Anomalies Of Intestinal Rotation Research Articles

Mesenteric Base Width: Abdominal Wall Diameter Correlates With Intestinal Rotational Anomalies in Infants

IntroductionSymptomatic malrotation requires urgent Ladd procedure. Patients with incidental or indeterminate findings have historically been managed with observation or operatively. Fluoroscopic identification of the duodenojejunal junction (DJJ) and ileocecal junction (ICJ) can guide operative decision-making, but algorithms have not been validated. This study aimed to determine whether fluoroscopic mesenteric base width (MBW) standardized to abdominal wall diameter (AWD) correlates with intraoperative anatomy in infants. MethodsWe retrospectively reviewed patients between 2013 and 2023 who were <1 year with fluoroscopy identifying DJJ and ICJ. Infants with normal rotation evaluated for digestive concerns were included. Congenital conditions with intestinal nonrotation were excluded. Two radiologists independently measured MBW as a diagonal line from DJJ to ICJ and maximal transverse AWD from inferior ribs. A ratio was calculated and compared between groups. Wilcoxon rank-sum and Kruskal–Wallis tests with p < 0.05 were considered significant. Area under the receiver operating characteristic curve (AUROC) was used to identify optimal ratio cutoff. ResultsFifty-eight patients, 22 normally rotated and 36 with intestinal rotational abnormality (IRA), met inclusion criteria. Preoperative radiographic concern for malrotation differed between groups (p < 0.0001). Median MBW:AWD was significantly lower in IRA than normal rotation based on imaging (0.31 vs 0.65, p < 0.0001). Optimal MBW:AWD of 0.55 had an AUROC of 0.9578. ConclusionsRadiographic measurement of MBW:AWD accurately predicted IRA from normal rotation with an optimal ratio cutoff of 0.55. Further validation will determine whether this ratio should play a role in management of incidental IRA or indeterminate findings on UGI. Level of EvidenceIII.

Malrotation and Fibroblast Growth Factor Receptor-2-Associated Craniosynostosis: An Underrecognized Association and Management Pathway.

Feeding difficulties are common and multifactorial in children with Fibroblast Growth Factor Receptor-2 (FGFR-2) mutations. Intestinal rotation anomalies have been demonstrated to occur more frequently in animals with FGFR-2 mutations. This study aims to describe intestinal rotation anomalies, surgical management, and feeding assistance in children with FGFR-2 mutations who have undergone upper gastrointestinal (UGI) contrast studies. Retrospective data were collected of children born between 1988 and 2020 in a UK quaternary craniofacial unit with FGFR-2-associated craniosynostosis. A consultant survey of approach to malrotation was undertaken. Thirty-four children were included, 17 (50%) female. Six (18%) had UGI symptoms, which included bilious vomiting (n=2), nonbilious vomiting (n=5), retching (n=1), feed intolerance (n=3), and failure to thrive (n=3). Nine had a gastrostomy in situ. Intestinal rotation anomalies occurred in 4 (12%) children, 3 of whom underwent a Ladd procedure and two third required gastrojejunal feeding postoperatively. Consultants agreed that all children with FGFR-2 mutation and UGI symptoms should undergo UGI contrast study, as should children requiring a gastrostomy. Intestinal rotation anomalies in children with FGFR-2 mutations occur more frequently than the general population. Prompt consideration of UGI contrast in symptomatic children with FGFR-2 mutation is recommended to enable early surgical management of children with malrotation.

Late presentation of midgut malrotation with obstruction in a child

Midgut malrotation is an anomaly of intestinal rotation that occurs during embryology and usually presents during neonatal period. Symptoms are vague as presentation is delayed whilst some can be identified incidentally during adulthood. Herein we report of a 6-year-old girl who presented with food intolerance, abdominal pain and failure to gain weight. CT-scan confirmed midgut malrotation and was successfully managed by open Ladd's procedure. • Midgut malrotation is a congenital anomaly that usually presents in the neonatal period. • Symptoms are unspecific in older children and sometimes asymptomatic in others. • Midgut malrotation has a high risk of bowel ischaemia due to compression of the SMA. • CT-scan with contrast is diagnostic tool of choice and shows “whirlpool” sign. • Mainstay management is surgery (Ladd's procedure) with promising outcome.

Small Bowel Volvulus on Incomplete Common Mesentery - A Rare Complication in Adults: A Case Report

A small bowel volvulus on the common mesentery was discovered in an 18-year-old young adult of occlusive syndrome associated with septic shock. In this observation it is the imaging which made the diagnosis by showing the volvulus and by visualizing the anomaly of intestinal rotation. We are doing a literature review of this rare entity.

La malrotazione intestinale: cosa deve sapere il pediatra

Intestinal malrotation includes anomalies of intestinal rotation and fixation occurred during embryogenesis. It may present with acute or chronic-intermittent symptoms or remain asymptomatic. There are several methods to identify a defect of intestinal rotation, however an upper gastrointestinal series remains the method of choice in non-acute cases. Surgical correction is performed by open laparotomy, while the laparoscopic approach is reserved only to selected cases. This article suggests an evaluation of children with suspected intestinal malrotation and gives information about its management and complications to paediatricians.

Diagnostic Conundrum in Intestinal Obstruction; Malrotation in Elderly

Congenital intestinal malrotation is an anomaly of intestinal rotation and fixation which occurs during fetal development. It is rare for a malrotation to be present in adulthood. Indeed, most adult patients are asymptomatic and are later discovered incidentally during operation for other clinical conditions. Here we presented a case of malrotation in a 79-year-old elderly man presented with intermittent colicky abdominal pain, obstipation and vomiting for 2 days. Abdominal examination revealed distended abdomen, tenderness at right upper abdominal quadrant and peritonism. A contrast-enhanced computed tomography scan of the abdomen showed small bowel malrotation with suspected volvulus. The patient underwent exploratory laparotomy in which the intraoperative findings were small bowel malrotation, tight Ladd's band and midgut volvulus on top of perforated gallbladder empyema. The patient also underwent cholecystectomy, release of Ladd's band, re-rotation of the bowel, fixation of caecum to the left lower quadrant and appendicectomy. A complete recovery and relief of gastrointestinal symptoms were achieved postoperatively.

Reversed Bowel Rotation with Midgut Volvulus and Internal Hernia

Congenital intestinal malrotation is a group of intestinal rotational anomalies occurring during embryogenesis. Reversed rotation is considered the rarest type of malrotation and often presents with symptoms of bowel obstruction. We present a rare case of a 27-year-old woman who presented with acute abdomen. The patient’s preoperative computed tomography (CT) scan and operative findings confirmed reverse malrotation, internal hernia of foramen of Winslow, and midgut volvulus. The transverse colon, duodenum, small bowel, cecum, and appendix were abnormally located, with the presence of Ladd bands. The patient underwent an emergency laparotomy (Ladd procedure) with an uneventful postoperative recovery and an unremarkable follow-up CT scan of the abdomen. A review of the literature for intestinal reverse malrotation is also presented to provide an understanding of the expected clinical picture and imaging findings for this rare anomaly.

Congenital Left Mesenteric-Parietal Hernia Associated to a Small Bowel Volvulus: About a Case

Introduction: The left mesenterico-parietal hernia or left paraduodenal hernia is an anomaly of intestinal rotation which may be responsible for intestinal obstruction. It is rare. Observation: A 5-year-old boy was admitted for abdominal pain with episodes of vomiting and cessation of matters and gases. After clinical and paraclinical investigations, the diagnosis of occlusion was accepted and the child was operated. Exploration revealed a mesenterico-parietal hernia with a retrocolic sac measuring 11 cm of collar and a deep of 18 cm containing a twisted bowel. After reduction of the content, we untwisted the small intestine and released adhesions. The hernial sac was partially resected and the defect was closed. Operating outcomes were simple. Conclusion: The mesenterico-parietal hernia is a cause of organic intestinal obstruction. Although of congenital origin, it can have a late clinical manifestation. It should be considered in case of episodes of repetitive abdominal pain and vomiting to avoid complications.

Mesenteric cyst as a presentation of chronic midgut volvulus in a 12 year old boy

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

Intestinal volvulus without malrotation in a dichorionic diamniotic twin: case report and review of literature

Volvulus is a condition in which the small bowel and proximal colon twist around the superior mesenteric artery, and a surgical emergency associated with high intrauterine morbidity and mortality. Intestinal volvulus can occur at any age, but most cases of volvulus occur in infants with anomalies of intestinal rotation and fixation. Intestinal volvulus without malrotation is a rare condition, as only normal intestinal rotation and fixation are present. The authors report a case of intestinal volvulus without malrotation in one fetus of twins.

Acute Intestinal Obstruction in an Adult Patient Due to Ladd’s Band: A Case Report

Introduction: In adult patients some adhesions between intestinal segments and malignities are common in the ethiology of mechanic intestinal obstruction whereas congenital anomalies are seen rare. In this case report we present a 60-year-old patient presents with mechanical bowel obstruction symptoms and diagnosed as Ladd's bands. Case presentation: A 60-year-old male applies emergency servise with vomiting and nausea. According to the laboratory and radiological tests the paient was diagnosed as mechanical bowel obstruction and underwent exploratory laparatomy. In exploration Ladd's band was seen and cut for releasing the herniated intestinal segments. Discussion: Congenital anomalies of intestinal rotation are often seen in infants and children therefore this pathology can be missed in adult patients. We must be aware of these pathologies on the way of diagnosis of the patients with mechanical bowel obstruction.

A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation.

Altınay-Kırlı E, Özcan R, Öncül M, Özmen E, Eliçevik M, Büyükünal C, Emir H, Topuzlu-Tekant G. A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation. Turk J Pediatr 2017; 59: 699-703. Ectopic ovary is a rare anomaly that can be associated with unicornuate uterus and renal anomalies. Intestinal rotational anomalies are failure of normal rotation and this arrest in development can predispose to develop a malfixated midgut that is a risk factor for volvulus and significant morbidity and mortality especially in early childhood. Cyclic abdominal pain is a common symptom for both of two distinct pathologies in adolescent ages. Here, we report a case of unicornuate uterus together with right ectopic ovary and intestinal malrotation.

Is Screening of Intestinal Foregut Anatomy in Heterotaxy Patients Really Necessary?: A Systematic Review in Search of the Evidence.

(1) Is screening of intestinal rotational anatomy obligatory in "asymptomatic" patients with heterotaxy? (2) Does detection of an anomaly warrant surgical correction? Heterotaxy is an abnormal arrangement of thoraco-abdominal viscera across a left-to-right axis. Intestinal rotational anomalies are frequent among patients with heterotaxy, but debate exists as to whether they are benign in nature, requiring careful observation alone, or if surgical correction is warranted to prevent obstruction or midgut volvulus. A systematic review [according to PRISMA guidelines] was conducted using CINAHL, EMBASE, Medline, and Cochrane Databases. Article quality was assessed using MINORS criteria. Conference proceedings and unpublished data were screened additionally. Nineteen studies met the eligibility criteria but reporting was adequate for 9. All were observational studies. These included a total of 414 patients managed expectantly, that is, "asymptomatic patients" in whom no intestinal rotation screening was undertaken (group A), 191 cases in whom screening was performed routinely (group B), and 92 patients considered "symptomatic" of potential rotational anomalies and therefore underwent imaging or laparotomy (group C). In group A, 1 patient developed symptoms attributable to malrotation in whom laparotomy confirmed the diagnosis (0.24%). Among groups B and C, 151 had Ladd's operations (53%) and 14 cases of malrotation with obstruction or volvulus were described (4.9%), of which 2 "symptomatic patients" died before laparotomy. Overall surgical complication rate was 17% with 30-day mortality rate of 2.6% to 4.6%. The evidence base for screening "asymptomatic" patients is weak especially considering the life-limiting comorbidities.

Endoscopic treatment of intestinal malrotation in newborns and infants

To compare treatment of intestinal malrotation in newborns and infants using laparoscopy and laparotomy. For the period from January 2004 to December 2013 34 Ladd's procedures were performed. Children were divided into 2 groups by 17 patients: laparoscopic (group I) and open treatment (group II). Both groups had similar demographic and other preoperative parameters. There were significant differences in duration of operation between both groups (61 vs. 70 minutes). Nutrition was initiated earlier after mini-invasive treatment (1.5 days vs. 3 days) and the time need for full enteral nutrition was also less (4.2 days vs. 6.9 days). Hospital-stay was shorter in group I (7.7 vs. 10.2 days). Number of early postoperative complications was similar in groups. Incidence of remote complications was higher in laparotomy group but the differences were not significant. Our results showed that endoscopic correction of congenital anomalies of intestinal rotation provides better postoperative results than open surgery and can be widely used in young children.

Heterotaxy and intestinal rotation anomalies: 20 years experience at a UK regional paediatric surgery centre.

The association of heterotaxy with intestinal rotation anomalies is well described. However debate exists with regard optimal management notably should 'asymptomatic' bowel rotation anomalies undergo operation? The present study therefore sought to determine: (1) the risk(s) of volvulus in patients diagnosed with heterotaxy and (2) define morbidity associated with operation for 'asymptomatic' anomalies in a fragile patient cohort with co-existent congenital heart disease. Medical case record reviews of ALL heterotaxy patients born during January 1993-December 2013 and attending a UK paediatric centre were analyzed. Of a total of 92 patients, 16 (17.4%) cases underwent foregut imaging studies. Three examinations were performed in 'symptomatic' patients. Twelve studies reported 'abnormal anatomy' with only five patients undergoing surgical correction. No complication(s) were recorded after Ladd's operation to correct defects. A single fatality occurred within 30 days postoperatively from cardiac failure. In 87 patients in whom Ladd's operation was not undertaken, no single patient developed intestinal volvulus (median length of follow-up 27.2 months, total 446.1 person years). This study strongly supports a 'watchful waiting' policy for heterotaxy patients. Many children who ultimately die from heart disease may avoid unnecessary abdominal surgery.

Heterotaxy syndrome and intestinal rotational anomalies: Impact of the Ladd procedure

BackgroundChildren with heterotaxy syndrome and intestinal rotational anomalies (IRA) are at risk for midgut volvulus and ischemia. Controversy exists regarding risks and benefits of prophylactic Ladd procedures. The purpose of this study is to assess the impact of Ladd procedures on adverse events for children with heterotaxy and IRA. MethodsA retrospective cohort study using the Pediatric Health Information System (PHIS) database was performed. All children with heterotaxy and IRA admitted at age ≤30days and discharged between 1/1/2004 and 1/1/2011 were included with a minimum 2-year follow-up period. The primary outcome was major morbidity, defined as a composite variable for intestinal obstruction/volvulus, ischemia, or resection, or inhospital mortality. Univariable and multivariable analyses were performed. ResultsThere were 325 patients who met the inclusion criteria, including 92 (28%) patients with single ventricles. Mean gestational age was 38.0±2.1weeks and birth weight was 3.1±0.6kg. Ladd procedure was performed during initial hospitalization on 188 (58%) children. In multivariable analyses, Ladd procedure on initial hospitalization was associated with a 2.2 times increased odds of adverse events on subsequent admissions (95% CI 1.3–4.0, p=0.007). ConclusionsLadd procedure is associated with increased odds of adverse events for children with heterotaxy and IRA.

Heterotaxy Syndrome Infants Are at Risk for Early Shunt Failure After Ladd Procedure

Cardiac-specific risks and complications after a Ladd procedure in patients with heterotaxy syndrome (HS) and intestinal rotational anomalies (IRA) are unknown. We sought to (1) describe rates of hospital mortality and early systemic-to-pulmonary (S-P) artery shunt failure after the Ladd procedure in patients with HS and (2) explore risk factors associated with early shunt failure in patients with HS with single ventricle (SV). This retrospective study included all Ladd procedures performed from January 1999 to December 2012 in patients with HS at a single center. Risk factors investigated for early S-P artery shunt failure included birth weight, gestational age, sex, age at and timing of Ladd procedure relative to cardiac operations, and shunttype. Ladd procedure was performed on 54 infants with HS and congenital heart disease. Hospital mortality for the entire cohort was 5.6% (3 of 54 patients). Early shunt failure occurred in 19% (4 of 21) of HS infants with SV. Mean preoperative blood urea nitrogen (BUN) levels were higher in HS infants with early shunt failure (20 versus 12.5 mg/dL; p= 0.054). Patients with SV and HS with S-P artery shunts are at risk for early shunt failure after a Ladd procedure. A higher mean preoperative BUN level is noted in patients with HS and early shunt failure. Careful risk-benefit analysis is indicated before recommending routine elective Ladd procedures in patients with HS.

ADULT MIDGUT MALROTATION WITH LADD’S BAND: A RARE CASE REPORT WITH REVIEW OF LITERATURES

Background: Midgut malrotation is an anomaly of intestinal rotation that occurs during fetal development and usually presents in the neonatal period. It is rare for midgut malrotation to present in adulthood. Method: We present a case of 15 year old girl presenting with acute abdomen of 15 days duration. Clinical evaluation and investigations revealed features of acute small gut obstruction with peritonitis. Patient underwent exploratory laparotomy which revealed Ladd ? s band with midgut malrotation with gangrenous and perforated duodeno-jejunal segment. Derotation with release of band with appendicectomy and resection of gangrenous segment with end to end anastomosis and anatomical repositioning of the bowel was performed. Results: Complete resolution of symptoms seen in 4 months follow-up. We report this case for the rarity of the case with an unusual presentation of adult midgut malrotation due to Ladd?s band presenting with gangrene of the affected gut with perforation, which was managed successfully.

PS-291 Heterotaxia And Intestinal Rotational Status: Is It Safe To Watch And Wait?

Background The association of heterotaxia with intestinal rotation anomalies is well documented. Controversy still persists though in regards to the optimal diagnostic strategy, as well as to the benefit of surgical correction of identified rotational abnormalities in patients with no gastrointestinal symptoms, especially given the frequent associated cardiac anomalies. The aim of this study was to test the validity of the watchful wait approach in a series of patients with heterotaxia. Method A retrospective analysis of the case notes and electronic records of patients diagnosed with heterotaxia syndromes was performed at a single tertiary paediatric referral centre that offers paediatric cardiac surgery services. All patients with situs anomalies, born within the 1st January 1998 to 31st December 2013, were included. Results 59 patients were identified, of which 13 underwent contrast studies to identify rotational anomalies. Only 2 studies were performed in symptomatic patients (vomiting, failure to thrive). 10 of the studies identified abnormal anatomy, but only 4 patients proceeded to surgical correction. No complications were reported following the Ladd’s procedures. There was a single death within 30 days postoperatively, attributed to cardiac failure. Regarding the patients that had no surgery, during the follow-up period (median 9.2 years, range 11- 187 months), no patients presented clinically with malrotation or volvulus. Conclusions This study supports the ‘watch and wait’ approach for children with heterotaxia, provided that close surveillance can be achieved. Diagnosis and treatment should be individualised and tailored to each patient’s clinical characteristics in order to avoid unnecessary morbidity and mortality.

PS-288 The Performance Of Imaging For Suspected Malrotation: Not So Bad After All

Despite reports of imaging characteristics that would identify intestinal rotational anomalies that predispose to volvulus, most paediatric surgeons would proceed to an operation when imaging is suggestive. The aim of this study was to test the performance of the upper gastrointestinal (UGI) contrast studies and its restrictions that lead to false positive or false negative reports. Methods We performed a retrospective analysis of patients investigated for malrotation, or treated for it over 5 years (2008–2013). Radiology reports were graded as positive, equivocal or negative. If the study was inconclusive, further imaging or treatment was dictated by clinical status. Main Results 279 patients were investigated due to suspected malrotation, while in 24 patients malrotation was an incidental finding. In total, 86 patients underwent a Ladd’s procedure. If the definite positive and equivocal reports were considered as positive regarding the intention to intervene surgically, the sensitivity of UGIS was 94%, specificity was 95.4%, with 18% false positive results and 1.4% false negatives. In the 11 patients with proven false positive results, two had a negative laparotomy, but all the remaining 9 patients had surgical findings (including 3 duodenal stenoses, and 3 intestinal atresias). Conclusion The UGI series proved to be very sensitive and specific for the diagnosis of malrotation, when the clinical pathway dictates prompt surgical intervention in the presence of positive or at least non-negative radiology report. This approach can lead to a significant number of false positive results, although other surgically treatable pathology is commonly identified in these patients.