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Background Adult midgut malrotation is a rare cause of an acute abdomen requiring urgent intervention. It may also present in the non-acute setting with chronic, non-specific symptoms. The objective of this study is to identify the clinical features, appropriate investigations and current surgical management associated with adult malrotation. Methods A systematic review was conducted according to PRISMA guidelines, identifying confirmed cases of adult malrotation. Patient demographics, clinical features, investigation findings and operative details were analysed. Results Forty-five reports met the inclusion criteria, totalling 194 cases. Mean age was 38.9 years ( n  = 92), and 52.3% were male ( n  = 130). The commonest presenting complaints were abdominal pain (76.8%), vomiting (35.1%) and food intolerance (21.6%). At least one chronic symptom was reported in 87.6% and included intermittent abdominal pain (41.2%), vomiting (12.4%) and obstipation (11.9%). Computerised tomography scanning was the most frequent imaging modality (81.4%), with a sensitivity of 97.5%. The whirlpool sign was observed in 30.9%; abnormalities of the superior mesenteric axis were the commonest finding (58.0%). Ladd’s procedure was the most common surgical intervention (74.5%). There was no significant difference in resolution rates between emergency and elective procedures ( p  = 0.46), but length of stay was significantly shorter for elective cases. ( p  = 0.009). There was no significant difference in risk of mortality, or symptom resolution, between operative and conservative management ( p  = 0.14 and p  = 0.44, respectively). Conclusion Malrotation in the adult manifests with chronic symptoms and should be considered as a differential diagnosis in patients with abdominal pain, vomiting and food intolerance.

PurposeIntestinal malrotation, characterized by abnormal intestinal positioning, can lead to severe complications like volvulus and internal hernias, especially in neonates and children. Our aim was to evaluate the diagnostic methods, treatment results and postoperative follow-up of pediatric patients treated for intestinal malrotation.MethodsThis retrospective study reviewed medical records of pediatric patients who underwent surgery for intestinal malrotation between January 2013 and January 2022. Data on demographics, symptoms, diagnostic approaches, surgical interventions, and postoperative outcomes were analyzed.ResultsThe study included 45 patients, with a male predominance (68.8%). Ages ranged from 1 day to 15 years, averaging 1.54 years. Presenting symptoms were acute abdomen (n = 21) and chronic abdominal pain with vomiting (n = 24). Diagnoses were established via physical exams and imaging, including upper gastrointestinal contrast studies and abdominal ultrasonography. All patients received the Ladd procedure, with some requiring necrotic bowel resection due to volvulus.ConclusionThe diagnosis and management of pediatric intestinal malrotation present significant challenges due to its variable symptoms and potential for life-threatening complications. Early and accurate diagnosis, followed by appropriate surgical management, is crucial. This study emphasizes the importance of diligent postoperative follow-up to identify and mitigate complications, particularly in younger and severely affected patients.

ContextDespite the advantages of ultrasound (US), upper gastrointestinal contrast series (UGI) remains the first-line diagnostic modality in the diagnosis of midgut malrotation and volvulus in children.ObjectiveEvaluate the diagnostic accuracy of US in the diagnosis of malrotation with or without volvulus in children and adolescents aged 0–21 years, compared with the reference standard (diagnosis by surgery, UGI, CT, MRI, and clinical follow-up individually or as a composite).Data sourcesWe searched the electronic databases Ovid-MEDLINE, Embase, Scopus, CINAHL, and the Cochrane library in October 2019 and updated on 18 August 2020.Study selectionStudies evaluating the diagnostic performance of US for diagnosis of midgut malrotation with or without volvulus in children (0–21 years).Data extraction and synthesisThe data were extracted independently by two authors and a bivariate model was used for synthesis.ResultsMeta-analysis of 17 cohort or cross-sectional studies and 2257 participants estimated a summary sensitivity of 94% (95% CI 89% to 97%) and summary specificity of 100% (95% CI 97% to 100%) (moderate certainty evidence) for the use of US for the diagnosis of malrotation with or without midgut volvulus compared with the reference standard. Subgroup analysis and meta-regression revealed better diagnostic accuracy in malrotation not complicated by volvulus, in the neonatal population and enteric fluid administration before US.ConclusionsModerate certainty evidence suggests excellent diagnostic accuracy and coupled with the advantages, a strong case exists for the use of abdominal US as the first-line diagnostic test for suspected midgut malrotation with or without volvulus in children and adolescents.

Currarino syndrome is a rare condition with an incidence of approximately 1 in 100,000. It is characterised by the triad of a sacral defect, a presacral mass and anal atresia or stenosis, and it is commonly associated with genetic mutations in the MNX1 gene.We report a case of an infant presenting with recurrent right gluteal abscess secondary to an infected sacrococcygeal teratoma, associated with anal stenosis. Extensive workup was performed to investigate for the source of her recurrent gluteal abscess and anal stenosis, leading to the diagnosis of Currarino syndrome. She underwent multiple surgical procedures, including excision of the infected teratoma, and remains on multidisciplinary surveillance for disease recurrence.Although sacrococcygeal teratomas are not uncommon, they rarely present as gluteal abscesses. Such atypical presentations are difficult to diagnose and can mimic common acute surgical conditions. We discuss the diagnostic and therapeutic challenges faced when managing such cases.

Purpose The purpose of the study was to evaluate our experience with enteric duplication cysts in 40 children during the past 26 years, while assessing the variability of their presentations and to propose an algorithm for surgical management. Methods We retrospectively analysed sex, age, clinical presentations, duplication site, surgical treatment, presence of ectopic tissue, complications, associated anomalies, and prognosis of 40 patients with gastrointestinal tract duplications who were surgically treated in our clinic. Results Overall, there was a predominance of boys (28 males, 70 %; 12 females, 30 %). The presenting symptom was vomiting in 23 patients, rectal bleeding in 11 patients, abdominal mass in 10 patients, abdominal pain in 9 patients, constipation in 6 patients, cough in 2 patients, and respiratory distress in 2 patients. In 30 patients, a complete excision of the cyst with additional segmental intestinal resection and anastomosis was performed. Cystectomy was performed in seven patients, while complete excision of the cyst with additional wedge resection was performed in two. A Wrenn procedure (mucosectomy) was performed in one patient. Conclusion Due to the variability in the site of enteric duplications, a wide range of presenting symptoms can exist, which is challenging for diagnosis. In children with a diagnosis of acute abdomen, enteric duplication cysts should be considered, and these children should be further investigated for additional skeletal, urogenital, and gastrointestinal system pathologies. Surgical treatment depends on the site and type of the cyst.

Purpose Prospective randomized comparison of the functional and cosmetic results of anterior sagittal anorectoplasty (ASARP) and posterior sagittal anorectoplasty (PSARP) in the management of intermediate anorectal malformations (ARMs) in girls was performed. Methods Thirty-eight girls with intermediate ARMs were randomly allocated to group A treated by ASARP technique and group B treated by PSARP technique: 14 girls from each group passed 30-month age for early functional assessment by Templeton and Holschneider scores, 18 cases passed 55 months for late functional evaluation. Results were compared statistically where P value ≤ 0.05 was considered significant. Results Cosmetic satisfaction was higher in ASARP group with a median score of 3 compared to 2 in PSARP group. Functional results were assessed early at median of 33.5 months post-repair where Templeton score was 4 versus 3.75 and Holschneider score was 11 versus 11. Late functional assessment was done for 8 cases from each group where Templeton score was 4 versus 4, while Holschneider score was 12 versus 11. Conclusions ASARP is an optimal technique for treatment of intermediate ARM in girls. Cosmetic results were superior to PSARP. Despite the observations that functional results give higher scores in ASARP group versus PSARP group, no statistical significance could be reached.

Midgut malrotation with volvulus is a surgical emergency with potentially devastating outcomes which include short gut syndrome necessitating long-term parenteral nutrition, overwhelming sepsis, and death. The clinical presentation is most frequently with bilious vomiting in the first days–weeks of life, which is non-specific and common. Timely imaging investigation is therefore crucial to prevent delays to diagnosis and treatment and avoid unnecessary surgical exploration in infants with non-surgical bilious vomiting. Fluoroscopic upper gastrointestinal contrast series (UGI) has been the first-line imaging modality to investigate midgut malrotation at pediatric surgical centers worldwide. However, there is a growing body of evidence to indicate that ultrasound (US) has greater diagnostic accuracy than UGI in this context. Furthermore, US offers the benefits of accessibility, portability, lack of ionizing radiation, and the ability to identify alternative diagnoses, and is beginning to attract significant attention and consideration in the literature. Over the last 3 years, we have transitioned to an “US-first” pathway for the investigation of midgut malrotation in infants with bilious vomiting. This pictorial essay illustrates our comprehensive approach, describes unique troubleshooting techniques, and highlights the variably published pitfalls we have encountered with the aim of encouraging wider adoption.

Gallbladder agenesia is a rare congenital anomaly that is frequently overlooked in clinical practice, often misdiagnosed as chronic sclero-fibrous cholecystitis or cholelithiasis. Failure to recognize it intraoperatively can prompt unnecessary and hazardous exploration of the biliary tree and liver, increasing the risk of iatrogenic injuries. This report describes a rare case of gallbladder agenesia coincidentally discovered during surgery, alongside adult midgut malrotation—both diagnosed intraoperatively. The patient presented with symptoms mimicking biliary colic, which, in 23–55% of gallbladder agenesia cases, lead to symptomatic presentation. Preoperative ultrasound commonly misinterprets the absent gallbladder as a shrunken, hypoplastic, or contracted organ due to artifacts from intestinal gas or adjacent structures. In the presented case, symptoms and ultrasound misinterpretation were likely attributable to the malrotated jejunum positioned beneath the liver. Magnetic resonance imaging (MRI), particularly MR cholangiography, is recommended when ultrasound findings are inconclusive or suggest a scleroatrophic gallbladder, though misinterpretation remains possible due to the condition’s rarity and low clinical awareness. Intraoperatively, overzealous dissection poses the greatest risk. Authors advocate aborting the procedure upon suspicion of agenesia, followed by postoperative confirmatory imaging (CT or MRI), rather than extensive exploration. Adult midgut malrotation is also uncommon, with only 10% diagnosed in adulthood. Most cases are asymptomatic or cause vague abdominal symptoms (pain, nausea, bilious vomiting), often mistaken for biliary pathology. The patient exhibited duodenal nonrotation with right jejunal positioning and normal colon (DNJRCR type). In asymptomatic incidental findings, a conservative approach is recommended. In summary, heightened suspicion for gallbladder agenesia is essential in ambiguous ultrasound reports. Preoperative MRI can prevent unnecessary surgery, while intraoperative recognition should prompt procedure termination and postoperative imaging to avoid iatrogenic complications.

PurposeCongenital duodenal obstruction (CDO) is associated with trisomy 21 (T21), or Down’s syndrome, in around a third of infants. The aim of this study was to explore the impact of T21 on the epidemiology, management, and outcomes of infants with CDO.MethodsData were prospectively collected from specialist neonatal surgical centres in the United Kingdom over a 12 month period from March 2016 using established population-based methodology for all babies with CDO. Infants with T21 were compared to those without any chromosomal anomaly.ResultsOf 102 infants with CDO that underwent operative repair, T21 was present in 33 [32% (95% CI 23–41%)] babies. Cardiac anomalies were more common in those with T21 compared to those without a chromosomal anomaly (91 vs 17%, p < 0.001), whereas associated gastrointestinal anomalies were less common in infants with T21 (3 vs 12%, p = 0.03). Surgical management was not influenced by T21. Time to achieve full enteral feed, need for repeat related surgery, and mortality were similar between groups. Infants with T21 had a longer median initial inpatient stay (23 vs 16.5 days, p = 0.02).ConclusionsInfants with T21 have a higher incidence of cardiac anomalies and a longer initial inpatient stay; however, it does not change CDO management or outcomes. This information is important for prenatal and postnatal counselling of parents of infants with CDO and T21.

This study investigated neurodevelopment and risk factors in children surgically treated for congenital gastrointestinal anomalies (CGIA), excluding those with known high-risk factors such as low birth weight or chromosomal anomalies. Data of children born between 2008 and 2015 who underwent surgical treatment for CGIA were retrieved from the Korean National Health Insurance Database. CGIA included esophageal atresia, duodenal atresia, jejunoileal atresia, anorectal malformations, and congenital megacolon. Neurodevelopmental impairment (NDI) was defined as Korean Ages and Stages Questionnaire scores below the determined cut-off or Korean Developmental Screening Test scores < 2 standard deviations at 3 years of age. Children with CGIA had a significantly higher risk of NDI than controls (6.2% vs. 2.7%, p  < 0.001). Growth failure was correlated with NDI. Longer durations of oxygen support (adjusted odds ratio [aOR], 1.037; 95% confidence interval [CI], 1.013–1.063), mechanical ventilation (aOR, 1.053; 95% CI, 1.018–1.089), and number of surgeries (aOR, 1.137; 95% CI, 1.016–1.273) were significantly associated with NDI. These findings emphasize that cautious yet proactive neurodevelopmental monitoring is crucial in affected children, ensuring timely intervention and that excessive concern among families is unnecessary.