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Objectives: Abnormal relative positioning of the superior mesenteric artery (SMA) and vein (SMV) can lead to intestinal malrotation that predisposes to midgut volvulus. The aim of this study was to assess the prenatal ultrasound ability to visualize the relative position of SMA and SMV in normal pregnancies. Methods: Prospective cohort study performed in Montpellier University Hospital Centre, including 80 fetuses during routine 3rd trimester ultrasound scan. For each fetus included, the relative position of the vessels on an axial image was defined as SMV on the right, forward, or on the left of SMA. Doppler imaging was additionally used if necessary. Data were compared to the neonatal abdominal scans performed by pediatric radiologist. Results: The superior mesenteric vessels were identified in 79 fetuses. Prenatal findings showed a usual relative position of the vessels, that is, the vein on the right of the artery, in 96.2%. In 2 cases, the vein was strictly in front of the artery, and in 1 case, the vein was on the left side of the artery. Seventy‐four neonates were examined and comparison with prenatal finding showed a perfect agreement (Kappa coefficient of 100%). An intestinal malrotation was postnatally diagnosed corresponding to the case where vein was on the left side of the artery. Conclusion: This study showed that the relative position of the SMA and SMV could be assessed using ultrasound prenatal examination with a perfect agreement with postnatal findings. In case of abnormal vessels positioning more examinations should be promote including prenatal MRI and postnatal conventional radiologic examinations to confirm intestinal malrotation. [ABSTRACT FROM AUTHOR]

Managing colon cancer with intestinal nonrotation, a type of congenital intestinal malrotation, is challenging due to the presence of anatomical abnormalities and severe adhesions. When patients have nonrotation, it is markedly more difficult to determine which vessels correspond to the colic vessels and ileal vessels until all vascular branching patterns become evident. The optimal approach for right‐sided colon cancer with intestinal nonrotation has yet to be established. In the present case of ascending colon cancer with intestinal nonrotation, we performed laparoscopic right hemicolectomy with D3 dissection using a modified cranial approach. This approach involves tracing, without resecting, branches from the superior mesenteric vein and superior mesenteric artery in a cranial‐to‐caudal manner until the ileocolic artery and ileocolic vein, which course toward the cecum, are identified, followed by the dissection of the colic vessels and lymph nodes in a caudal‐to‐cranial fashion. [ABSTRACT FROM AUTHOR]

ABSTRACT Pneumatosis cystoides intestinalis can present with concurrent diaphragmatic cysts, a previously undocumented phenomenon. Surgical management may be required, but further investigation is needed to understand the pathogenesis and optimize management in atypical and chronic cases, such as this case with a history of corrected intestinal malrotation.

Congenital diarrhea and enteropathies (CODEs) are a rare heterogeneous group of inherited disorders that typically present with severe chronic diarrhea during the first weeks of life. As a broad range of illnesses can present similarly in infants, establishing a definitive cause for CODEs is challenging. In this report, two infants were suspected to have CODE, with neonatal-onset chronic diarrhea and protein-losing enteropathy finally found to be due to intestinal malrotation and chronic midgut volvulus. Although the disease onset time was during the neonatal period, the typical findings of intestinal malrotation and volvulus were not present. Following the diagnostic approach for CODEs, both patients underwent extensive examinations without a definitive diagnosis. Intestinal malrotation was incidentally detected by an abdominal CT examination. After surgical correction of the underlying malrotation (Ladd's procedure), both infants had resolution of their diarrhea and hypoalbuminemia.

Mid gut volvulus is a rare clinical presentation in adults though it frequently occurs in infants and children. We report a case of 30 years male (post renal transplant patient) who presented with symptoms of abdominal pain, nausea and vomiting for 3 days. After demonstration of twisting of bowel loops around mesenteric vessels on Ultrasonography (USG). Contrast enhanced computed tomography (CECT) abdomen was done which showed small bowel malrotation, inverted relation of Superior mesenteric artery-Superior mesenteric vein and whirl pool sign of midgut volvulus. However, no significant bowel obstruction was noted. Patient underwent explorative laparotomy with ladd's procedure. Post-operatively, it was uneventful. Patient became symptoms free and was discharged after 5 days of surgery. Intestinal malrotation is a congenital abnormality that arises from disrupting the 270◦ counterclockwise midgut rotation during the embryonic period. In our case malrotation was predisposing factor for volvulus. CECT abdomen is the imaging procedure of choice in adults. Ladd's procedure is the treatment of choice in symptomatic patients though it remains controversial in asymptomatic patients. Though midgut volvulus is rare in adults, it should be kept as differential in adults with symptoms of intestinal obstruction and workup should be done accordingly.

Background: Intestinal malrotation is a condition in which the process of counterclockwise rotation and fixation to the peritoneum and retroperitoneum during fetal life is incomplete. In adults, it is generally asymptomatic and is often discovered incidentally. We report a case of laparoscopic partial resection of the descending colon for a tumor of the descending colon with a rare form of intestinal malrotation in which the inferior mesenteric artery ran symmetrically and the sigmoid colon was fixed to the dorsal cecum and right-sided retroperitoneum. Case presentation: A 75-year-old man was referred to our department of internal medicine due to a positive fecal occult blood test. Lower endoscopy revealed a laterally spreading tumor in the descending colon, and endoscopic submucosal dissection was attempted; however, this procedure was difficult, and the patient was referred to our department for surgical treatment. Contrast-enhanced computed tomography revealed that the endoscopic clip was located in the descending colon on the right side, the inferior mesenteric artery was symmetrical, and the sigmoid colon was located on both the right and dorsal sides of the cecum. Laparoscopic ileocecum and sigmoid colon mobilization was performed from the left side of the patient. After the completion of sigmoid colon mobilization, which returned the sigmoid colon and descending colon to anatomical normalcy, laparoscopic partial resection of the descending colon was performed. Based on the results of a histopathological examination, a granular type of laterally spreading tumor was diagnosed. The patient was discharged uneventfully on postoperative day 8. Conclusions: Detailed preoperative imaging and surgical simulation are necessary for abdominal surgery involving intestinal malrotation. [ABSTRACT FROM AUTHOR]

Introduction Intestinal malrotation (IM) is characterized by abnormal intestinal rotation and fixation predisposing to a risk of midgut volvulus. The aim of this study was to describe the clinical presentation and outcome of IM from birth through childhood. Materials and Methods This was a retrospective study of children with IM managed at a single center between 1983 and 2016. Data were retrieved from medical records and analyzed. Results Three hundred nineteen patients were eligible for the study. Using strict inclusion and exclusion criteria, 138 children were included. Vomiting was the most common symptom up to 5 years of age. At 6 to 15 years of age, abdominal pain was the predominant symptom. One hundred twenty-five patients underwent a Ladd's procedure and of 124 patients with available data, 20% had a postoperative complication (Clavien-Dindo IIIb-V) within 30 days. The odds ratio to develop postoperative complications was significantly increased in extremely preterm patients (p = 0.001) and in patients with severely affected intestinal circulation (p = 0.006). Two patients had intestinal failure due to midgut loss after midgut volvulus, one of whom needed intestinal transplantation. Four patients, all extremely preterm, died related to the surgical procedure. In addition, seven patients died of reasons not related to IM. Fourteen patients (11%) had adhesive bowel obstruction and one patient had recurrent midgut volvulus requiring surgical treatment. Conclusions IM presents with different symptoms through childhood depending on age. Postoperative complications are common after Ladd's procedure, particularly among extremely preterm infants and patients with severely affected circulation caused by midgut volvulus. [ABSTRACT FROM AUTHOR]

This literature review aims to acquaint readers with current radiological diagnostic methods utilized in detecting intestinal malrotation in newborns. The review incorporates 28 sources published from 2001 to 2021, examining radiological diagnostic methods both individually and in combination. Article selection was conducted from open-access scientific medical journals and references cited in the primary sources. The review encompasses studies highlighting the advantages and disadvantages of various techniques, with a specific focus on the potential substitution of the radiological method with ultrasound. Despite the benefits and well-established application techniques of the latter, based on the opinions of different authors, it can be concluded that a complete abandonment of the radiological method involving contrast agents is not currently feasible.

A paraduodenal hernia is a rare cause of an internal hernia that may require massive bowel resection; prompt diagnosis and surgical treatment are essential. In cases of malrotation, strangulation may occur both inside and outside the hernial sac. Strangulation outside the hernial sac makes the preoperative diagnosis more difficult. Herein, we report a patient with a right paraduodenal hernia, intestinal malrotation, and strangulation outside the hernia. An 86-year-old woman was admitted to our hospital with abdominal pain. Enhanced computed tomography showed a closed-loop obstruction of the hypo-enhancing small bowel and absence of a horizontal duodenal leg. The patient underwent an emergency laparotomy and was diagnosed with strangulated bowel obstruction due to a right paraduodenal hernia and malrotation. The patient underwent resection of the ischemic ileum, closure of the hernial orifice, and repositioning of the intestine. The postoperative course was uneventful. The patient reported no abdominal discomfort after 7 months of follow-up. [ABSTRACT FROM AUTHOR]

Background/Aim: Intestinal malrotation (IM) often remains undetected until adulthood, being discovered during testing or surgery for other comorbidities. Preoperative understanding of this anatomical abnormality is crucial. Case Report: An 80-year-old woman presented with cecal cancer. Three-dimensional computed tomography (CT) revealed that the cecum was located at the midline of the abdominal cavity, the duodenum did not cross the midline, and the ileocolic vein ran to the left. Clinically diagnosed with stage IVc cecal cancer complicated by IM, the patient underwent laparoscopic surgery. The ascending colon and cecum were not fixed to the retroperitoneum. The duodenum lacked the second, third, and fourth portions and the small bowel was distributed on the left and right sides of the abdominal cavity. Adhesions had shortened the mesentery, which were released close to their normal positions. Conclusion: Although laparoscopic surgery is superior to open surgery in terms of securing the field of view in a narrow space, providing a magnifying effect, and minimal invasiveness, it has a limited field of view and is inferior in terms of grasping the overall anatomy, which may be disadvantageous in cases of anatomical abnormalities. Colorectal cancer with IM is rare; however, the rate of preoperative diagnosis seems to be increasing thanks to improvements in diagnostic imaging, such as three-dimensional CT scans. In this study, we also reviewed 49 cases of colorectal cancer associated with IM. [ABSTRACT FROM AUTHOR]

Most cases of intestinal malrotation appear in neonates with bilious vomiting due to midgut volvulus, whereas in cases that develop beyond infancy, the initial symptoms vary. This study investigated the clinical features of these two populations and identified issues that should be considered in daily practice. A retrospective chart review was conducted from January 1, 2010, to December 31, 2022. Data on patients with intestinal malrotation were collected in an anonymized fashion from five pediatric surgical hub facilities in the Southern Kyushu and Okinawa areas of Japan. Of the 80 subjects, 57 (71.3%) were neonates (Group N) and 23 (28.7%) were infants and schoolchildren (Group I). The frequencies of initial symptoms, such as abdominal distention (Group N: 19.3% vs. Group I: 13.0%), bilious vomiting (59.6% vs. 43.5%), and hematochezia (8.8% vs. 21.7%), were not skewed by the age of onset (p = 0.535, 0.087, and 0.141, respectively). Midgut volvulus was significantly more frequent in Group N (71.9% [41/57] vs. 34.8% [8/23]; p = 0.005), while the degree of torsion was greater in group I (median 360° [interquartile range: 180–360°] vs. 450° [360–540°]; p = 0.029). Although the bowel resection rate was equivalent (7.0% [4/57] vs. 4.3% [1/23]; p = 1.000), half of the patients in Group N presented with 180° torsion. The neonatal intestine has been highlighted as being more susceptible to ischemia than that in older children. The incidence of midgut volvulus is higher in neonates than in older children. Even relatively mild torsion can cause ischemic bowel changes during the neonatal period. LEVEL III. [ABSTRACT FROM AUTHOR]

ABSTRACT: A congenital defect of the fetal intestinal rotation, known as malrotation of the gut, is most often identified as an acute intestinal occlusion in early infancy. Adults with this disease tend to be asymptomatic and extremely rare. We present a case of a 47-year-old woman with duodenal obstruction due to Ladd's band admitted with acute abdominal pain and vomiting.

BackgroundIntestinal malrotation is a rare condition, and its delayed diagnosis can lead to fatal consequences. This study aimed to investigate the identification and treatment of malrotation in children.MethodsClinical data, imaging, operative findings, and early postoperative outcomes of 75 children with malrotation were retrospectively analyzed.ResultsThe mean age was 6.18 ± 4.93 days and 51.26 ± 70.13 months in the neonatal group (56 patients) and non-neonatal group (19 patients), respectively. Sixty-seven patients were under the age of 1 year at the time of diagnosis. The occurrence of bilious vomiting and jaundice was significantly higher in the neonatal group (89.29%) than that in the non-neonatal group (37.5%), p 10% of neonates and nearly 40% of non-neonates. This highlights the importance for emergency physicians and surgeons to be cautious about ruling out malrotation in patients with non-bilious vomiting. Utilizing US can obviate the need for contrast examinations owing to its higher diagnostic accuracy and rapid diagnosis and can be recommended as a first-line imaging technique. Additionally, open surgery is still an option for neonatal patients.

Purpose: Midgut volvulus is an urgent disease often occurring in neonates. This study clarified the clinical features of midgut volvulus and evaluated predictors to avoid bowel resection. Methods: This bi-center retrospective study enrolled 48 patients who underwent surgery for intestinal malrotation between 2010 and 2022. Patients’ background characteristics and preoperative imaging findings were reviewed. Results: Midgut volvulus was recognized in 32 patients (66.7%), and 6 (12.5%) underwent bowel resection. Based on a receiver operating curve analysis of bowel resection, the cut-off value of the body weight at birth and at operation were 1984 g [area under the curve (AUC) 0.75, 95% confidence interval (CI) 0.52–0.99] and 2418 g (AUC 0.70, 95% CI 0.46–0.94), respectively. The cut-off value of intestinal torsion was 540° (AUC 0.76, 95% CI 0.57–0.95), and that of the time from the onset to the diagnosis was 12 h (AUC 0.85, 95% CI 0.72–0.98). For midgut volvulus with an intestinal torsion > 540°, the most sensitive preoperative imaging test was ultrasonography (75%) Patients with bloody stool tended to undergo bowel resection. Conclusions: Patients with a low body weight and bloody stool should be confirmed to have whirlpool sign by ultrasonography and scheduled for surgery as soon as possible. [ABSTRACT FROM AUTHOR]

Abstract Background Intestinal duplication and ectopic pancreas are two rare independent congenital anomalies. Few reports describe cases of patients with ectopic pancreas in an intestinal duplication causing acute peritonitis. Case presentation A 31-year-old man was admitted to the hospital for epigastric pain. The patient was diagnosed with acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication, with intestinal malrotation. The patient underwent the partial resection of the jejunum and Ladd’s procedure. The histopathological findings indicated ectopic pancreatitis in the jejunal duplication. Conclusions We presented the case of acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication in an adult with intestinal malrotation. Surgery is the primary treatment and is necessary for a definitive diagnosis.

Ladd's band malrotations are a subtype of embryogenesis abnormalities consisting of fibrous prolongations resulting from anomalous fixation of the mesentery. They extend from a malrotated cecum towards the retroperitoneum and can produce extrinsic compression of the duodenum. In 90% of cases, the clinical presentation occurs within the first year of life as an acute condition consisting of duodenal occlusion or small bowel volvulus with consequent small bowel ischemia or internal hernia. In adulthood, the presentations are less specific. The gold standard methods used for diagnosis are gastroduodenal series and computed tomography. Surgical treatment consists of Ladd's surgery, whose conventional approach was described in 1936 by William Ladd. We present the case of an adult patient with an occlusive condition caused by this anomaly, diagnosed promptly and safely resolved laparoscopically.

Intestinal malrotation and duplication of the inferior vena cava are rarely diagnosed in adult patients; however, incidence is likely underestimated as they are usually asymptomatic. These congenital malformations have been previously reported in the same patient twice but never with colonic obstruction or ischaemia. A 25-year-old female presented with nausea, vomiting, obstipation, and abdominal pain, and on computed tomography of the abdomen and pelvis was diagnosed with a caecal volvulus and pneumatosis coli associated with intestinal malrotation requiring emergency right hemicolectomy. Incidentally, the patient was noted to have duplication of the inferior vena cava, azygos continuation of the inferior vena cava, and splenic fragmentation. This constellation of symptoms has not been reported in the literature previously. The pattern of malformations follows that of polysplenia syndrome. Although rare, awareness of these malformations can be useful to clinicians. [ABSTRACT FROM AUTHOR]

Background: It is not often written in medical journals that preduodenal portal vein, biliary atresia, intestinal malrotation, and situs inversus totalis are all related. This is a rare association. Case reports: A 2-month-old female infant had biliary atresia type III, situs inversus totalis, midgut malrotation, and a preduodenal portal vein. She had been operated on by the Kasai procedure (hepato-portoenterostomy). Discussion: It is important to carefully look into the relationship between the preduodenal portal vein and biliary atresia because the patient is at risk of injury from this aberrant vein during operative intervention. Conclusion: The relationship between biliary atresia and other congenital anomalies like preduodenal portal vein, intestinal malrotation, and situs inversus must be taken into consideration to avoid other diverse effects during surgical intervention. [ABSTRACT FROM AUTHOR]

Introduction. Intestinal malrotation is a congenital pathology with potentially catastrophic complications, such as volvulus, whose treatment has barely not changed in nearly 100 years (Ladd's procedure). Dr. Abu-Elmagd recently described a new technique that was applied in one of our patients. Clinical case. 12-year-old boy who had undergone Ladd's procedure as a result of intestinal volvulus secondary to malrotation when he was 2 days old. He had subocclusion and eventually obstruction, with intestinal volvulus compatible imaging. Intraoperative findings: duodenal subocclusion, volvulus and lymphangiectasias. Kareem's procedure: bowel positioning in normal rotation, duodenopexy (duodenal C posterior to the mesenteric vessels), formation of neo-Treitz, and fixation of the cecum, the ascending colon, and the mesenteric root. The patient was discharged on postoperative day 6 and remains asymptomatic after 1 year of follow-up. Discussion. Kareem's procedure is a safe and effective malrotation repair technique. It can replace Ladd's procedure as it reduces the risk of re-volvulation and improves digestive symptoms. [ABSTRACT FROM AUTHOR]

Aim: Demand for upper gastrointestinal contrast series (UGI) to investigate bilious vomiting (BV) has increased in recent years, mostly due to greater awareness of the need to rule out malrotation and midgut volvulus (MGV). We aimed to examine predictive value of clinical parameters in the management of healthy neonates presenting with BV and re‐assess the role of UGI in their management. Methods: A retrospective cohort study including medical, imaging and surgical data of neonates who underwent UGI due to BV. Results: A total of 157 term neonates, eight neonates (5.1%) had confirmed surgical diagnosis of malrotation, five of them had malrotation with MGV, including two neonates who underwent extensive intestinal resection due to necrosis. Neonates with a combination of abnormal plain radiograph and abdominal distention had 10 times higher odds of malrotation diagnosis, adjusting for age at first BV (p = 0.017). Neonates with a combination of abnormal plain radiograph, abdominal distention and abdominal tenderness had 25 times higher odds of MGV (p = 0.002). Conclusion: This study reaffirms the role of UGI as the current main diagnostic tool for malrotation and MGV. Physical examination and plain radiograph findings can help but cannot substitute UGI study. [ABSTRACT FROM AUTHOR]

Abstract Background Intestinal malrotation is a rare congenital condition with potentially devastating consequences due to potential volvulus and massive intestinal necrosis. Diagnosis is often delayed and long-term symptoms following surgical correction are poorly characterized. We developed the Intestinal Malrotation Patient Outcomes and WEllness Registry (IMPOWER), a national patient-generated registry (PGR), to capture data related to presenting symptoms, testing, diagnosis, treatment, and follow-up of individuals diagnosed with malrotation. IMPOWER captures patient-reported information from adult patients and parents/caregivers of children diagnosed with malrotation at the time of enrollment and at ongoing 6-month intervals. We present baseline characteristics of patients enrolled during the first two months of the registry. Results Within the first two months, 354 patients with malrotation enrolled in IMPOWER, and 191 (53.9%) completed all baseline assessments. Nearly 90% of the 119 pediatric participants and 37.7% of the 72 adult participants experienced symptoms prior to diagnosis. Vomiting was the predominant symptom for pediatric participants compared to abdominal pain in adults. Yellow bilious emesis was more commonly reported than green, and volvulus at diagnosis occurred in 70% of pediatric and 27% of adult participants. One-third of pediatric participants had a bowel resection as part of their initial surgical procedure, resulting in 23.4% with diagnosed short bowel syndrome. More than 60% of pediatric and 80% of adult registrants reported gastrointestinal symptoms that persisted throughout the first year following their initial operation. Approximately 25% of registrants reported visiting four or more gastroenterologists for management of ongoing symptoms. Conclusions Fewer than half of pediatric patients presented with the “classic” presentation of green bilious colored emesis. Yellow bilious emesis was more commonly reported, and chronic gastrointestinal symptoms (i.e., abdominal pain, reflux, constipation, diarrhea) and feeding intolerance were common following surgical procedures for malrotation. This novel PGR highlights the need for a multicenter prospective registry to characterize the natural history and develop consistent standards of care related to the diagnosis, treatment, and long-term care for patients with malrotation.

Background: Malrotation commonly presents in neonates with bilious vomiting. It may also present in post neonatal age group with vague symptoms such as chronic abdominal pain, vomiting, and failure to thrive. These indistinct symptoms also result in delay of diagnosis. Aims and Objectives: This study is done with an objective to know whether age of presentation in malrotation determines morbidity and mortality. Materials and Methods: This is a retrospective study conducted in Gandhi medical college and Hospital, Hyderabad, from 2017 to 2020, based on the records of 50 patients who were diagnosed to have malrotation. The differences in clinical presentations, diagnosis, management, and outcomes were analyzed with respect to age. Results: Children who presented in neonatal period had classical symptoms (P1 month of age) had higher incidence of atypical symptoms (P

Background: Intestinal duplication and ectopic pancreas are two rare independent congenital anomalies. Few reports describe cases of patients with ectopic pancreas in an intestinal duplication causing acute peritonitis. Case presentation: A 31-year-old man was admitted to the hospital for epigastric pain. The patient was diagnosed with acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication, with intestinal malrotation. The patient underwent the partial resection of the jejunum and Ladd's procedure. The histopathological findings indicated ectopic pancreatitis in the jejunal duplication. Conclusions: We presented the case of acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication in an adult with intestinal malrotation. Surgery is the primary treatment and is necessary for a definitive diagnosis. [ABSTRACT FROM AUTHOR]

Unknown intestinal rotation anomaly poses a challenge in laparoscopic gastric bypass surgery. We present a case of a patient with intestinal non-rotation that stayed undetected throughout performing a laparoscopic Roux-en-Y gastric bypass. As a result, the alimentary limb was constructed in an anti-peristaltic way, and the whole gastric bypass was located far more distally than usual. Postoperatively, the patient presented with recurring nausea and vomiting. After several diagnostic steps, a computed tomography finally revealed the inadvertently reverse directed gastric bypass and the pre-existing condition of intestinal non-rotation. This was followed by a diagnostic laparoscopy and the reconstruction of the gastric bypass in "mirrored" technique. [ABSTRACT FROM AUTHOR]

Introduction: Reversed rotation is an extremely rare congenital anomaly that is often associated with intestinal obstruction or midgut volvulus. Although reverse rotation is classified into four types embryologically, there has been no comprehensive review of the mechanisms of the symptoms, imaging findings, or surgical management. Case presentation: A newborn with repeated non-bilious vomiting was admitted to our hospital because of a change to bilious vomiting. Abdominal ultrasonography revealed that the duodenum was in front of the superior mesenteric vessels. An upper gastrointestinal series demonstrated bowel obstruction. Intestinal malrotation was suspected, and surgery was emergently performed. The operative findings revealed reversed intestinal rotation (retroarterial-type). Adhesiolysis and relocation of the small intestine and colon were performed using the Ladd procedure. No recurrence was observed during 2 years of follow-up. Conclusion: A review of the relevant literature on reversed rotation revealed that the retroarterial type was the most common type and that the Ladd procedure was appropriate. Due to the rarity of the condition, the characteristic imaging findings based on the embryological background should be kept in mind to facilitate an adequate diagnosis and treatment in cases of intestinal reversed rotation.

Introduction: The vesico-allantoic cyst is a communication between the fetal bladder and the allantois through a patent urachus. Case Report: We describe a 17-week of gestational age (WGA) fetus with a 40 x 30 mm vesico-allantoic cyst. At 19 WGA, ultrasound (US) detected bilateral dilatation of renal pelvis (5-6 mm), hydroureters, and hypospadias. Amniotic fluid, umbilical cord flow, and fetal biometry were regular. Due to uncertain prognosis, the parents opted for legal termination of pregnancy. Autopsy confirmed the prenatal findings, also revealing intestinal malrotation and Meckel's diverticulum. Discussion/Conclusion: Probably an initial urinary tract obstruction occurred, not yet affecting the amniotic fluid volume, but evident as pyelectasis. This case highlights the possibility that genito-urinary and intestinal anomalies may be found in association with the vesico-allantoic cyst. [ABSTRACT FROM AUTHOR]

Intestinal malrotation may be asymptomatic in patients with heterotaxy syndrome (HS), and whether these newborns benefit from prophylactic Ladd procedures is unknown. This study sought to uncover nationwide outcomes of newborns with HS receiving Ladd procedures. Newborns with malrotation were identified from the Nationwide Readmission Database (2010-2014) and stratified into those with and without HS utilizing ICD-9CM codes for situs inversus (759.3), asplenia or polysplenia (759.0), and/or dextrocardia (746.87). Outcomes were analyzed using standard statistical tests. 4797 newborns with malrotation were identified, of which 16% had HS. Ladd procedures were performed in 70% overall and more common in those without heterotaxy (73% vs. 56% HS). Ladd procedures in newborns with heterotaxy were associated with higher complications compared to those without HS including surgical site reopening (8% vs. 1%), sepsis (9% vs. 2%), infections (19% vs. 11%), venous thrombosis (9% vs. 1%), and prolonged mechanical ventilation (39% vs. 22%), all p < 0.001. HS newborns were less frequently readmitted with bowel obstructions (0% vs. 4% without HS, p < 0.001) with no readmissions for volvulus in either group. Ladd procedures in newborns with heterotaxy were associated with increased complications and cost without differences in rates of volvulus and bowel obstruction on readmission. Retrospective Comparative. III. [ABSTRACT FROM AUTHOR]

Background: Intestinal malrotation is a congenitally acquired condition of abnormally rotated proximal small bowel in neonates and infants. Prompt recognition prevents lifethreatening complications. A structured approach to diagnosing malrotation at UGIS is required for accurate diagnosis. Objectives: Retrospective analysis of the images and radiological reports of UGIS, with the aim of identifying potential shortfalls in diagnosing malrotation. A secondary objective is to formulate a reporting template to improve overall quality of UGIS reports, specifically in cases of suspected malrotation. Method: Identification and retrospective review of UGIS studies which were subsequently re-read by a blinded consultant radiologist using the proposed reporting template adapted from the literature. Results: 367 UGIS studies between 1 January 2016 and 31 December 2021 were included in the study cohort, which were then re-read. Using McNemar's chi-square test, we found discrepancy between the number of studies positive for malrotation on the original reports versus the re-read studies, highlighting shortfalls in our current practise. Conclusion: A structured approach is paramount to the correct diagnosis of malrotation at UGIS. The position of the DJ-flexure (on frontal and lateral projections) proves most sensitive and specific in the diagnosis of malrotation at UGIS. Dedicated true lateral images were often found to be excluded in daily practise. We propose a structured inclusive reporting template. Contribution: Our proposed standardized reporting template aims to improve radiological, clinical, and surgical outcomes at UGIS, specifically in patients with suspected malrotation.

Introduction: Congenital diaphragmatic hernia (CDH) usually presents with respiratory distress at birth, which can be severe enough to cause cardiorespiratory failure. Late presentation of CDH is less common. CDH can be associated with malrotation, which is typically asymptomatic. Case presentation: We describe a case of a 7-year-old child who had non-specific gastrointestinal symptoms and no respiratory symptoms. The patient had been suffering from non-bilious vomiting for a week, had abdominal pain and was dehydrated and malnourished. Breath sounds were diminished and bowel sounds were present in the left hemithorax. A diagnosis of CDH was suggested on a chest x-ray and confirmed by a barium study. On exploration, a small-sized left CDH associated with malrotation and Ladd's bands were discovered. The diaphragmatic defect was closed primarily, and the malrotation corrected with a Ladd's procedure. The post-operative course was uneventful. Conclusion: Patients with non-specific gastrointestinal symptoms and a suspicious chest X-ray should undergo further imaging studies to rule out a CDH. Failure to do so can potentially lead to complications such as bowel obstruction and volvulus. Respiratory symptoms are uncommon in late-presenting CDH.

Background: Intestinal malrotation is a rare congenital condition with potentially devastating consequences due to potential volvulus and massive intestinal necrosis. Diagnosis is often delayed and long-term symptoms following surgical correction are poorly characterized. We developed the Intestinal Malrotation Patient Outcomes and WEllness Registry (IMPOWER), a national patient-generated registry (PGR), to capture data related to presenting symptoms, testing, diagnosis, treatment, and follow-up of individuals diagnosed with malrotation. IMPOWER captures patient-reported information from adult patients and parents/caregivers of children diagnosed with malrotation at the time of enrollment and at ongoing 6-month intervals. We present baseline characteristics of patients enrolled during the first two months of the registry. Results: Within the first two months, 354 patients with malrotation enrolled in IMPOWER, and 191 (53.9%) completed all baseline assessments. Nearly 90% of the 119 pediatric participants and 37.7% of the 72 adult participants experienced symptoms prior to diagnosis. Vomiting was the predominant symptom for pediatric participants compared to abdominal pain in adults. Yellow bilious emesis was more commonly reported than green, and volvulus at diagnosis occurred in 70% of pediatric and 27% of adult participants. One-third of pediatric participants had a bowel resection as part of their initial surgical procedure, resulting in 23.4% with diagnosed short bowel syndrome. More than 60% of pediatric and 80% of adult registrants reported gastrointestinal symptoms that persisted throughout the first year following their initial operation. Approximately 25% of registrants reported visiting four or more gastroenterologists for management of ongoing symptoms. Conclusions: Fewer than half of pediatric patients presented with the "classic" presentation of green bilious colored emesis. Yellow bilious emesis was more commonly reported, and chronic gastrointestinal symptoms (i.e., abdominal pain, reflux, constipation, diarrhea) and feeding intolerance were common following surgical procedures for malrotation. This novel PGR highlights the need for a multicenter prospective registry to characterize the natural history and develop consistent standards of care related to the diagnosis, treatment, and long-term care for patients with malrotation. [ABSTRACT FROM AUTHOR]

Background: Irritable bowel syndrome (IBS) is a complex disorder involving a variety of severe life-altering symptoms and yet-to-be-discovered biologic mechanisms. Patients with IBS are often referred to gastroenterologists who initiate a work-up to rule several potential confounding disease processes. This work-up often involves a consult with a general and/or vascular surgeon to determine if their IBS-like symptoms have an anatomic or surgical etiology. Potential concurrent problems may include the discovery of points or angulations at which gastrointestinal flow is impaired, median arcuate ligament syndrome/angulation, superior mesenteric syndrome/compression, the incidental finding of intestinal malrotation, and others. Once these diagnoses are entertained and/or documented in a patient's chart, "anchoring" on the diagnosis can lead to the belief that their IBS-like symptoms are due to a well-defined, operatively treatable anatomic condition. Methods: In this piece, we will breakdown the potential pitfalls in communicating, advising, and caring for patients with IBS-like symptoms presenting for surgical consultation. Results: Discussion points are offered with the goal of avoiding unnecessary operations even when faced with compelling anatomic evidence, a highly motivated patient, and a desperate and persuasive family. Conclusion: Patients suffering from IBS presenting for surgical consultation with a defined anastomotic abnormality that may or may not be related to their symptoms represent a major challenge for surgeons. A multidisciplinary team approach can be useful to avoid unindicated surgery in these patients. [ABSTRACT FROM AUTHOR]

Background: Intestinal obstruction in neonates is a common problem that requires prompt diagnosis and treatment, and ultrasound could be a potential tool for it. The purpose of this study was to investigate the accuracy of ultrasonography in diagnosing and identifying the cause of intestinal obstruction in neonates, the corresponding ultrasonic manifestations, as well as to utilize the diagnostic method. Methods: We conducted a retrospective study of all neonatal intestinal obstruction in our institute between 2009 and 2022. The accuracy of ultrasonography in the diagnosis of intestinal obstruction and the identification of its etiology was compared with the results of operation as the gold standard. Results: The accuracy of the ultrasonic diagnosis of intestinal obstruction was 91%, and the accuracy of the ultrasonic etiological diagnosis of intestinal obstruction was 84%. The main ultrasound findings for the neonatal intestinal obstruction were dilation and high tension of the proximal bowel and collapse of the distal intestinal. Other major manifestations were the presence of corresponding diseases causing intestinal obstruction at the junction of the dilated and collapsed bowel. Conclusions: Ultrasound has the advantages of being a flexible multi-section dynamic evaluation and a valuable tool to diagnose and identify the cause of intestinal obstruction in neonates. [ABSTRACT FROM AUTHOR]

Abstract Background Right-side fixation of the sigmoid colon is a rare anatomical variant associated with intestinal malrotation (Choi et al. in J Korean Surg Soc. 84(4):256–60, 2013). Differently from other forms of malrotation, this variant has not been associated thus far with acute surgical conditions. Case presentation In this report, we present a 65-year-old Caucasian patient admitted for bowel obstruction symptoms. Computed tomography scan revealed right-side fixation of the sigmoid colon extended to the subhepatic recess complicated by obstructed internal herniation of the ileum. In this patient, the sigmoid colon occupied a recess posterior to the ascending colon and right Toldt’s fascia. Within this narrow anatomical space, an ileal loop was trapped causing internal herniation with resultant close-bowel obstruction of both ileum and sigmoid colon. The ileal loop was released surgically and the anatomical abnormality corrected. Conclusions To our knowledge, this is the first case of right-side fixation of the sigmoid colon causing acute obstruction secondary to internal herniation of the small intestine. Early recognition and precise anatomical definition of such anatomical variants are essential to optimize their surgical approach.

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Intestinal malrotation (IM) is an abnormality due to a failure of the normal midgut rotation and fixation. We report a case of 46‐year‐old man with ulcerative colitis whose IM was apparent after laparoscopically total proctocolectomy (TPC) followed by ileal‐pouch‐anal anastomosis (IPAA) and ileostomy. There was no abnormal anatomy except for mobile cecum/ascending colon during the initial operation. Intestinal obstruction occurred after ileostomy closure. The computed tomography scan showed the duodeno‐jejunal transition was located in right abdomen, the superior mesenteric vein was located left of the superior mesenteric artery (SMA) and the obstruction point was the distal ileum near the pouch. We performed an ileo‐ileo bypass across the ventral side of the SMA to relieve the intestinal obstruction. The patient would have incomplete IM preoperatively, which became apparent by TPC. In case of TPC for mobile colon, anatomy of small intestine should be checked before IPAA. [ABSTRACT FROM AUTHOR]

Background: Malrotation commonly presents in neonates with bilious vomiting. It may also present in post neonatal age group with vague symptoms such as chronic abdominal pain, vomiting, and failure to thrive. These indistinct symptoms also result in delay of diagnosis. Aims and Objectives: This study is done with an objective to know whether age of presentation in malrotation determines morbidity and mortality. Materials and Methods: This is a retrospective study conducted in Gandhi medical college and Hospital, Hyderabad, from 2017 to 2020, based on the records of 50 patients who were diagnosed to have malrotation. The differences in clinical presentations, diagnosis, management, and outcomes were analyzed with respect to age. Results: Children who presented in neonatal period had classical symptoms (P<0.00001) and early diagnosis (P<0.0239). Children who presented later (>1 month of age) had higher incidence of atypical symptoms (P<0.00001) and delay in diagnosis (P<0.0239) compared to neonatal age group. Plain X-ray abdomen is readily diagnostic in neonatal age group (P<0.0004) and diagnosis was confirmed by contrast study, whereas in older children, plain abdomen X-ray findings were not always distinct and most of them needed contrast series to reach definitive diagnosis. The incidence of bowel gangrene and mortality was not statistically significant (P value of 1) between neonatal and post neonatal groups. Conclusion: In post neonatal age group, lack of classical presentation may lead to difficulty in diagnosing malrotation, but may not have significant difference in terms of morbidity and mortality. Considering malrotation early in differential diagnosis leads to timely intervention even in older age group children. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to develop a predictive model using a random forest algorithm to determine the likelihood of postoperative adhesive small bowel obstruction (ASBO) in infants under 3 months with intestinal malrotation., Methods: A machine learning model was used to predict postoperative adhesive small bowel obstruction using comprehensive clinical data extracted from 107 patients with a follow-up of at least 24 months. The Boruta algorithm was used for selecting clinical features, and nested cross-validation tuned and selected hyper-parameters for the random forest model. The model's performance was validated with 1000 bootstrap samples and assessed using receiver operating characteristic (ROC) analysis, the area under the ROC curve (AUC), sensitivity, specificity, precision, and F1 score., Results: The random forest model demonstrated high diagnostic accuracy with an AUC of 0.960. Significant predictors of ASBO included pre-operative white blood cell count (pre-WBC), mechanical ventilation (MV) duration, surgery duration, and post-operative albumin levels (post-ALB). Partial dependence plots showed non-linear relationships and threshold effects for these variables. The model achieved high sensitivity (0.805) and specificity (0.952), along with excellent precision (0.809) and a robust F1 score (0.799), indicating balanced recall and precision performance., Conclusion: This study presents a machine learning model to accurately predict postoperative ASBO in infants with intestinal malrotation. Demonstrating high accuracy and robustness, this model shows great promise for enhancing clinical decision-making and patient outcomes in pediatric surgery., Competing Interests: Conflicts of interest The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2025 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Abstract Background Pancreaticobiliary maljunction is a congenital anatomical abnorma l junction of the pancreatic duct and bile duct into a common channel outside the duodenal wall. Pancreas divisum is also a congenital anatomical abnormality characterized by unfused pancreatic ducts. Intestinal malrotation is caused by the failure of bowel rotation and fixation. We reported an optimal surgical intervention for the rare case of pancreaticobiliary maljunction and pancreas divisum accompanied intestinal malrotation. Case presentation A 2-year-old female presented with fever and jaundice. Abdominal ultrasound showed dilated common bile duct and intrahepatic bile ducts; MRCP showed pancreaticobiliary maljunction, pancreas divisum, and dilated biliary system; Abdominal contrast-enhanced CT showed a reversed relationship between the superior mesenteric artery and the superior mesenteric vein. An operation of laparoscopic resection of the extrahepatic bile duct, Roux-en-Y hepaticojejunostomy, and Ladd’s procedure was performed after the inflammation of the biliary system was treated. The post-operative follow-up period was uneventful. Conclusions The management of pancreas divisum can be conservative. We present an optimal pattern of Roux-en-Y hepaticojejunostomy to deal with pancreaticobiliary maljunction associated with intestinal malrotation.

Abstract Background Congenital abnormalities are not very common and are even rarer when two or more are combined. Congenital malformation of the superior mesenteric vein may not affect normal development, or it may lead to moderate or even severe symptoms. In combination with intestinal malrotation, however, it may lead to the need for surgical intervention in the early years of life. Case presentation We present the case of a 22-year-old patient who had been diagnosed with iron deficiency anaemia at the age of two months. As a result of the absence of the proximal section of the superior mesenteric vein, the patient has always needed iron supplements and an occasional erythrocyte transfusion. This has resulted from the formation of collaterals throughout the small bowel, causing chronic blood loss with its clinical manifestation. Although, there are some congenital abnormalities of the superior mesenteric vein, the absence of the superior mesenteric vein is rare, and in this case the clinical course was quite severe. Therefore, we planned bypass surgery for this patient to reduce the duodenal collaterals and resolve the persistent anaemia caused by chronic blood loss from the duodenum. We successfully performed the surgery consisting of the formation of anastomosis between the large collateral vein from the distal end of the superior mesenteric vein and the anterior inferior pancreaticoduodenal vein. Conclusion The purpose of this case report is to describe the rare anatomical malformation of the superior mesenteric vein accompanied by intestinal malrotation, with its potential clinical implications regarding symptoms, clinical presentation, and the impact on potential surgery planning.

Abstract Background Laparoscopic Ladd’s procedure for malrotation in children is still a controversial approach. Although some retrospective studies have compared the outcomes of the two types of procedure with inconsistency outcomes. Currently, there are few large-scale studies on laparoscopic treatment in malrotation with neonates and infants. We did a study based on propensity score matching to compare the effects of the two kinds of approach in neonates and infants. To investigate the therapeutic effect of laparoscopy and open Ladd’s procedure by the propensity score-matching (PSM) to enhance the validity of the comparison. Methods A total series of 143 cases of intestinal malrotation without intestinal necrosis was included in the study during the 8 years from January 2012 to January 2020, including 68 cases of open Ladd’s procedure and 75 cases of laparoscopic Ladd’s procedure including five cases of transfer laparotomy. By a propensity score 1:1 matching, 62 patients were stratified for each group. Results and conclusion There was no significant difference in volvulus degree, weight and gender between the two groups (p > 0.05). Laparoscopic surgery took more time than open surgery (105.9 min vs 70.6 min, p 0.05). The cosmetic effect of laparoscopic surgery is better than that of open surgery. Laparoscopic Ladd’s procedure is a safe approach. It can reduce the length of hospital stay and incision infection, but the operation time was extended, the other complications are similar compared with open procedure for intestinal malrotation in neonates and infants.

Acute appendicitis is one of the most frequent causes of acute right lower quadrant abdominal pain requiring emergency surgical intervention. Clinical examination and routine blood tests are sufficient for a provisional diagnosis, with CT scan as the most sensitive modality for diagnostic confirmation. Rare instances of appendicitis presenting as epigastric or left quadrant pain in cases of undiagnosed intestinal malrotation particularly in adults may present as a diagnostic dilemma and may at times even progress to catastrophic outcome. We report a case of 39 years old male patient with undiagnosed intestinal malrotation presenting with a recent history of left lower quadrant pain where acute appendicitis and associated left isomerism and aberrant vascular anomalieswere ultimately grasped on the abdominal CT examination. [ABSTRACT FROM AUTHOR]

Intestinal malrotation is a congenital anomaly caused by incomplete rotation or absence of rotation of the primitive intestine along the axis of the upper mesenteric artery during embryonic development. Embryonic development and its anatomical variations were described by Dott in 1923. Intestinal malrotation is a rare condition among adults – prevalent in a mere 0.0001% to 0.19% of the population –, and it may be associated with other anatomical deformities. It can be asymptomatic or manifest with varying intensity, from obstruction to necrosis of intestinal segments. In general, this abnormality is diagnosed in the first year of life; however, symptoms may appear later in life, making diagnosis in adults difficult on account of non-specific symptoms. In the present study, we report a case of intestinal malrotation associated with chronic non-specific symptoms progressing to mesenteric angina.

Congenital intestinal malrotation and duodenal duplication cysts are congenital anomalies that are present during the neonatal period. The presence of both in a single patient is exceedingly rare with limited similar case reports present in the literature. We present the case of a neonate diagnosed with a right-sided ovarian cyst in utero, who was later found to have a concurrent duodenal duplication cyst and intestinal malrotation. Treatment consisted of open Ladd's procedure, resection of duplication cyst, and primary duodenoduodenostomy. The report serves as a data point for this rare phenomenon as well as provides surgical options for similar cases.

Abstract Background Bochdalek hernia is a common congenital diaphragmatic defect that usually manifests with cardiopulmonary insufficiency in neonates. It is very rare in adults, and symptomatic cases are mostly left-sided. Diaphragmatic defects generally warrant immediate surgical intervention to reduce the risk of incarceration or strangulation of the displaced viscera. Case presentation A 47-year-old woman presented with dyspnea on exertion. Computed tomography revealed that a large part of the intestinal loop with superior mesenteric vessels and the right kidney were displaced into the right thoracic cavity. Preoperative three-dimensional (3D) simulation software visualized detailed anatomy of displaced viscera and the precise location and size of the diaphragmatic defect. She underwent elective surgery after concomitant pulmonary hypertension was stabilized preoperatively. The laparotomic approach was adopted. Malformation of the liver and the presence of intestinal malrotation were confirmed during the operation. The distal part of the duodenum, jejunum, ileum, colon, and right kidney were reduced into the abdominal cavity consecutively. A large-sized oval defect was closed with monofilament polypropylene mesh. No complications occurred postoperatively. Conclusion Symptomatic right-sided Bochdalek hernia in adults is exceedingly rare and is frequently accompanied by various visceral anomalies. Accurate diagnosis and appropriate surgical repair are crucial to prevent possible incarceration or strangulation. The preoperative 3D simulation provided comprehensive information on anatomy and concomitant anomalies and helped surgeons plan the operation meticulously and perform procedures safely.

Pre-duodenal portal vein (PDPV) is a rare anomaly and a rare cause of duodenal obstruction (DO), with only a few cases reported in the literature. We present an infant whose bilious vomiting persisted despite having Ladd's procedure for intestinal malrotation due to a missed diagnosis of DO from PDPV that was found at re-exploration. The patient was diagnosed with malrotation and had Ladd's procedure at 12 weeks of age, but bilious vomiting persisted post-operatively. The patient presented to us after 4 weeks, was clinically malnourished and dehydrated, resuscitation was done and re-exploratory laparotomy performed, where an obstructing PDPV was found and a duodeno-duodenostomy was performed anterior to PDPV. However, the patient died on post-operative day 7 probably from severe malnutrition due to delayed diagnosis and absence of parenteral nutrition. We conclude that PDPV may be a cause of DO in infants with malrotation and should be properly sought for during Ladd's procedure for possible bypass surgery if found.