Malrotation Research Articles

Variations of intestinal malrotation in adults: A case report of midgut volvulus and literature review for the surgeon.

IntroductionIntestinal malrotation is a rare congenital abnormality occurring in 0.2–1% of the population. Adult presentations comprise only 0.2–0.5% of all cases leading to diagnostic challenges and worse outcomes in adults. We present a rare case of chronic/intermittent midgut volvulus with unique anatomic findings in an adult with intestinal malrotation.Presentation of caseAn 18-year-old Caucasian male presented to a community hospital with abdominal pain, nausea, and vomiting. He underwent a CT scan demonstrating concern for small bowel volvulus and subsequently underwent a negative exploratory laparotomy. He was discharged post-operatively with no identified etiology for his presentation. He subsequently had multiple presentations to the ED with recurrent symptoms, review of imaging led to concern for duodenal volvulus resulting in transfer to a tertiary hepatobiliary centre. Repeat CT scan two weeks following initial presentation was consistent with intestinal malrotation with midgut volvulus. Bloodwork was unremarkable and physical exam demonstrated normal vital signs with a tender epigastrium. He underwent an exploratory laparotomy with Ladd's procedure. Intra-operative findings included a midgut volvulus and uniquely positioned Ladd's bands to the transverse colon. Post-operatively he tolerated oral intake and was discharged with three-month follow-up.DiscussionAdults with intestinal malrotation suffer from delays in diagnosis and management. In contrast to the neonatal population, adults often present with vague, or chronic symptoms, which obscures the diagnosis.ConclusionThe increased morbidity and mortality observed in adults with intestinal malrotation highlights the need for surgeons to appreciate the challenges associated with this diagnosis in the adult population to ensure early recognition and management.

Prenatal imaging features and perinatal outcomes of foetal volvulus-A literature review.

To conduct a review of the literature on foetal volvulus with emphasis on prenatal imaging, pregnancy characteristics and clinical outcomes. A review of all published cases of foetal volvulus diagnosed prenatally and indexed in Medline, EBSCOhost, CINAHL, SOCIndex and Healthy Policy Reference Centre. Studies without antenatal sonographic signs of foetal volvulus and without a postpartum surgical diagnosis were excluded. Data were analysed for frequencies and distributions and tested for statistical significance. Eighty-eight cases of foetal volvulus were identified from 58 published case reports/series. The most common ultrasound findings were dilated bowel/stomach (77.3%), polyhydramnios (30.7%) and whirlpool/snail sign (28.4%). Median gestation at diagnosis was 31.9weeks (IQR 27-34) and mean gestation at delivery was 34.5weeks (SD 2.8). Underlying aetiology included intestinal malrotation (15.9%), cystic fibrosis (14.8% of all cases, 32.5% of tested cases) and abnormal mesenteric fixation (12.5%). Complications included intestinal atresia (36.4%) and foetal anaemia (9.1%). The overall perinatal mortality rate was 14.5%. Foetal volvulus is a rare condition with high rates of preterm birth and perinatal mortality. Intestinal malrotation and cystic fibrosis are common predisposing causes, although the majority are idiopathic. Bowel and/or gastric dilatation is by far the most common sonographic finding.

Case Series of Acute Abdomen with Midgut Malrotation Presenting in Adulthood

Introduction: Intestinal malrotation (IM) is a congenital anomaly due to partial or complete failure of the 270° counter-clockwise rotation of the midgut around the superior mesenteric vessels in 5th to 12th week of fetal life. Midgut malrotation usually presents as an emergency in the 1st month of life or within infancy. Adult cases are rarely reported and usually present with chronic nonspecific symptoms. Here, we present a case series of acute abdomen with midgut malrotation presenting in adulthood that came to the Department of Surgical Gastroenterology of a tertiary hospital in Salem, Tamil Nadu. Case Summary: Four patients presented with acute abdominal complaints, diagnosed, and proceeded with emergency laparotomy. Three patients had abdominal abnormalities unrelated to coexisting IM, and the other one showed sequelae of IM with Ladd's band and obstruction. Except the first patient, all other patients improved well postoperatively and were discharged without complications. Conclusion: IM should be considered in differential diagnosis of acute abdomen and bowel obstruction in adults. IM may be encountered as an incidental imaging/laparotomy finding or be the cause of acute abdomen.

Neonatal intestinal malrotation: A diagnostic pitfall

Intestinal malrotation is a rare abnormality, resulting in intestinal malrotation and impaired fixation. This clinical entity encompasses spectrum of anatomical conditions and associated clinical disorders. Main symptoms of intestinal obstruction related to malrotation are sudden bilious emesis in a previously healthy term neonate. Upper gastrointestinal (UGI) contrast study is the examination of choice and remains as gold standard for intestinal malrotation. With 90% positive predictive value, misdiagnosis in rotational abnormalities could still happen in UGI contrast study. We report a case of pitfall diagnosis in an 8-day-old male neonate, in which obstruction has been misinterpreted as duodenal web rather than malrotation that was furthered found during surgery.

A Rare Cause of Left Lower Quadrant Pain: Acute Appendicitis in a Patient with Intestinal Malrotation

A Rare Cause of Left Lower Quadrant Pain: Acute Appendicitis in a Patient with Intestinal Malrotation

LAPAROSCOPIC LADD'S PROCEDURE IN A 58YR FEMALE WITH TRANSVERSE COLON TUBERCULOSIS MASS WITH MALROTATION - A RARE CASE REPORT

Intestinal malrotation is partial or complete failure of 270% counterwise rotation of midgut around the superior mesenteric vessels in embryonic life. Malrotation can present in any age group. Most of the patients present within rst month of life. However late presentation is rare, we present a case report of 58 yr female with tuberculosis transverse colon mass with malrotation with ladd's band with sub acute intestinal obstruction. She presented with epigastric pain,mass and vomiting. Patient underwent laaproscopic ladd's procedure and descending colon and ileum bypass with Ileo Descending anastomosis , She had good postoperative recovery. In this article we describe our experience in creating descending colon and ileum bypass with Ileo Descending anastomosis.

A Case of Meckel's Diverticulitis with Intestinal Malrotation Diagnosed Preoperatively and Treated Surgically

A Case of Meckel's Diverticulitis with Intestinal Malrotation Diagnosed Preoperatively and Treated Surgically

Short-term curative effect of laparoscopic assisted surgery on patients with congenital intestinal malrotation and its effect on gastrointestinal function in very low birth weight infants.

Some studies have shown that laparoscopic-assisted surgery may have a good effect on gastrointestinal function in patients with congenital intestinal malrotation. To investigate the short-term curative effect on patients with congenital malrotation of the bowel after laparoscopic-assisted surgery and the impact on gastrointestinal function. We selected 100 patients with congenital intestinal malrotation who underwent surgery between June 2019 and June 2021. Among them, the control group underwent traditional laparotomy, and the observation group underwent the laparoscopic-assisted Ladd procedure. We observed and compared the differences in surgical indicators, immune function, short-term curative effect and gastrointestinal function of the two groups of patients. There was no significant difference in immune function between the two groups of patients before surgery (p > 0.05), while the comparison of CD8+ and B cells after 1 week of surgery was significantly different, and the observation group was better than the control group, which was statistically significant (p < 0.05). There was no statistically significant difference in NK cells after 1 week of surgery (p > 0.05). The clinical treatment efficiency of the observation group was 94.00% significantly higher than that of the control group, 78.00%, and the difference was statistically significant (p < 0.05). A comparative study of congenital malrotation of the bowel was performed. The effect of the Ladd procedure is significantly better than that of traditional open surgery, effectively improving the gastrointestinal function of patients after surgery, and the postoperative abdominal incision is hidden, providing a certain reference for clinical surgery of congenital malrotation.

Current understanding of the treatment of newborns with intestinal malrotation: a systematic review

This review aimed to analyze and organize information on the treatment of newborns with bowel malrotation. A systematic review was conducted using the databases PubMed, Web of Science, Google Scholar, Scopus, and eLibrary and journals of Pediatric Surgery, Russian Bulletin of Pediatric Surgery, Anesthesiology and Reanimatology, and Pediatric Surgery from 2010 to 2020. The search terms were as follows: malrotation, neonatal, infant, newborn, intestinal malrotation, malrotation, neonatal period, Ledds syndrome, and midgut volvulus. All journal issues were reviewed for the selected period.&#x0D; Following the screening of published articles, six full-text articles that met the selection criteria were included in the systematic review. A total of 191 patients with bowel malrotation that received various treatments were described: 98 patients underwent defect correction by laparoscopy and 93 patients underwent laparotomy. Bloating was found in 103 children. Reoperations were performed on 18 newborns because of relapse. Complications in the postoperative period were noted in 25 cases, and treatment of 16 children was fatal. No study was performed following a high methodological level.&#x0D; To date, studies on the surgical treatment of newborns with bowel malrotations are limited. In addition, none of the studies were conducted at a high methodological level. The lack of randomized controlled trials can be compensated for by closer collaboration between medical clinics, collection of a larger evidence base based on the analysis of the results of cohort and casecontrol studies, and examination of the reasons for the variability of results among centers.

Acute primary gastric volvulus occurring after overeating in a 12-year-old girl

Gastric volvulus is a rare cause of recurrent abdominal pain or vomiting. This report presents a case of a 12-year-old girl with acute gastric volvulus and 2-hour history of epigastric pain after overeating. Computed tomography showed severe gastric distension without other abnormal findings. The gastric fluid decompressed through a nasogastric tube was approximately 4,000 mL in volume and non-bilious in color. Upper gastrointestinal series showed organo-axial volvulus without intestinal malrotation. Emergency laparotomy was performed. Because the color of stomach was pinkish, detorsion of the volvulus and gastropexy was performed. Postoperatively, the girl recovered uneventfully.

“Triple duodenal obstruction” complicated with intestinal malrotation and several cardiovascular malformations

Triple duodenal obstruction is an extremely rare disorder—apparently only two cases were reported in the English literature. We present a third case complicated with some cardiovascular anomalies (interrupted inferior vena cava, left-sided inferior vena cava, preduodenal portal vein, and ventricular septal defect) and intestinal malrotation. Operative procedure to reconstruct continuity of the duodenal lumen was a combination of longitudinal incision, partial web resection, and transverse closure at the first obstruction site together with diamond-shaped duodeno-duodenostomy between the oral side of the second obstruction and the anal side of the third obstruction.

Novel abdomino-pelvic anomalies in Kagami-Ogata syndrome

Kagami-Ogata syndrome (KOS) is a rare genomic imprinting disorder of chromosome 14 with characteristic facial features, a small, bell-shaped thorax, muscular hypotonia, and abdominal wall defects. We present a case of a kidney incarcerated in a posterolateral Bochdalek type congenital diaphragmatic hernia (CDH), intestinal malrotation and stage 2 rectocele in KOS. Successful repair of CDH and malrotation was achieved with Ladd's procedure, CDH repair, Nissen fundoplication and gastrostomy tube placement. She had no post-operative complications and is currently tolerating 100% enteral feeds. CDH, intestinal malrotation and rectocele have not been previously reported in KOS. Despite overall poor prognosis, KOS patients with CDH and malrotation can be repaired successfully with improvement in quality of life.

Pseudo-Bartter Syndrome as an Atypical Presentation of Intestinal Malrotation: a Case Report

Intestinal malrotation is a congenital intestinal rotation anomaly and can present with various symptoms. Pseudo-Bartter syndrome (PBS), which mimics the manifestations of Bartter’s syndrome, can be caused by a severe chloride deficiency and can be seen due to cystic fibrosis, hypertrophic pyloric stenosis, diuretic abuse, and so on. We presented a 3.5-month-old boy who had convulsion after multiple vomiting, followed up in the intensive care unit, and was diagnosed with malrotation. The patient complained of vomiting 10 times and diarrhea 3–4 times a day in the last 15 days. He went to another urban hospital after having a tonic–clonic seizure. Hypochloromic hypokalemic metabolic alkalosis was detected and he was admitted to the intensive care unit of our hospital. He was examined for differential diagnosis. The intestinal malrotation was diagnosed with esophagogastroduodenography. After surgery, he made a good recovery and was discharged symptom free. Cystic fibrosis, Barter syndrome, and hypertrophic pyloric stenosis are known diagnoses in patients presenting with PBS. Only two patients diagnosed with malrotation have been reported in the literature. We wanted to present this case so that the diagnosis of malrotation should be considered in patients presenting with PBS.

Splenic torsion in the setting of polysplenia syndrome

Polysplenia syndrome is a rare condition that occurs when polysplenia co-exists with various other congenital anomalies. Splenic torsion in the setting of polysplenia syndrome is extremely rare, having been documented only five times previously in the English literature. We present a case of a 9 year-old female who underwent laparoscopic splenectomy for ischemic polysplenia secondary to splenic torsion in the setting of polysplenia syndrome. This previously healthy patient presented to the emergency department with a 3-day history of abdominal pain and emesis. She was found to have leukocytosis and computed tomography (CT) revealed evidence of polysplenia with torsion of multiple splenules, as well as an annular pancreas, intestinal malrotation, and an interrupted inferior vena cava (IVC) with azygous continuation. To our knowledge, this was the first documented case of splenic torsion in the setting of polysplenia syndrome with concurrent anomalies of intestinal malrotation, interruption of the IVC with azygous continuation, and preduodenal portal vein (PDPV). The goal of our case report is to describe this rare phenomenon while detailing the diagnostic and management considerations. Splenic Torsion, Polysplenia Syndrome, Polysplenia, Malrotation.

Imaging findings in intestinal malrotation

Imaging findings in intestinal malrotation

Left Amyand’s hernia with inflamed appendix and sealed of perforated caecum an unusual case

Amyand’s hernia is defined as the hernia with appendix normal, inflamed or perforated as content. 1% of inguinal hernias are Amyand’s and amongst them 0.1% contains inflamed appendix. Commonly encountered on right size due to anatomical position of appendix. Left Amyand’s is rare and associated with intestinal malrotation, situs invertus and mobile caecum. Here, we presented an interesting case of left irreducible hernia in 70 years old gentleman with no signs of acute obstruction or strangulation, patient underwent emergency laparotomy in which hernial sac contents were inflamed ileal loop, inflamed appendix and perforated caecum in 70 years old man is rare presentation and not reported in any literature as per our knowledge. Resection of inflamed bowel loop with ceacum done along with ileo ascending anastomosis with primary tissue repair done. Post-operative period was uneventful. Hernia sac contents are most of the time surprising and their management sometimes differ according to the content. Appendix in hernia sac is found in 1% of all hernia but lack of facility for the pre-operative diagnosis and varied presentation it is challenging to diagnose and operate accordingly.

Gigant Morgagni Congenital Hernia Associated with Intestinal Malrotation and Meckel Diverticulum in Child with Down Syndrome- Clinical Case Presentation and Literature Review

Gigant Morgagni Congenital Hernia Associated with Intestinal Malrotation and Meckel Diverticulum in Child with Down Syndrome- Clinical Case Presentation and Literature Review

A Case of Midgut Volvulus with Intestinal Malrotation in a 4-year-old Boy

A Case of Midgut Volvulus with Intestinal Malrotation in a 4-year-old Boy

Determining oncogenic patterns and cancer predisposition through the transcriptomic profile in Mitchell\u2013Riley syndrome with heterotopic gastric mucosa and duodenal atresia: a case report

BackgroundHomozygous mutations in the transcription factor RFX6 are the cause of the Mitchell–Riley syndrome (MRS) associating neonatal diabetes, congenital digestive system, such as biliary atresia, pancreatic hypoplasia, duodenal and/or jejunal atresia, intestinal malrotation, gallbladder aplasia, cholestasis. A constitutive inactivation of RFX6 leads also to gastric heterotopia. Application of RNA-seq in human diseases may help to better understand pathogenic mechanism of diseases and to predict the risk of developing chronic disorders and personalizing their prevention and treatment. We evaluated oncogenic patterns and cancer predisposition using the transcriptomic profile in a case of MRS with neonatal diabetes, duodenal atresia, and extensive intestinal tract gastric heterotopia.ResultsWe signalled the interactors of RFX6 with other up and downregulated genes, that may be interested in severity of diabetic condition, in multi-organs impairment and cancer predisposition. Furthermore, several dysregulated genes are involved in biological processes that can lead to promote cancer including “Evading apoptosis” (BAD, BBC3, EGF, FGFR2, FLT3LG, HMOX1, HRAS, IFNAR2, IGF1R, IL12RB1, IL13RA1, IL15, IL2RB, IL2RG, IL6R, KEAP1, MGST1, PDGFA, PDGFRB, PIK3R3, RALB, RALGDS, RASSF1, SOS1, TGFA, TXNRD3), “Proliferation” (APC, BRAF, CCND2, CCND3, CCNE2, FGFR2, FLT3LG, FZD1, FZD6, HMOX1, HRAS, IGF1R, KEAP1, LRP6, MAPK3, MGST1, PDGFA, PDGFB, PDGFRB, RB1, SOS1, TGFA, TXNRD3, WNT10B), “Sustained angiogenesis” (BRAF, FGFR2, FLT3LG, HRAS, IGF1R, JAG1, MAPK3, NOTCH2, PDGFA, PDGFB, PDGFRB, SOS1, TGFA, TGFB1), “Genomic instability” (BAD, BBC3) and “Insensitivity to anti-growth signals” (SMAD2, TGFB1). We also inspected the signalings and their related genes in cancer, such as “PI3K signaling”, “ERK signaling”, “JAK-STAT signaling”, “Calcium signaling”, “Other RAS signaling”, “WNT signaling”.ConclusionsIn our MRS patient, we signaled the interactors of RFX6 with other up- and downregulated genes that may be related to severe diabetic condition, multi-organ impairment, and cancer predisposition. Notably, many dysregulated genes may lead to triggering carcinogenesis. The possibility of the patient developing cancer degeneration in heterotopic gastric mucosa and/or additional long-term tumoral sequelae is not excluded. Personalized prevention and treatment strategies should be proposed.

The Management of A Small Bowel Obstruction Caused by A Right Paraduodenal Hernia

Right paraduodenal hernia (RPH) is a type of internal hernia and is frequently associated with intestinal malrotation. The preoperative diagnosis is often difficult. We report the case of a