secondary to intestinal malrotation is a rare cause of an acute abdomen in adults, with 92 confirmed cases in the literature. Incidence of malrotation is estimated 1 in 6000 live births. 64–80% of malrotation cases present in the first month of life and 90% within the first year. Adult presentation is very rare accounting for only 0.2–0.5% of cases, of which only 15% present with midgut volvulus. Presentation of case We report a rare case of a 20 year old male with spontaneous midgut volvulus secondary to congenital malrotation of the bowel. Additionally we performed a literature review and analysis of the 92 cases of adult presentations of midgut volvulus secondary to malrotation. Discussion Of the 92 cases, average patient age was 40 years old and a 1.7:1 male:female ratio. Diagnosis of midgut volvulus was predominantly made via CT (67%) but also by ultrasound (15%) and theatre (18%). Midgut volvulus is associated with a high risk of ischaemia and necrosis of bowel supplied by the SMA (35). 19% of cases reported required a bowel resection. The case discussed in this report required a 130 cm bowel resection which is similar to the mean bowel resection length in the literature of 121 cm. Mean associated mortality rate is 5%. Conclusion This case reinforces the importance of maintaining a high index of suspicion and closely monitoring patients presenting with non-specific abdominal pain, to allow early recognition and management of rare causes of the deteriorating surgical patient.

Introduction: Intestinal malrotation is a consequent of deviation from the normal 270 degrees counter-clockwise rotation of the midgut during embryogenesis. It is a typically paediatric disease that manifest itself in the first month of life, usually as midgut volvulus. However adult manifestation of intestinal malrotation is not unheard of, albeit rare. Case report: his is a case report of 46 year old man with chronic abdominal pain who was subsequently diagnosed with intestinal malrotation. He was initially referred for barium enema following an incomplete colonoscopy. The suspicion of intestinal malrotation arose during barium enema, as the large bowel loops were all located on the left side. He subsequently had a plain CT following the barium enema, which confirmed the suspicion. In view of this finding, the patient was offered Ladd’s procedure by the surgical team. The patient duly declined and opted for conservative watchful waiting instead.

Background The safety of the laparoscopic treatment of intestinal malrotation remains controversial. This study compared the outcomes of laparoscopic and open surgical treatment of intestinal malrotation. Methods A multicentric retrospective study included pediatric cases of intestinal malrotation operated on between 2005 and 2016. Results This study included 227 children with a median age of 17 days (0–17.2 years), including 161 with a midgut volvulus. Forty-six(20.3%) procedures were started by laparoscopy and 181(79.7%) by laparotomy. Laparoscopy was more frequent for elective surgery (45.9%) than for emergency procedures (10.8%, p Conversions were significantly more frequent during emergency procedures (66.7% vs 17.9%)(p = 0.001). Considering only 61 elective surgeries, the mean hospital stay was significantly shorter after laparoscopy (5.3 days +/−5.2 vs 10.1 days +/−13, p = 0.01), the overall complication rate was comparable (15.8% vs 21.7%, p = 0.7) but post-operative volvulus was significantly more frequent after laparoscopy (13% vs 0%, p = 0.04). Outcomes of the two approaches were not significantly different after 166 emergency procedures. Conclusion Laparoscopy can be performed by experienced team for the treatment of selected cases of intestinal malrotation. Conversion to open surgery should be done with a low threshold, as the rate of volvulus recurrence is concerning. Level of Evidence Level III.

Midgut volvulus in pregnancy is rare but life-threatening, resulting in high maternal and fetal mortality. This surgical emergency commonly masquerades as symptoms of pregnancy, which together with its low incidence often leads to delay in diagnosis and definitive treatment. Here, we review the last three decades of the literature, discuss the challenges in managing this rare condition, and raise awareness among clinicians to minimise loss of life.

Fetal volvulus of the midgut occurs when the bowel twists around the axis of the superior mesenteric artery. It is usually diagnosed postnatally but with improving ultrasonography, there have been increasing number of cases reporting antenatal diagnosis that had allowed planning of obstetric intervention and prompt surgery in the postnatal period. We present a case of dichorionic diamniotic twin pregnancy wherein Twin A had multiple anomalies, which included an Ivemark heterotaxy syndrome with a double outlet right ventricle. This twin developed dilated bowel loops at 33 weeks of gestation. The pregnancy continued to have regular antenatal surveillance and was managed by a multidisciplinary team. At 36 weeks of gestation, these bowel loops were found to have absence of peristalsis compared to previous ultrasounds. An emergency caesarean section was performed, which was uncomplicated and Twin A weighing 2760g was born with Apgars of 61 and 85. The diagnosis of volvulus in Twin A was confirmed during its emergency laparotomy along with a Type IV jejunal atresia and was successfully treated surgically with resection and administration of tissue plasminogen activator (TPA). This is the first description of a case treated with TPA after fetal diagnosis. Keywords: Volvulus, Antenatal diagnosis, Tissue plasminogen activator, Congenital anomalies

Enteric duplication cysts occurring in association with intestinal malrotation are rare, with only a handful of case reports published. We describe a 5-week-old female patient who presented to our institution with a one-day history of bile-stained vomiting with an associated large palpable abdominal mass on clinical examination. Ultrasound evaluation suggested a large cystic lesion arising from the right kidney, however, Intra-operatively we found a jejunal duplication cyst with an associated malrotation and midgut volvulus.

Background Portal venous gas has been considered as a radiological sign requiring urgent operative intervention; however, the reports concerning portal venous gas associated with favorable outcome are recently increasing. Case presentation We describe a 9-month-old boy with acute onset high fever and vomiting. The ultrasonography demonstrated micro-gas bubbles continuously floating in the intrahepatic portal vein. Contrast-enhanced CT, performed 1 h later from echography, revealed a whirlpool sign suggesting an intestinal malrotation with midgut volvulus, but with no signs of residual intrahepatic gas. Operative findings showed a mild volvulus with neither congestion nor ischemic change of the twisted bowel. Detorsion and Ladd’s procedure were completed laparoscopically. Conclusions Transient portal venous gas bubbles may be generated even in the mild intestinal volvulus with no bowel ischemia. Ultrasonography can be a sensitive detector to visualize such small amounts of gas.

Background Open Ladd's procedure is the gold standard for the correction of intestinal malrotation and laparoscopic approach remains controversial. This study aimed to evaluate our experience in laparoscopic management of malrotation. Methods Single center retrospective study including patients who underwent a laparoscopic assessment of intestinal malrotation with correction if appropriate between 2007 and 2017. Results Sixty-five patients (median age 7 months) had a laparoscopic assessment with and without correction of malrotation. Forty-five (69%) were symptomatic, including 16 (25%) with a midgut volvulus. The procedure was completed laparoscopically in 55 (86%) patients in 110 min (30–190). Conversions happened more frequently at the beginning of the experience. With a follow-up of 12.5 months (8 days–5.3 years), morbidity rate was 15% and 4 (6%) patients underwent a redo surgery, all in the first 5 months after surgery, compared with 3/53 (6%) in a contemporaneous group undergoing open Ladd's. Conclusion This is the largest series reported so far of the laparoscopic management of malrotation. Laparoscopic Ladd's procedure is reliable but still exposes to open conversion which may be in part owing to a learning curve. A low conversion threshold is important in cases with volvulus. The redo rate is similar to that of the open procedure. Level of evidence Level III retrospective comparative treatment study.

A cause of acute intestinal obstruction in adults, midgut volvulus can be categorized into two types: primary type with no identifiable underlying cause, and secondary type that occurs in the presence of a predisposing condition such as, postoperative adhesions. Primary midgut volvulus can lead to bowel ischemia and necrosis, making an extensive bowel resection imminent. A potential consequence of bowel resection is short-bowel syndrome - a failure of digestion and absorption by the intestines, leading to malnutrition and other complications. As such, we report the diagnosis and management of primary midgut volvulus - a rare entity in adults - occurring in an adult patient.

Marfan syndrome is a mutation in the fibrillin-1 gene resulting in a connective tissue disorder primarily affecting musculoskeletal, cardiovascular and ocular systems. However, patients with Marfan’s rarely manifest gastrointestinal symptoms. Midgut volvulus is abnormal twisting of small bowel around its mesentery that can result in compromising blood flow to the bowel causing intestinal ischemia and obstruction. Primary midgut volvulus is a term used when there is no underlying cause for the volvulus. This case describes an 80-year-old female with Marfan syndrome presenting with primary midgut volvulus, which preoperatively was suspected based on imaging, and later confirmed upon operative exploration. The small bowel mesentery was long with a narrow base twisted around its mesentery 360°. The long narrow base and floppy mesentery likely contributed to hypermobility leading to volvulus and small bowel obstruction. To our knowledge, this is the first reported case of primary midgut volvulus associated with Marfan’s syndrome.

Point-of-care ultrasound can be used to screen for malrotation with and without volvulus in the newborn with bilious vomiting, as well as children with unexplained intermittent abdominal pain. We discuss cases where infants and children presenting to pediatric emergency departments with bilious vomiting and/or intermittent abdominal pain were initially screened for small bowel pathology with point-of-care ultrasound. Bedside findings suggestive of midgut volvulus were confirmed with radiology-performed ultrasound or upper gastrointestinal fluoroscopy. In all cases, operative findings were consistent with malrotation of the small bowel with or without evidence of midgut volvulus.

Intestinal malrotation with midgut volvulus presenting in adults is a rare entity, and association with jejunal diverticulum is rarer still. Herein, we report and review such a case of malrotation and volvulus, associated with intestinal band adhesions and a solitary jejunal diverticulum. This 67 years old gentleman had presented with complaints of intermittent abdominal pain for past several years. Imaging of the abdomen revealed twisting of superior mesenteric artery and vein, evidence of midgut volvulus and intestinal malrotation with “whirlpool sign”. Laparotomy revealed a midgut volvulus, extensive adhesions involving the root of the mesentery and a jejunal diverticulum. Adhesiolysis was performed, untwisting of the bowel was done and the jejunal diverticulum was resected. Post-operative period was uneventful. This case is being reported on account of its extreme rarity.

Intramesenteric internal hernia is a rare type of small bowel mesentery related internal hernia which usually occur in pediatric population. It is characterized by herniation of small bowel loops through a congenital or acquired defect in one layer of the mesentery producing a mesenteric pouch type hernia. The defect usually occurs near the ileocaecal valve or ligament of treitz and the herniated bowel loops are prone to strangulation or volvulus, presenting as small bowel obstruction that warrants early recognition and emergency laparotomy to confirm the diagnosis and prevent bowel gangrene and its complication. We present a case of 59 year old male presenting with features of an acute intestinal obstruction since morning and a vague firm mass in the right upper quadrant which was confirmed by CT scan to be an internal hernia and patient was taken up for emergency laparotomy. Intraoperative findings revealed a sac of small bowel loops herniating into the mesentery through a defect near the terminal ileum. Upon reducing the contents a volvulus was evident and resulted in the gangrene of the small bowel loops. The gangrenous bowel was resected and a single layered end to end ileo-ileal anastamosis was done. The diagnosis of internal hernia in a adult with no previous history of abdominal surgery or trauma is a very rare entity and requires a high degree of suspicion and close monitoring of the patient to ensure early surgical intervention and optimal outcome.

An otherwise healthy 36-year-old man presented with sudden-onset right upper quadrant abdominal pain and vomiting. A bedside ultrasound, performed to evaluate hepatobiliary pathology, revealed a normal gallbladder but free intraperitoneal fluid. After an expedited CT and emergent explorative laparotomy, the patient was diagnosed with a small bowel obstruction with ischemia secondary to midgut volvulus. Though midgut volvulus is rare in adults, delays in definitive diagnosis and management can result in bowel necrosis. Importantly, an emergency physician must be able to recognize bedside ultrasound findings associated with acutely dangerous intrabdominal pathology.

We report a case of a 10-month-old girl who presented with a 10-day history of emesis that became bilious on the last day. The initial evaluation suggested small bowel obstruction. An upper gastrointestinal study confirmed the normal location of the duodenojejunal junction with normal rotation and no evidence of midgut volvulus. Ultrasound (US) evaluation demonstrated two intraluminal lesions that do not follow the typical features of solid or cystic lesions. Two rubber balls were found at surgery to be responsible for the small bowel obstruction. Foreign body ingestion is common in children, but this case demonstrates a unusual foreign object to be ingested as well as the US appearance of this particular foreign body.

Midgut volvulus complicating congenital malrotation is a rare but life-threatening condition that can occur in pregnancy. We present a case of intestinal infarction resulting from midgut volvulus in a healthy 32-week pregnant woman who underwent emergency laparotomy and small bowel resection in the setting of fetal death in utero. This case highlights several challenging issues in diagnosing and managing this uncommon condition which leads to increased adverse perinatal outcomes. Prompt investigation and definitive surgical treatment are required when pregnant women present with bilious vomiting and new-onset abdominal or back pain especially beyond the first trimester.

The purpose of this study was to describe long-term outcomes of pediatric-onset ultrashort bowel syndrome owing to midgut volvulus managed at an interdisciplinary intestinal rehabilitation center.Patients with a history of malrotation and pediatric-onset midgut volvulus causing extensive bowel loss (20% residual small bowel length expected for postconception age) and treated between 2010 and 2017 were reviewed. Data are expressed as median (IQR).Twenty-three patients had midgut volvulus at age 1 (0-21) day leading to 9 (8-12) percent predicted residual bowel length. Eight (35%) had gastroschisis. Follow-up was 8.5 (6.6-12.2) years from volvulus. Five (22%) patients underwent intestinal/multivisceral transplantation, and all achieved enteral autonomy. Eighteen (78%) patients remained transplant-free, 7 of whom achieved enteral autonomy after 718 (682-1030) days of parenteral nutrition. Transplant-free enteral autonomy was achieved by 0/6 patients with gastroschisis, compared to 7/12 without gastroschisis (p = 0.04). For the overall group, 18 (78%) patients had small bowel bacterial overgrowth, and 7 manifested symptomatic D-lactic acidosis. We observed 2 mortalities, one awaiting transplant and one 4 years following transplantation.Midgut volvulus owing to malrotation with extensive bowel loss is associated with favorable long-term survival. Transplant-free enteral autonomy may be feasible, particularly in the absence of gastroschisis.Prognosis study.IIb, retrospective cohort study.

Malrotation classically presents as a full-term infant with bilious emesis. Any patient who presents with bilious emesis should be assumed to have malrotation with a midgut volvulus until proven otherwise. Initial workup should include a physical examination to rule out peritonitis or hemodynamic instability, followed by an abdominal X-ray to evaluate for proximal or distal bowel obstruction or a surgical emergency, such as pneumatosis or free air. Any patient with a worrisome physical exam or abdominal X-ray findings should be taken to the operating room (OR) emergently to rule out malrotation with midgut volvulus. The current gold standard in diagnosing malrotation is an upper gastrointestinal contrast study, and the key feature to look for is the location of the duodenal-jejunal junction (DJJ). In patients with an abnormal DJJ position, the treatment is a Ladd’s procedure. This chapter presents an algorithm that discusses the work-up and management of a patient with malrotation.

Intestinal malrotation is a developmental anomaly characterized by a midgut fixation disorder and failure to complete its normal fetal rotation around the superior mesenteric artery inside the peritoneal cavity. Malrotation is a rare cause of mechanical intestinal obstruction in adults. It may manifest as an emergency condition or with chronic abdominal symptoms. Although its diagnosis remains difficult, early diagnosis and treatment usually yield favorable outcomes. Intestinal malrotation rarely has an asymptomatic course, in which the diagnosis is usually made incidentally. Multislice computed tomography is quite helpful for making a correct diagnosis. In this study, we reported a 56-year-old patient incidentally who was detected to have intestinal malrotation and was managed symptomatically after presenting at our clinic with signs and symptoms of intestinal obstruction; we also provided a discussion of the relevant literature.

Antenatal midgut volvulus is a rare surgical emergency in which bowel is severely compromised. Rarely the etiology is a mesenteric defect. Early diagnosis is essential and lifesaving in the immediate newborn period. Typically upper gastrointestinal or ultrasound imaging can be suggestive of the diagnosis of volvulus in the neonate. Sometimes, however, the diagnosis may be elusive. Herein, we report on the use of neonatal magnetic resonance imaging to diagnose a midgut volvulus that occurred through a congenital mesenteric defect.

Intestinal malrotation complicated by the development of midgut volvulus presents a difficult management dilemma because of the risk of short bowel syndrome. Here, we present our experience with a case of severe intestinal ischemia after derotation in a newborn successfully managed using systemic tissue plasminogen activator. The present report supports the usefulness of thrombolysis as a therapeutic option for reperfusion of ischemic small bowel due to midgut volvulus in neonates.

To evaluate whether prenatal diagnosis of intestinal midgut volvulus (a rare condition due to the small bowel loops twisting) can improve the prognosis of the newborns.In our Prenatal Diagnosis Center, eight cases of intestinal volvulus observed between 2007 and 2014 were retrospectively considered. Ultrasonographic signs can be direct and specific (whirlpool sign, coffee bean sign) or indirect and non-specific (abdominal mass, dilated bowel loops, pseudocysts, ascites, polyhydramnios).Prenatal diagnosis was performed at 20-34 weeks of gestation. All newborns were exposed to an emergency surgery: the major complication was due to cystic fibrosis.An early suspicion of intestinal volvulus allows the clinician to refer the patient to a tertiary center so to confirm the diagnosis and perform an appropriate follow-up in order to identify the proper time of delivery. The prognosis of the babies with prenatal intestinal volvulus depends on the length of the segment involved, on the level of intestinal obstruction, on the presence of meconium peritonitis and on the gestational age at birth. Our experience, according with the literature, suggests that ascites and absence of abdominal peristalsis are ultrasonographic signs that, in the third trimester of pregnancy, correctly lead to an immediate delivery intervention.