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Intestinal malrotation is life-threatening and often presents during infancy with bilious vomiting. The prevalence and presentation among extremely premature infants are unknown. We retrospectively reviewed all infants born at less than 28 weeks’ gestation diagnosed with symptomatic intestinal malrotation in a tertiary neonatal intensive care unit over a 10-year period (2010–2020). Seven of 514 (1.4%) extremely premature infants developed symptomatic intestinal malrotation during this period. All were non-syndromic. In comparison, the prevalence of symptomatic intestinal malrotation in 7382 infants ≥ 28 weeks’ gestation admitted during the same period was 0.2%. Intestinal malrotation was confirmed at laparotomy in all extremely premature infants and six of seven had midgut volvulus. All but one presented with marked abdominal distension; none had bilious vomiting and only three had bilious gastric aspirates. A subacute onset with non-specific features such as recurrent apnoea and bradycardia, feed intolerance, and intermittent abdominal distension was common. All infants underwent a Ladd procedure. Two required extensive bowel resection resulting in short gut syndrome and three underwent further surgery for adhesive small bowel obstruction. One patient died at 10 months of age from respiratory failure but the others were well 1–3 years later. Symptomatic intestinal malrotation in extremely premature infants has a relatively high prevalence. It may present with marked abdominal distension without bilious vomiting, demanding a high index of suspicion. An atypical presentation, potential alternative abdominal pathologies, coexisting comorbidities, and concerns about survival in these fragile babies may deter the surgeon despite the opportunity of a good outcome.

To investigate (1) the indications for reoperation after neonatal Ladd’s procedure, (2) the type of reoperation and (3) its outcome. We reviewed all neonatal Ladd’s procedures in our hospital from 2003 to 2017 and the outcomes of reoperation in these patients. 252 neonates had Ladd’s procedure: 59 were laparoscopic (23.4%) and 193 open (76.6%). 15 (6.0%) required reoperation with no difference between laparoscopic and open (p = 0.12). Overall, the indications for reoperation were: adhesive intestinal obstruction (n = 10, 4.0%), recurrent midgut volvulus (n = 4, 1.6%), and missed diagnosis of associated anomaly (n = 1, 0.4%). The incidence of recurrent midgut volvulus was higher after laparoscopic Ladd’s procedure (3/59; 5.1%) compared to open Ladd’s procedure (1/193; 0.5%) (p = 0.04). Adhesive intestinal obstruction developed after both open (8/193, 4.1%) or laparoscopic Ladd’s procedure (2/59, 3.3%). The duration of reoperation and the length of post-operative hospital stay were 63.4 ± 27.1 min and 10.1 ± 5.2 days, respectively. After reoperation, there were no post-operative complications. All children were well at follow-up (6 months–14 years). In neonates, laparoscopic Ladd’s procedure compared to the open Ladd’s procedure is associated with a significantly higher risk of recurrent volvulus. The risk of developing this potentially dangerous complication after laparoscopic Ladd’s procedure raises doubts about the effectiveness and safety of the laparoscopic approach in neonates.

Intestinal malrotation is often diagnosed in infancy. The true incidence of malrotation outside of this age is unknown. These patients can present atypically or be asymptomatic and diagnosed incidentally. We evaluate the incidence, clinical presentation, ideal imaging, and intra-operative findings of patients with malrotation over 1 year of age. Retrospective review was conducted in patients older than 1 year, treated for malrotation at a single pediatric tertiary care center from 2000 to 2015. Data analyzed included demographics, presentation, imaging, intraoperative findings, and follow-up. Patients predisposed to malrotation were excluded. 246 patients were diagnosed with malrotation, of which 77 patients were older than 1 year of age. The most common presenting symptoms were vomiting (68%) and abdominal pain (57%). The most common method of diagnosis was UGI (61%). In 88%, the UGI revealed malrotation. 73 of 75 were confirmed to have malrotation at surgery. Intra-operatively, 60% were found to have a malrotated intestinal orientation and 33% with a non-rotated orientation. Obstruction was present in 22% with 12% having volvulus. Of those with follow-up, 58% reported alleviation of symptoms. Despite age malrotation should be on the differential given a variable clinical presentation. UGI should be conducted to allow for prompt diagnosis and surgical intervention.

We report a case of gastroschisis in male twins. Both twins were preterm and low birth weight, with intestinal malrotation; they were diagnosed by antenatal ultrasound at 20 weeks of gestation. Immediately after delivery, they underwent evaluation and early surgical one-stage repair under anesthesia. One of the twins was found to have duodenal perforation at laparotomy, at the horizontal part near Treitz ligament. Both twins stayed in hospital for 30 days and were in good health at discharge.

Although the principles of the Ladd’s procedure for intestinal malrotation in children have remained unchanged since its first description, in the era of minimally invasive surgery it is controversial whether laparoscopy is advantageous over open surgery. The aim of our study was to determine whether the surgical approach for the treatment of malrotation had an impact on patient outcome. Using a defined strategy (PubMed, Cochrane, Embase and Web of Science MeSH headings), two investigators independently searched for studies comparing open versus laparoscopic Ladd’s procedure in children. Case reports and opinion articles were excluded. Outcome measures included age at operation, time to full enteral feeding, length of hospital stay, and post-operative complications. Maneuvers were compared using Fisher’s exact test and meta-analysis was conducted using RevMan 5.3. Data are expressed as mean ± SD. Of 308 abstracts screened, 49 full-text articles were analyzed and nine (all retrospective) met our search criteria. Selected articles included 1003 patients, of whom 744 (74 %) underwent open surgery and 259 (26 %) laparoscopy. Patients who had open surgery were younger (0.9 ± 1.2 years) than those who underwent laparoscopy (2.6 ± 3 years; p

Chylous ascites is a rare entity in infants. An uncommon cause of chylous ascites is intestinal malrotation. We report a case of a neonate with heterotaxia-polysplenia syndrome and intestinal malrotation who presented with chylous ascites and subsequently developed duodenal obstruction from midgut volvulus. He was successfully treated with peritoneal drainage, surgical intervention, and nutritional support. A brief overview of the association of chylous ascites, intestinal rotational anomalies and heterotaxia syndromes is discussed.

Contains fulltext : 71270.pdf (Publisher’s version ) (Closed access) Intestinal malrotation (IM) and cardiovascular defects (CCVD) are both common congenital defects. We investigated the prevalence and types of CCVD in a 25-year IM population, and its association with post-IM-operative morbidity and mortality. Data on the type of CCVD, other congenital defects, syndromes, associations, post-IM-operative morbidity and mortality were retrospectively reviewed from the records of IM patients born between 1980 and 2005. Data were analyzed on (significant) differences between CCVD subgroups, and risk factors for both morbidity and mortality were calculated. Seventy-seven of 284 IM patients (27.1%) were diagnosed with a major or minor CCVD (37 and 40 patients, respectively). Syndromes and associations were more frequently diagnosed in patients with major than with a minor CCVD (67.6 vs. 40%, respectively). Post-IM-operative complications, although frequently observed (61%), did not differ between patients with major and minor CCVD. Physical CCVD signs before IM surgery increased post-IM-operative morbidity significantly (OR 4.0, 95% CI 1.4-11.0). Fifteen patients died (19.5%), seven due to cardiovascular cause. Mortality risk was increased by intestinal ischemia and post-IM-operative complications and by major CCVD after correction for age at weight at the time of IM operation. Congenital cardiovascular defects in children with intestinal malrotation are common, with high morbidity and mortality rates after IM operation. Elective IM surgery in young patients with CCVD should be performed in a centre with adequate paediatric cardiac care. Benefits of laparoscopic intervention need further study.

The malrotation and no well fixation anomalies of the digestive tract is also frequent in older child, young and adult, with characteristic and specific clinical presentation. Actually, the diagnostic and treatment seem to be late, after suffering prolonged symptoms and/or in emergency. We present nine cases of anomalies in the embryonic development of the digestive tract which were diagnosed and treated in infants or young, all above 2 years old. Eight cases were of more or less complete intestinal malrotation; one of them was a complete malrotation with an intrinsic duodenal stenosis associated (no bands of Ladd) and another one was a right paramesocolic hernia, always accompanied by malrotation. The association with other extra-digestive anomalies, especially urological, was 70%. The predominant symptom was intermittent abdominal pain (IAP)-80%-sometimes accompanied by vomiting (35%) and episodes of diarrhoea (25%). In all the cases, while the clinical background was early, diagnosis was late. Indeed, in 60% of the cases diagnosis was made intra-operatively in emergency surgical interventions. The imaging procedures employed were scanning and Doppler ultrasound, CT scan, and contrast gastrointestinal series (GIS). Up to 30% of errors in interpretation occurred, although they were eventually corrected with other tests. The most reliable diagnostic procedures were GIS and CT scan with contrast, although partial interpretation errors occurred with the latter procedure. Surgery was essential in 80% of the pre-operative cases, and in another two it was required as a preventative measure. Post-operatively, there was notable persistence of SBS in the cases of intestinal necrosis, and of other lesser symptoms in the rest. We conclude that: intestinal malrotations and malfixations are still being diagnosed very late, with serious systemic consequences such as intestinal obstructions or necroses, and prolonged clinical suffering. This could all be avoided if more attention were paid to the digestive symptoms associated with IAP, and to subocclusion or other abdominal phenomena (distension,...), together with, in the case of doubt about the findings with the previous procedures, the opportune imaging tests (e.g., abdominal Doppler ultrasound, CT scan with contrast, and barium GIS). Unlike other authors, we consider that the morbidity/mortality associated with cases of late diagnosis of these anomalies is high, and calls for earlier surgical treatment.

Two neonatal cases of cow's milk allergy showing bilous vomiting and massive bloody stool are presented. In both cases, laboratory data on admission showed no eosinophilia. The immediate upper gastrointestinal series of both cases demonstrated no evidence of intestinal malrotation, but revealed a narrowing of the duodenum and upper jejunal loops and marked stasis of barium in one case, suggesting an edema with inflammation. Rectal mucosal punch biopsy of the other case showed eosinophilic infiltration in the lamina propria. These findings suggested an allergic origin, useful information for avoiding confusion with some diseases requiring emergency surgery. In both cases, bleeding resolved soon after elimination of cow's milk from the diet. Thereafter both cases showed a moderate to marked increase in peripheral eosinophilia, while serum radioallergosorbent tests for cow's milk protein were negative. Definitive diagnosis of allergic enterocolitis is difficult because there is neither specific laboratory data nor tests. Allergic enterocolitis can occur in the early neonatal period and should be considered in differential diagnosis of any newborn developing gastrointestinal bleeding.

Background: It is accepted that children with volvulus require urgent surgery. However the long term sequelae and late complications of its surgical therapy are less well understood. We hypothesised that the surgical corrected intestinal malrotation have significant long term impact on the patients quality of life. Methods: Forty-six children with intestinal malrotation were operated on at a tertiary referral centre over a fifteen year period. Their charts were retrospectively reviewed and the patients were contacted. Results: The study revealed two distinct groups, those without complications 25 (54%) and those without 21 (46%). In the acute post operative period four (9%) patients had on going feeding difficulties and one (2%) developed chronic abdominal pain. However 12 (26%) required readmission within the first six months after the initial operation. Eleven (24%) patients were readmitted with acute bowel obstruction. Six (13%) patients required multiple admissions due to small bowel obstruction and six (13%) patients underwent further surgery for adhesion related obstruction. There were four (9%) deaths in the study group, three due to other medical conditions and one following small bowel obstruction. There was zero mortality immediately after the primary operation. There was no significant difference in the initial presentation, age and operative findings in those requiring further surgery and those who did not. Conclusions: We demonstrated that there is a significant long-term morbidity associated with intestinal malrotation even after corrective surgery. Detailed education about the potential for small bowel obstruction must be given to the parents of these children.

The association between gastroesophageal reflux (GER) and intestinal malrotation (IM) has been well described. Delayed or impaired gastric emptying in IM is thought to be a contributing factor in the development of gastroesophageal reflux disease (GERD). The current study assessed the role of malrotation in delayed gastric emptying in children with GERD. We also evaluated the interactions between GERD, malrotation, gastric pH abnormalities, and gastric dysmotility. Sixty-seven patients between 1 and 5 years of age (mean 3.08+/-1.2) and with symptoms of GER, such as emesis, reactive or recurrent lung disease, and/or growth retardation, were studied in 2001-2005. Upper and lower gastrointestinal contrast studies were performed for the diagnosis of malrotation. Gastric motility was evaluated with a liquid gastric emptying protocol. GER was documented by upper gastrointestinal studies, scintigraphy, and/or 24-h pH monitoring. In our series of 44 children with GERD, there was an unexpectedly high incidence of IM: 54.5% (24/44). IM has previously been known to occur in 25% of patients with GERD. GERD was found in 24 (82.7%) of 29 patients with IM. Mean nuclear gastric emptying (MNGE) was 51.6+/-8.04 min in patients with isolated GERD and 96.6+/-20.5 min in children with IM and GERD. There was a statistically significant difference in MNGE time (p0.05) between children with primary GERD and in those with GERD and IM. Esophageal pH monitoring showed that mean fraction time below pH 4 was 7.06+/-1.1% in patients with isolated GERD and 14.7+/-4.1% in patients with IM and GERD. GERD is common in children between 1 and 5 years old. Using gastric emptying studies and esophageal pH monitoring, we have shown that gastric dysmotility and esophageal pH abnormalities are highly prevalent, especially in children with malrotation compared with children with isolated GERD. These findings suggest that malrotation is an important factor responsible for delayed gastric emptying in GERD. Hence, we recommend that all infants and children with GERD and delayed gastric emptying undergo careful evaluation for malrotation.

Herniation through the foramen of Winslow is a rare variety of paraduodenal hernia. We report a 19-month-old child with obstructive jaundice due to midgut herniation through the foramen of Winslow with associated volvulus. The herniation and volvulus were precipitated by intestinal malrotation. The patient underwent extraamniotic silo repair of exomphalos major in the neonatal period. Investigation for malrotation is recommended after extraamniotic closure of exomphalos.

Intestinal malrotation associated with a volvulus requires immediate surgical intervention. The long-term sequelae of Ladd’s procedure and its complications are not well defined. We designed this study to investigate the long-term complications following operative intervention for intestinal malrotation. Patients who have undergone a Ladd’s procedure for malrotation from January 1999 till December 2008, from two tertiary centres, were identified using the Hospital Inpatient Enquiry system. Charts were analysed to obtain information about mode of presentation, method of diagnosis and postoperative outcomes. Patients were contacted for follow-up. One hundred and sixty-one patients were identified over the 10-year period with a postoperative follow-up time ranging from 2 months to 10 years. The median age at surgery was 9 days (1 day–12 years); 38 (23%) underwent elective Ladd’s procedure for malrotation. Thirty-eight patients had intraoperative incidental findings of a malrotation during different procedures; 120 (74.5%) patients were performed as an emergency procedure. Fourteen patients (8.7%) developed complications following surgery. Nine patients developed adhesive small bowel obstruction, five required operative adhesiolysis having failed conservative treatment. There was one case of recurrent volvulus and three mortalities in our series. In our experience, looking at 161 patients with a mean follow-up of 5 years Ladd’s procedure has a low postoperative morbidity and remains a vital treatment for malrotation in children.

Management of foreign body ingestion is usually nonsurgical unless atypical foreign bodies or anatomical variants are suspected. The present article illustrates a case of atypical foreign bodies ingestion in the presence of intestinal malrotation. The patient required surgical treatment and had an uneventful postoperative recovery. The article also reviews the cases reported in the literature of magnetic ingestion and the possible complications associated with it.

Waugh's syndrome (WS) is the association of intussusception and intestinal malrotation. The association has not been widely reported in the literature. In the only prospective study, Brereton et al. reported a high frequency of the association, which suggests non-random association. Six patients with this association presented to our unit over a 4-month period. The clinical findings and management are presented and discussed. We suggest that malrotation by its very nature is associated with a mobile right colon, which may be a prerequisite for intussusception.

The treatment of "asymptomatic" intestinal malrotation remains controversial, particularly beyond the neonatal period. Two cases illustrate the application of laparoscopy for correction of malrotation: one in an older child and another in an asymptomatic infant. Both patients recovered well and were discharged 2 days after surgery. Laparoscopic appendectomy and duodenocolonic dissociation allows excellent visualization of the duodenocolic and Ladd's bands and easily accommodates appendectomy. The availability of this minimal-access technique should encourage correction of malrotation in the asymptomatic patient.

We herein report the case of three siblings presenting with intestinal malrotation. Their medical history and circumstances of diagnosis are described. Barium meal demonstrated a minor duodenal anomaly in the mother. As far as we can ascertain, this is the third report of isolated familial malrotation in more than one generation, raising questions about its developmental mechanism. We thus highlight in what circumstances familial investigations should be undertaken in case of malrotation.

An abnormal cecum position is usually found in patients with intestinal malrotation. We report one case with intussusception and intestinal malrotation in a 10-month-old infant. An unusual radiologic imaging feature and also abnormal intussusception mass location are discussed.

Intestinal malrotation is most commenly complicated by volvulus in the first weeks of life. Early diagnosis and surgical intervention are needed to prevent gangrene of the midgut and peritonitis. When the diagnosis of intestinal malrotation is suspected, a plain X-ray film of the abdomen must be perfomed. In 4 of 21 cases we found a low intestinal obstruction, which was complicated by a high risk of intestinal gangrene and high mortality. These X-ray findings should lead to emergency surgery. If the plain X-ray film shows the “double bubble sign” (N = 14), then intestinal malrotation and volvulus as well as duodenal atresia are possible diagnosis. As both conditions require surgery, no further examinations are necessary. With a nonspecific pattern (N = 3), a barium meal should be performed in order to classify the disease type. We recommend that operation should be performed in most cases based on the findings on the plain X-ray film and that cases with low intestinal obstruction should be operated upon as emergencies.

Dear editor, We read the article of Demirbilek et al. on delayed gastric emptying and malrotation in gastroesophageal reflux disease with much interest [1]. The authors deal with several difficult issues at the same time. As regarding the issue of gastric emptying in children, what are the normal values in children? Hopefully normal values will soon become available with the less invasive C-acetate breath test [2]. Delayed gastric emptying has been correlated with many diseases, e.g. gerd, neurological impairment and intestinal malrotation. Moreover improvement of gastric emptying after fundoplication has been described as well. The reader wonders how many children were neurologically impaired and how many of these belonged to either group. When looking at the two curves of gastric emptying there were two, maybe three outliers in group II. As far as malrotation is concerned, it leaves little doubt that this is a spectrum. The question is not whether or not there is a malrotation but whether that particular malrotation needs treatment. Treatment seems only indicated when there are signs of obstruction or when there is an increased incidence of volvulus. A duodenojejunal flexure that is not located in a position where we think it should be is certainly not an indication on its own for surgical treatment. We have seen patients with a lower-than-normal insertion of the duodenojejunal flexure during laparoscopy but with a broad mesentery and good fixation of the mesenterial root. Under such circumstances, there is no indication for further surgery. The authors write that a dramatic rise in the number of operations for malrotation in their institution has taken place. We wonder about overdiagnosis. Laparoscopy is certainly helpful in avoiding unnecessary laparotomies. Lastly there is a relationship between intestinal malrotation and chronic intestinal pseudo-obstruction [3]. Correction of the malrotation does not correct symptoms in these patients. If there is a causal relationship between malrotation, delayed emptying and GERD as the authors suggest, then surgery should correct the problem. The authors did not provide any data on this issue.

Dear Sir, We read with great interest the article by Hamada et al. entitled ‘‘Postoperative intussusception after incidental appendicectomy’’. A sliding appendiceal inguinal hernia was diagnosed on the presented patient, who was a 12-month-old boy. The incidental appendicectomy followed by herniotomy was successfully performed. On the second post-operative day, the patient showed signs and symptoms of intussusception. The intussusception reduced spontaneously as general anaesthesia was induced, and the leading point of the ileocolic intussusception was observed as the inverted appendiceal stump [1]. We believe it was necessary that malrotation associated with intussusception could have been discussed by the authors in the presented paper. The association of intussusception and intestinal malrotation is named ‘‘Waugh s syndrome’’. lt was suggested that colonic mobility is a sine qua non of intussusception [2]. Tatekawa et al. reported that the mobile caecum associated with malrotation is suspected to be a factor allowing the terminal ileum to pass into the caecum and the caecum further into the distal colon [3]. The authors, in the presented case, reported that the inverted appendiceal stump or local oedema of the wall of the caecum or appendiceal stump may be a leading point, but did not enlighten us and discuss colonic mobility as a cause of intussusception, because, a sliding appendiceal inguinal hernia may be further evidence of the colonic mobility in the presented patient. A prospective study amongst 49 consecutive children with intussusception found a 40% incidence of malrotation [4]. We must be aware that the incidence of Waugh s syndrome is higher than expected. Therefore, we believe, every intussusception patient should be investigated, by upper and lower gastrointestinal series, for malrotation, so that the child can be protected from the life-threatening complications of volvulus and internal herniation.

Intestinal malrotation is well covered in the surgical literature from the point of view of operative management, but few reviews to date have attempted to provide a comprehensive examination of the topic from the point of view of aetiology, in particular genetic aetiology. Following a brief overview of molecular embryology of midgut rotation, we present in this article instances of and case reports and case series of intestinal malrotation in which a genetic aetiology is likely. Autosomal dominant, autosomal recessive, X-linked and chromosomal forms of the disorder are represented. Most occur in syndromic form, that is to say, in association with other malformations. In many instances, recognition of a specific syndrome is possible, one of several examples discussed being the recently described association of intestinal malrotation with alveolar capillary dysplasia, due to mutations in the forkhead box transcription factor FOXF1. New advances in sequencing technology mean that the identification of the genes mutated in these disorders is more accessible than ever, and paediatric surgeons are encouraged to refer to their colleagues in clinical genetics where a genetic aetiology seems likely.