A Rare Case of Delayed Presentation of Acute Midgut Volvulus in an Adolescent.

Intestinal malrotation is primarily diagnosed in the neonatal period, when symptoms typically first appear. In contrast, occurrences in adults are quite uncommon. Adult cases are less frequently reported, often because the condition remains asymptomatic or presents with nonspecific symptoms that can delay diagnosis. Intestinal malrotation in adults can show a range of symptoms, from acute bowel obstruction to vague and chronic symptoms, often leading to a delay in the diagnosis compared to children. Patients with this condition present a distinctive challenge for surgeons regarding diagnosis and treatment, especially in acute situations. This study presents a rare case of a 16-year-old boy who came with chief complaints of acute abdominal pain and multiple episodes of bilious vomiting. The patient underwent an emergency laparoscopy converted to an open Ladd's procedure. During surgery, the duodenum, small intestine, cecum, and appendix were discovered to be abnormally positioned, and the transverse colon and mesentery were twisted along with the superior mesenteric artery and superior mesenteric vein, with the presence of classical Ladd's bands confirming preoperative CT findings of midgut volvulus. The patient tolerated the procedure well, with no intraoperative complications. Acute midgut volvulus is rare in adults and older children. Still, the differential diagnosis should be kept in mind in patients with pain in the abdomen and multiple episodes of bilious vomiting in cases with an uncertain diagnosis to plan proper management and avoid intraoperative surprises. Early detection, accurate imaging methods, and prompt intervention can mitigate complications that could increase morbidity and mortality.

Adult Intestinal Malrotation With Congenital Transverse Meso-Colic Internal Hernia: An Infrequent Cause of Small Bowel Obstruction.

Adult intestinal malrotation along with congenital transverse-mesocolic internal hernia causing small bowel obstruction is extremely rare. Most of these patients don't have any obvious clinical symptoms. Only a few cases have been documented in the English literature. We present the unique case of a 43-year-old male without any prior surgical history who presented with nonspecific abdominal pain and was diagnosed with malrotation of the small intestine by computed tomography (CT) scan and underwent exploratory laparotomy found to have internal herniation through the transverse-mesocolon. The patient underwent an emergency laparotomy; a Ladd's procedure and repair of the hernial orifice were performed. This case highlights the association of adult intestinal malrotation with internal hernias and small bowel obstruction; it also explores the importance of timely diagnosis and adequate management of this condition.

Severe Duodenal Bleeding with Heterotaxy Syndrome Controlled by Combined Hemostatic Strategies: A Case Report.

A 73-year-old man with a history of heterotaxy syndrome and intestinal malrotation developed hemorrhagic shock due to bleeding from a duodenal ulcer. Esophagogastroduodenoscopy failed to achieve hemostasis because of restriction of the view by massive hemorrhage. Transcatheter arterial embolization cannot achieve endovascular hemostasis due to the vascular anatomy. Therefore, surgical hemostasis was achieved. Intestinal malrotation due to heterotaxy syndrome is a risk factor for gastrointestinal bleeding. Severe gastrointestinal bleeding in patients with intestinal malrotation is difficult to control with endoscopy alone. Therefore, combined therapies with endoscopy, interventional radiology, surgical procedures, and intensive care are vital for achieving a better prognosis.

Modified cranial approach to right-sided colon cancer in a patient with intestinal nonrotation: A case report.

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.

Attempted one anastomosis gastric bypass converted to a sleeve gastrectomy in an adult patient with asymptomatic intestinal malrotation: A case report.

Unexpected encounters during surgery for obesity such as midgut malrotation cause specific technical challenges to the surgeon. We present a rare case of asymptomatic complete intestinal malrotation midway during a one anastomosis gastric bypass procedure. A 62-year-old male with a body mass index of 49 kg/m2 and metabolic syndrome was planned for one anastomosis gastric bypass. A gastric tube was created along the lesser curvature. During the attempt to identify the suitable small bowel loop, an unexpected completely malrotated gut was noted. Due to the intraoperative difficulty in identifying the correct loop to anastomose to the gastric tube an intraoperative decision was taken to convert the procedure to a sleeve gastrectomy. The created gastric tube was re-anastamosed to distal stomach, and the redundant stomach was resected. Postoperative recovery was uneventful, and weight loss was satisfactory. Attempted one anastomosis gastric bypass converted to a sleeve gastrectomy was a successful bailout procedure.

Gut Malrotation in a Human Cadaver: Exploration Into the Prevention and Screening of Undiagnosed Malrotation in Adults.

Malrotation is a congenital anomaly that results from the abnormal rotation of the gut during fetal development. Malrotation may be missed in early life and can present later with non-specific, chronic abdominal symptoms and decreased quality of life and in some cases can lead to serious bowel complications. Most adult cases are discovered incidentally on imaging or during surgery. An 82-year-old male cadaver was identified as having probable malrotation of the intestines. The performance of a previous surgical procedure could not be confirmed due to a lack of medical and surgical history. The cadaver dissection raised the question regarding the screening modalities used to reliably identify malrotations in infants and adults. Implementing a standardized reliable screening tool in infants or adults complaining of chronic abdominal pain could largely reduce the incidence of undiagnosed malrotation. Along with the development of a screening tool, increasing understanding of the clinical presentation of malrotation in adults could help identify undiagnosed cases earlier, which can reduce morbidity and mortality in these patients.

Intestinal malrotation in the adult population: Diagnosis, management, and outcomes after laparoscopic Ladd procedure.

BACKGROUND: The Ladd procedure is the treatment of choice for patients with intestinal malrotation; however, the long-term outcomes of the laparoscopic Ladd procedure are not well documented. This study aimed to review the presentation, management, and outcomes of adult patients who underwent a laparoscopic Ladd procedure. METHODS: A retrospective review was conducted to identify adult patients with malrotation who underwent a laparoscopic Ladd procedure between January 1995 and June 2022 at the Mayo Clinic in Rochester, Minnesota. Patient details and follow-up data were obtained from the electronic medical records. Patients were invited to participate in a structured phone interview to assess symptoms and quality of life (QoL). RESULTS: A total of 44 patients underwent the laparoscopic Ladd procedure. Of the 44 patients, 42 (95.5 %) were symptomatic, with 7 (16.7 %) presenting with acute symptoms. Moreover, 8 laparoscopic procedures (13.6 %) required conversion to an open procedure. The median estimated blood loss was 20 mL (IQR, 10-50), operative time was 2.3 h (IQR, 1.8-2.8), and hospital length of stay was 2 days (IQR, 2-3). Postoperative ileus was the most common complication (18.0 %). The median follow-up was 8.00 years (IQR, 2.25-13.00), with more than 90.0 % of patients having partial or complete symptom resolution. Of note, 28 patients (63.6 %) completed phone interviews. Moreover, 1 patient (2.0 %) reported a postoperative volvulus. When asked to compare their current symptoms with those preoperatively, 78.6 % of patients noted that they were significantly better. Furthermore, 85.7 % of patients reported that their QoL was significantly better after surgery. Finally, 96.4 % of patients would recommend the procedure to a friend or family member with the same condition. CONCLUSION: The laparoscopic Ladd procedure is a safe and effective surgical procedure for adult patients with intestinal malrotation.

Laparoscopic Colectomy for Cecal Cancer and Intestinal Malrotation: A Case Report.

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.

Midgut volvulus in an adult without significant intestinal obstruction: A case report.

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.

A rare case of extremely low birth weight infant with Beckwith-Wiedemann syndrome.

INTRODUCTION: Beckwith-Wiedemann syndrome (BWS) manifests distinctive features, such as macroglossia, overgrowth, and abdominal wall defects. In this report, we describe a case of BWS in an extremely low birth weight infant diagnosed at three months after birth because of the intensive care for low birth weight. PRESENTATION OF CASE: A female infant was delivered at 24 weeks and 6 days of gestation with a weight of 845 g. After birth, significant small intestinal intra-umbilical prolapse was observed, and abdominal wall closure using a sutureless method was performed on day zero. Careful neonatal management was performed; however, an episode of bloody stools led to a diagnosis of intestinal volvulus due to intestinal malrotation. At 119 days of age, the Ladd procedure was performed. Notably, during anaesthesia induction, features suggestive of BWS were observed, leading to its diagnosis. DISCUSSION: Early diagnosis of BWS is vital because of its association with tumors. However, because she was an extremely low birth weight infant who required oral intubation and supine management for respiratory control, nevus flammeus and macroglossia were not observed. Therefore, BWS was not diagnosed for approximately three months after birth. It is important to recognize that omphalocele in extremely low birth weight infants is a risk factor for delayed diagnosis of BWS. CONCLUSION: Timely diagnosis of BWS is critical because of its association with tumors and varied clinical presentations. Early screening, especially for tumors, and awareness among surgical practitioners can aid in timely interventions and improved patient outcomes.

Right paraduodenal hernia presenting with strangulated obstruction with intestinal malrotation: a case report.

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.

Identification and treatment of intestinal malrotation with midgut volvulus in childhood: a multicenter retrospective study.

Background: Intestinal 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. Methods: Clinical data, imaging, operative findings, and early postoperative outcomes of 75 children with malrotation were retrospectively analyzed. Results: The 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 < 0.05 and p < 0.01, respectively. The incidence of abnormal ultrasound (US) findings was 97.30% and 100%, respectively, and the sensitivities of the upper gastrointestinal series were 84.21% and 87.5%, respectively. Sixty-six (88%) patients had midgut volvulus, including in utero volvulus (two patients) and irreversible intestinal ischemia (four patients). Most neonates (89.29%) underwent open Ladd's procedure with a shorter operative time (p < 0.01). Reoperation was performed for postoperative complications (four patients) or missed comorbidities (two patients). Conclusions: Non-bilious vomiting was the initial symptom in >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.

An Unusual Coexistence: Right-Sided Colon Cancer and Intestinal Malrotation in an Adult.

Intestinal malrotation (IM), a rare congenital anomaly disrupting typical embryonic rotation around the superior mesenteric artery, is exceptionally uncommon in adults, with its link to colon cancer being even rarer. This article presents a case of colonic cancer in conjunction with IM in a 63-year-old male. Image studies and intraoperative findings show signs of IM. Open resection was performed due to concerns about vascular anomalies and abnormal lymphatic drainage. The case underscores the rarity of colon cancer in a malrotated gut, highlighting the necessity of preoperative identification for precise surgical planning and emphasizing the importance of careful dissection to prevent inadvertent vascular injury.

Adult intestinal malrotation presenting as caecal volvulus with incidental findings of duplicate inferior vena cava and other rare abnormalities: case report.

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.

Malrotation in an Adult Patient With Pneumoperitoneum Following Nissen-Sleeve Gastrectomy: A Case Report.

Intestinal malrotation is typically encountered in the first year of life and is rarely seen in adult populations. Herein, we present the case of a 48-year-old woman with a surgical history of laparoscopic Nissen-sleeve gastrectomy before 11 months who was referred to the general surgery service after presenting to the emergency department with acute epigastric abdominal pain for one-day duration. Radiography and a computed tomography (CT) scan of the abdomen revealed a large pneumoperitoneum. Subsequently, a diagnostic laparoscopy was performed, which detected a sealed perforation in the fundus of the wrapped-sleeved stomach, along with an incidental finding of intestinal malrotation. The encountered variation of anatomy created an intraoperative challenge during the conversion from Nissen-Sleeve gastrectomy to single anastomosis gastric bypass. The diagnosis of intestinal malrotation in adults is often overlooked, posing substantial diagnostic and management challenges when encountered.

Waugh syndrome: A rare coexistence of intussusception and intestinal malrotation: Case report and literature review.

INTRODUCTION: Waugh's syndrome, characterized by the concurrent presence of acute intussusception and gut malrotation, is a rare clinical entity. The relationship between these conditions is not well-explored, and the true incidence may be underreported. CASE PRESENTATION: A 4 month old male infant, with an uneventful medical history, presented to the pediatric emergency department due to irritability and rectal bleeding lasting one day. The infant had a history of bilious vomiting and dark bloody rectal discharge. Physical examination revealed a hypoactive, dehydrated child with a palpable mass in the left lower abdomen. Abdominal ultrasound indicated Colo-colonic intussusception, and attempts at hydrostatic reduction were unsuccessful. Subsequent CT scan revealed malrotation with ileo-Colo-rectal intussusception. The patient underwent a two-step operation, involving manual reduction and Ladd's procedure, with a successful recovery and no post-surgical complications. CLINICAL DISCUSSION: Reviewing the cases, we explore the unique features of Waugh's syndrome, its diverse age presentation, and the challenges in timely diagnosis. Diagnostic modalities, including abdominal ultrasound and contrast studies, are discussed, emphasizing the importance of recognizing malrotation in conjunction with intussusception for appropriate management. CONCLUSION: Our experience highlights the potential underrecognition of Waugh's syndrome and emphasizes the need for a high index of suspicion. The rarity of laparoscopic interventions in documented cases is noted, emphasizing the prevailing reliance on open surgical approaches. A call for prospective studies is made to determine the actual incidence of intussusception in intestinal malrotation cases and to enhance understanding for optimal patient management.

Diagnostic Challenges in Adult Intestinal Malrotation: A Case Report and Literature Review.

Adult intestinal malrotation is a rare anatomical anomaly that typically manifests during infancy but can also present in adulthood. Symptoms are mainly digestive, with a long history of intermittent abdominal pain and epigastralgia. It often presents a diagnostic challenge due to the varied and nonspecific nature of clinical symptoms. Radiological evaluations reveal diverse patterns of malrotation, including incomplete rotation, mesenteric base abnormalities, and associated malformations. Computed tomography (CT) scans consistently identify characteristic anatomical distortions, aiding in accurate diagnosis. In this context, we present a unique case in which contrast-enhanced CT of the abdomen, initially conducted to investigate a suspected episode of acute pancreatitis accompanied by epigastralgia, fortuitously revealed the presence of intestinal malrotation. Once the diagnosis has been made, the therapeutic approach is based on monitoring or managing complications such as intestinal obstruction. Early recognition and accurate radiological assessment of intestinal malrotation play an essential role in establishing the diagnosis and guiding appropriate management strategies. Increased awareness among clinicians and radiologists is essential to avoid delays in diagnosis and the potential complications associated with this entity.

Intestinal malrotation complicated with gastric cancer: A case report.

BACKGROUND: Intestinal malrotation is a congenital defect of embryonic development caused by various teratogenic factors. In this condition, the intestinal tube, along with the superior mesenteric artery serving as the axis for the counterclockwise movement, is incomplete or abnormally rotated due to incomplete attachment of the mesentery and abnormal intestinal tube position. Such a case is usually asymptomatic and thus difficult to detect. Therefore, similar variant malformations are only found during an operation required for other abdominal diseases. CASE SUMMARY: An elderly male patient was admitted to the hospital due to gastric cancer. An abdominal computed tomography (CT) scan with contrast revealed that the ascending and descending colon were parallel on the right side of the abdominal cavity, while the sigmoid colon extended into the right iliac fossa, allowing the diagnosis of congenital midgut malrotation. Following thorough preoperative preparation, the patient underwent laparoscopic radical gastrectomy to treat his gastric cancer. Intraoperatively, an exploration of the abdominal cavity uncovered the absence of the transverse colon. The distal colon at the hepatic flexure, along with the ascending colon, extended into the right iliac fossa, where it continued as the sigmoid colon. As planned, the laparoscopic radical gastrectomy was performed, and the patient was discharged from the hospital 7 d after the surgery. CONCLUSION: Asymptomatic intestinal malrotation is best detected by CT, requiring no treatment but possibly interfering with the treatment of other diseases.

Diagnostic accuracy of upper gastrointestinal series in children with suspected intestinal malrotation.

Intestinal malrotation (IM) results from an altered or incomplete rotation of the fetal midgut around the superior mesenteric artery axis. The abnormal anatomy of IM is associated with risk of acute midgut volvulus which can lead to catastrophic clinical consequences. The upper gastro-intestinal series (UGI) is addressed as the gold standard diagnosis procedure, but a variable failure degree has been described in literature. The aim of the study was to analyze the UGI exam and describe which features are the most reproducible and reliable in diagnosing IM. Medical records of patients surgically treated for suspected IM between 2007 and 2020 at a single pediatric tertiary care center were retrospectively reviewed. UGI inter-observer agreement and diagnostic accuracy were statistically calculated. Images obtained with antero-posterior (AP) projections were the most significant in terms of IM diagnosis. Duodenal-Jejunal Junction (DJJ) abnormal position resulted to be the most reliable parameter (Se = 0.88; Sp = 0.54) as well as the most readable, with an inter-reader agreement of 83% (k = 0.70, CI 0.49-0.90). The First Jejunal Loops (FJL), caecum altered position and duodenal dilatation could be considered additional data. Lateral projections demonstrated an overall low sensitivity (Se = 0.80) and specificity (Sp = 0.33) with a PPV of 0.85 and a NPV of 0.25. UGI on the sole AP projections ensures a good diagnostic accuracy. The position of the third portion of the duodenum on lateral views showed an overall low reliability, therefore it was not helpful but rather deceiving in diagnosing IM.

Notable Clinical Differences Between Neonatal and Post-Neonatal Intestinal Malrotation: A Multicenter Review in Southern Japan.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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 OF EVIDENCE: LEVEL III.