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BACKGROUND A congenital defect known as intestinal malrotation, which has only rarely been described in adults, is most frequently identified in children. For life-threatening consequences, such as intestinal ischemia and death, to be avoided, prompt detection is essential. We report a rare case of adult intestinal malrotation to emphasize the difficulty in diagnosis and surgical treatment. CASE REPORT An 18-year-old Saudi woman presented with generalized, gradually intermittent abdominal pain accompanied by nausea, and non-bilious vomiting. An abdominal computed tomography (CT) scan showed that the small-bowel loops were identified at the right side of the abdomen with the duodenojejunal junction at the right side or just right paramedian level as well, the superior mesenteric artery and vein had an inverted relationship, and the cecum was observed in the left lumber region. She was treated by open exploratory laparotomy adhesiolysis, the cecum was mobilized, the small-bowel adhesion under the liver was released, and an appendectomy was carried out. Postoperatively, she was managed with a double antibiotic regimen of ceftriaxone and metronidazole for 7 days. CONCLUSIONS Although intestinal malrotation is uncommon in adults and very difficult to identify, it is frequently encountered in young populations. Adults with long history of vague or unexplained abdominal pain should undergo radiological studies to assess findings of intestinal malrotation. In our case, the result of a CT scan helped to make the diagnosis preoperatively.

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., (© 2024 Asia Endosurgery Task Force and Japan Society of Endoscopic Surgery and John Wiley & Sons Australia, Ltd.)

BACKGROUND Reversed intestinal malrotation is an extremely rare disease, with an incidence of 1 in 250 000. In Japan, application of robotic-assisted colorectal cancer surgery is expected to increase. There are no reports of robot-assisted surgery for cecal cancer with reversed intestinal malrotation. CASE REPORT An 84-year-old Japanese man with epigastric pain and abdominal distention was referred to our hospital's Department of Gastroenterology for thorough examination. Colonoscopy revealed a semicircumferential type 2 tumor in the cecum and ascending colon. Gastrografin contrast study showed that the large intestine was entirely on the patient's right side and the small intestine was shifted to the left side. Contrast-enhanced computed tomography revealed enlarged lymph nodes near the tumor, and masses were observed at the liver, which were believed to be metastases. Following examination, reversed intestinal malrotation and concurrent cecal cancer was diagnosed. The patient was referred to our department for surgery and underwent robot-assisted ileocecal resection with D3 lymphadenectomy. The postoperative course was favorable, and patient was discharged on the sixth postoperative day, without complications. According to the Japanese Classification of Colorectal, Appendiceal, and Anal Carcinoma 9th edition, the pathological diagnosis was pT4b (ileum), pN1b, cM1a (H1 [grade A]), and pStage IVa cancer. After considering tumor stage and patient's overall condition in consultation with his family, we decided against palliative systemic therapy. The patient was provided with best supportive care. CONCLUSIONS Robot-assisted surgery might be useful in manipulation of the dissection of adhesions, owing to its capacity for high-resolution 3-dimensional imaging and forceps manipulation, using articulated functions.

Enteric duplication has cystic and tubular varieties. A male infant presented with a large cystic, well-demarcated mass in the right flank. On exploratory laparotomy, multiple cystic and tubular lesions were present adjacent to the mesenteric border of the small bowel along with malrotation of the small bowel. The tubule-cystic structure was excised along with the involved normal bowel segment and Ladd's procedure was performed. Histopathological evaluation revealed an intestinal duplication cyst. The occurrence of midgut malrotation and volvulus along with duplication is uncommon. The cyst's substantial size could have been an aetiological factor for malrotation and volvulus. The child's small bowel had adapted remarkably with time. This case highlights a new variant of duplication cysts., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: Intestinal 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., Methods: This 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., Results: The 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., Conclusion: The 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., (© 2024. The Author(s).)

Alimentary tract duplications are heterogenous congenital anomalies of the digestive tract. Their form of presentation is varied, and they may lead to different complications, depending on their natural course. Infection is a rare complication, but it cannot be ignored because of its severity. Here we describe the case of an otherwise healthy 2-year-old girl with an atypical complication of alimentary tract duplication: septic shock. She initially consulted due to abdominal distension and pain associated with a palpable abdominal mass. The imaging studies showed a partial fluid septation in the right side of the abdomen. During hospitalization, an intratumoral infection developed, which progressed to septic shock. The patient responded favorably to medical treatment for shock, and surgical resection was subsequently performed. The pathology report confirmed the presence of alimentary tract duplication., (Sociedad Argentina de Pediatría.)

Introduction:  Intestinal malrotation (IM) is characterized by abnormal intestinal rotation and fixation predisposing to a risk of midgut volvulus. The aim of this study was to describe the clinical presentation and outcome of IM from birth through childhood., Materials and Methods:  This was a retrospective study of children with IM managed at a single center between 1983 and 2016. Data were retrieved from medical records and analyzed., Results:  Three hundred nineteen patients were eligible for the study. Using strict inclusion and exclusion criteria, 138 children were included. Vomiting was the most common symptom up to 5 years of age. At 6 to 15 years of age, abdominal pain was the predominant symptom. One hundred twenty-five patients underwent a Ladd's procedure and of 124 patients with available data, 20% had a postoperative complication (Clavien-Dindo IIIb-V) within 30 days. The odds ratio to develop postoperative complications was significantly increased in extremely preterm patients ( p  = 0.001) and in patients with severely affected intestinal circulation ( p  = 0.006). Two patients had intestinal failure due to midgut loss after midgut volvulus, one of whom needed intestinal transplantation. Four patients, all extremely preterm, died related to the surgical procedure. In addition, seven patients died of reasons not related to IM. Fourteen patients (11%) had adhesive bowel obstruction and one patient had recurrent midgut volvulus requiring surgical treatment., Conclusions:  IM presents with different symptoms through childhood depending on age. Postoperative complications are common after Ladd's procedure, particularly among extremely preterm infants and patients with severely affected circulation caused by midgut volvulus., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Developmental disorders of the gastrointestinal tract comprise a broad spectrum of congenital malformations of different etiologies and locations from the mouth to the anus., Methods: The authors present the most important malformations of the gastrointestinal tract on the basis of basic and current reviews., Results: Gastrointestinal developmental disorders occur both sporadically and in connection with malformation syndromes. Symptoms are highly variable and range from postnatal emergencies to asymptomatic abnormalities, which may be incidental radiological findings. Prenatal ultrasound examinations can often identify gastrointestinal developmental disorders at an early stage. Here, fetal magnetic resonance imaging can be a useful addition to the diagnostic process. In the first few days of life, simple X‑ray overview images, supplemented by images after the administration of contrast medium, are often sufficient., Conclusion: Many patients with a malformation of the gastrointestinal tract require lifelong medical care, so that not only pediatric radiologists need specific knowledge about this group of diseases., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Objective:  Intestinal volvulus in the neonate is a surgical emergency caused by either midgut volvulus (MV) with intestinal malrotation or less commonly, by segmental volvulus (SV) without intestinal malrotation. The aim of our study was to investigate if MV and SV can be differentiated by clinical course, intraoperative findings, and postoperative outcomes., Methods:  Using a defined search strategy, two investigators independently identified all studies comparing MV and SV in neonates. PRISMA guidelines were followed, and a meta-analysis was performed using RevMan 5.3., Results:  Of 1,026 abstracts screened, 104 full-text articles were analyzed, and 3 comparative studies were selected (112 patients). There were no differences in gestational age (37 vs. 36 weeks), birth weight (2,989 vs. 2,712 g), and age at presentation (6.9 vs. 3.8 days). SV was more commonly associated with abnormal findings on fetal ultrasound (US; 65 vs. 11.6%; p  < 0.00001). Preoperatively, SV was more commonly associated with abdominal distension (32 vs. 77%; p  < 0.05), whereas MV with a whirlpool sign on ultrasound (57 vs. 3%; p  < 0.01). Bilious vomiting had similar incidence in both (88 ± 4% vs. 50 ± 5%). Intraoperatively, SV had a higher incidence of intestinal atresia (2 vs. 19%; p  < 0.05) and need for bowel resection (13 vs. 91%; p  < 0.00001). There were no differences in postoperative complications (13% MV vs. 14% SV), short bowel syndrome (15% MV vs. 0% SV; data available only from one study), and mortality (12% MV vs. 2% SV)., Conclusion:  Our study highlights the paucity of studies on SV in neonates. Nonetheless, our meta-analysis clearly indicates that SV is an entity on its own with distinct clinical features and intraoperative findings that are different from MV. SV should be considered as one of the differential diagnoses in all term and preterm babies with bilious vomiting after MV was ruled out-especially if abnormal fetal US and abdominal distension is present., Competing Interests: None declared., (Thieme. All rights reserved.)

Aim: Intestinal malrotation (IM) represents a rare congenital anomaly in adults, although it is more common during infancy. This condition originates during embryonic development due to incomplete rotation of the midgut around the superior mesenteric vessels. The primary aim of this case study is to emphasize the importance of surgeon awareness and recognition of this congenital anomaly during laparoscopic gastric surgery to avoid serious complications., Case Presentation: A 45-year-old male presented to the clinic with a complaint of vague epigastric pain for two months. The patient underwent a comprehensive clinical evaluation, including laboratory tests, endoscopic examination, and radiological imaging. The diagnostic workup revealed early-stage gastric cancer. Incidentally, radiological studies also demonstrated a congenital non-rotation of the small bowel. Given the patient's congenital anatomical anomaly, a laparoscopic total gastrectomy was performed employing a reversed C-shaped Roux-En-Y limb reconstruction. Postoperative recovery was uneventful, and the patient was discharged in stable condition., Results: The patient was doing well at the 2-year follow-up, there were no complications related to the form of anastomosis, and the pathological result was comparable to that of patients with non-rotating small bowls., Conclusions: Intestinal malrotation is an uncommon asymptomatic congenital abnormality in adults. During laparoscopic gastric surgery, the surgeon should identify this anomaly to optimize surgical approaches, particularly during anastomosis formation. Accurate identification and appropriate management of intestinal malrotation are crucial to mitigate potential postoperative complications, including twisting, obstruction, tension, and anastomosis leak.

Abdominal adhesion occurs commonly in clinical practice, causing unfavorable symptoms and readmission. The ileostomy operation is a common surgical procedure and we utilized this model to evaluate abdominal adhesion. Adhesion grade score was calculated in 35 patients (Cohort 1) and subjected to correlation and receiver operating characteristic analysis. Then 98 consecutive patients (Cohort 2) who underwent ileostomy and ileostomy closure were included into a retrospective study. Logistic regression analysis was performed, and the risk of small bowel obstruction was also assessed. The time of ileostomy closure correlated with adhesion grade score in Cohort 1, justifying its use as an indicator of abdominal adhesion. All patients in Cohort 2 were then divided into the high- and low-adhesion group. A multi-variable logistic regression analysis indicated that type of surgery and peritoneum suture during ileostomy were significant factors affecting the risk of abdominal adhesion. Abdominal adhesion had the trend to prolong the length of stay postoperatively without increasing the risk of bowel obstruction. Nine patients suffered bowel obstruction, and age older than 65 significantly increased the risk. We proposed the ileostomy procedure to be a model of abdominal adhesion, and the operative time of ileostomy closure could be used as an alternative of adhesion score. Type of surgery and peritoneum suture may be risk factors of abdominal adhesion. Older age increased the risk of small bowel obstruction after ileostomy surgery., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Congenital pyloric atresia (CPA) is a rare condition that presents as gastric outlet obstruction in the first few weeks of life. Isolated CPA typically carries a good prognosis but when associated with other conditions such as multiple intestinal atresia or epidermolysis bullosa (EB), the outcomes are generally poor. This report describes a four-day-old infant who presented with nonbilious emesis and weight loss in whom an upper gastrointestinal contrast study revealed gastric outlet obstruction determined to be consistent with pyloric atresia. The patient underwent operative repair via Heineke-Mikulicz pyloroplasty. Postoperatively, the patient continued to have severe diarrhea and was found to have desquamative enteropathy though had no skin findings consistent with EB. This report emphasizes consideration of CPA as a differential diagnosis for neonates presenting with nonbilious emesis and demonstrates the association between CPA and desquamative enteropathy without EB., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

An infant girl first presented with recurrent episodes of non-bilious vomiting, having had five hospitalisations over the following months because of dehydration. Laboratory data showed no inflammatory response, normal pancreatic amylase, but increased lipase levels (between 67 and 425 U/L). Several abdominal ultrasound studies suggested an intestinal duplication cyst on left hypochondrium and, later, a dilated and irregular pancreatic duct. CT showed a bifid tailed pancreas and a change in the cyst's characteristics. A communication with the pancreatic duct was hypothesised, which was confirmed on MR cholangiopancreatography. On laparoscopic surgery, the cyst was confirmed to be at the end of the caudal side of the pancreatic bifid tail, having no communication with the stomach. Cystectomy with partial pancreatectomy was performed with pathological findings confirming a gastric duplication cyst originating from the pancreatic bifid tail. At latest follow-up, 4 months after surgery, she remains asymptomatic., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: The authors report no conflicts of interest.

Aim of the Study: We conducted a nationwide survey of persistent cloaca (PC) to determine its current status in Japan. This study clarifies the potential risk factors for defecation problems in patients with PC., Methods: Patient information was obtained via questionnaire, and a total of 213 PC patients who responded to a questionnaire on defecation problems and their bowel functions were enrolled in this study. We evaluated the constipation, incontinence, and soiling as bowel functions. Univariate and multivariate analyses were performed using a logistic regression analysis to clarify the risk factors for defecation problems., Results: Of 213 patients with PC, 55 (25.8%) had defecation problems. A multivariate logistic regression analysis showed that sacral agenesis, as an associated anomaly, was significantly associated with defecation problems (odds ratio [OR] 3.19, 95% confidence interval [CI] 1.11-9.16, p = 0.03). The other multivariate logistic regression analysis showed that the PC patients who underwent antegrade continence enema and regularly took laxatives after anorectoplasty had defecation problems (OR 12.4, 95% CI 2.35-65.6, p = 0.003, OR 2.84, 95% CI 1.24-6.55, p = 0.01)., Conclusion: Sacral agenesis is the potential risk factor of defecation problems in the patients with PC who underwent anorectoplasty. Those patients require vigorous defecation management., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Aims: To describe the clinical and evolutionary aspects of the primary closure of exstrophy at the CHU Gabriel Touré., Materials and Methods: This was a retrospective and prospective study carried out from January 2014 to December 2019 in all the children admitted and operated on for bladder exstrophy at the CHU Gabriel Touré., Results: We collected 35 cases of exstrophy, ie25 boys and 10 girls. The mean age at diagnosis was 4.8 months. The bladder plate was both normal and budded, ie 28.6% of cases. Plaque infection was found in 45.7%. A malformation was associated in 34.3% of cases. Primary plaque closure was achieved in all of our patients. Postoperative morbidity was 28.6% of cases and mortality 11.4% of cases., Conclusion: Bladder exstrophy is a rare malformation of the urogenital sphere, its management is complex and its mortality is not null., (Le comité de rédaction se réserve le droit de renvoyer aux auteurs avant toute soumission à l'avis des lecteurs les manuscrits qui ne seraient pas conformes à ces modalités de présentation. En outre il leur conseille de conserver un exemplaire du manuscrit, des figures et des tableaux.)

Gastrointestinal duplications are rare congenital malformations occurring in embryonic period of development of digestive system. These abnormalities are usually found in infancy or early childhood. Clinical presentation is extremely diverse depending on dimensions, localization and type of duplication. The authors present duplication of antral and pyloric parts of the stomach, the 1st segment of the duodenum and pancreatic tail. Mother with a 6-month-old child turned to the hospital. According to the mother, the child was sick for about 3 days when episodes of periodic anxiety first appeared. Upon admission, abdominal neoplasm was suspected after ultrasound. On the second day after admission, anxiety increased. There was impairment of appetite, and the child rejected food. Abdominal asymmetry in umbilical area was observed. Considering clinical data on intestinal obstruction, emergency transverse right-sided laparotomy was performed. A tubular structure was found resembling intestinal tube was found between the stomach and transverse colon. Surgeon found duplication of antral and pyloric parts of the stomach, the 1st segment of the duodenum and its perforation. During further revision, additional pancreatic tail was diagnosed. En-bloc resection of gastrointestinal duplications was carried out. Postoperative period was uneventful. Enteral feeding was initiated after 5 days, and the patient was transferred to surgical unit. The child was discharged after 12 postoperative days.

Purpose: Fetuses with persistent cloaca are known to develop urine or meconium backflow into the abdominal cavity caused by obstruction of the common channel, thus leading to fetal peritonitis with fetal ascites. We analyzed the impact of prenatal fetal ascites on postnatal clinical features and management., Methods: This retrospective single-center cohort study was conducted to compare the perinatal parameters of patients with isolated persistent cloaca who were born and treated at our hospital between 1991 and 2021. The clinical features and management of those with and without fetal ascites were compared., Results: Among the 17 eligible patients, fetal ascites were recognized in seven. The occurrence of fetal ascites was significantly related to preterm birth, higher birth weight z-score, birth via emergency cesarean delivery, low Apgar scores at 1 min and 5 min, higher C-reactive protein levels at birth, longer duration of oxygen administration, the need for a urinary drainage catheter at initial discharge, and shorter neonatal hospital stays., Conclusions: The postnatal management of patients with persistent cloaca with fetal ascites differed significantly from that of patients without fetal ascites. For patients with unexplained fetal ascites, magnetic resonance imaging may be helpful for determining the definite diagnosis of persistent cloaca., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Currarino Syndrome (CS) is a rare autosomal dominant genetic disorder that is defined by a triad of: presacral mass, anorectal malformations, and sacral bone dysplasia. Once discovered, these lesions are often surgically treated to avoid life threatening complications such as meningitis and malignant transformation of a sacral teratoma. As this syndrome is usually diagnosed in childhood, accurate diagnosis in adults presenting with this syndrome can be challenging and delay treatment. We present a case report with diagnostic and surgical management strategies of CS presenting in an elderly patient with accompanying review of literature., Methods: We performed a literature review by searching PubMed, Ovid Embase, and Scopus electronic databases with the predetermined inclusion criteria of cases of CS in the adult population., Results: A 70-year-old male with newly diagnosed CS and meningitis successfully underwent resection of his lesion as an interdisciplinary case between neurosurgery and colorectal surgery. At six-month follow up, the patient reports resolution of constipation and urinary symptoms, no longer has signs of infection, and remains neurologically full strength in his lower extremities. A review of literature revealed only 5 previously reported cases of CS presenting in the adult population with 3 of these cases requiring surgical intervention., Conclusion: Currarino Syndrome (CS) is an autosomal dominant genetic disorder characterized by a presacral mass, sacral bony deformities, and anorectal malformations. It is usually diagnosed in pediatric age group. In this article, we present a case of a 70-year-old male presenting with meningitis, encephalopathy, and gastrointestinal disturbances., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Rationale: Upper gastrointestinal hemorrhage (UGIH) is defined as hemorrhage originating from the gastrointestinal tract proximal to the ligament of Treitz. The causes of UGIH include esophagitis, gastritis, peptic ulcers, Mallory-Weiss syndrome, and cancer. However, a rare cause of UGIH, such as an accessory spleen, may lead to serious complications if left untreated and can sometimes be very difficult to diagnose preoperatively., Patient Concerns: An 18-year-old man was admitted to the Department of Gastroenterology of our hospital due to "repeated black stool for 2 months with aggravation, accompanied by hematemesis for 9 days." He denied any history of hepatitis, trauma, or surgery., Diagnosis: Laboratory evaluation revealed severe anemia (hemoglobin, 6.4 g/dL). Computed tomography revealed a mass measuring 127 mm in its largest dimension, located in the upper left abdomen, with varicose veins in the gastric fundus. Moreover, distended blue-purple tortuous veins were observed by gastroscopy in the gastric fundus. We believed the mass was likely an abnormally proliferating accessory spleen; however, the causes of severe anemia and gastrointestinal hemorrhage were unknown., Interventions: After discussion in a multidisciplinary conference, the mass was completely resected laparoscopically, and the subserosal veins in the gastric fundus were sutured using absorbable threads., Outcomes: After the surgery, the patient recovered uneventfully without any complications. Clinicopathological examination showed that the mass was chronic congestive splenomegaly. Gastrointestinal hemorrhage secondary to an abnormally proliferating accessory spleen was confirmed as the diagnosis. Laboratory evaluation revealed hemoglobin at 12.1 g/dL 2 months after surgery. At the 12-month follow-up, the patient showed no recurrence of gastrointestinal hemorrhage., Lessons: UGIH caused by accessory spleen is extremely rare. This entity should be considered in differential diagnosis of gastrointestinal hemorrhage. Surgical intervention is necessary for timely diagnosis and treatment in case of gastrointestinal hemorrhage in critical clinical situations., Competing Interests: All authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Midgut malrotation is a rare congenital abnormality resulting from failure of complete intestinal rotation and subsequent fixation during early fetal development. There appeared to be no obvious symptoms in most patients, and a few patients may exhibit symptoms similar to hyperemesis gravidarum, such as nausea and vomiting. Here, we present a case of midgut malrotation presenting as hyperemesis gravidarum., Patient Concerns: A 27-year-old woman with an intrauterine pregnancy of 27 + 6 weeks complained of severe nausea and vomiting for 2 weeks., Diagnosis: Magnetic resonance imaging showed obvious dilatation in the proximal part of the duodenum and gastric cavity and the absence of a duodenal path dorsal to the superior mesenteric artery, which was diagnosed as midgut malrotation., Interventions: Considering that the patient's vital signs were stable, without manifestation of peritonitis or the risks of surgery to the fetus, conservative treatment was adopted. Unfortunately, the fetus developed severe hydrocephalus at 32 weeks. The patient and her family decided to abandon the fetus, and a mid-trimester-induced abortion was performed., Outcomes: The related symptoms completely disappeared after delivery, and the relevant examination after discharge also confirmed the presence of midgut malrotation without gastrointestinal discomfort within 1 year after delivery., Lessons: Midgut malrotation can be considered as a differential diagnosis of hyperemesis gravidarum. Conservative treatment under close monitoring is desirable in pregnant women diagnosed with midgut malrotation., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Competing Interests: Competing interests: None declared.

The present study aims to report a rare case of obstructive choledocholithiases found in an elderly female with heterotaxy syndrome (HS) with polysplenia, and to review the available literature on hepatobiliary pathologies associated with this syndrome. We present the case of an 82-year-old female patient with multiple comorbidities who presented to our hospital with abdominal pain and jaundice. An abdominal ultrasound and subsequent computed tomography showed several obstructive choledocholithiases. Incidentally, an abnormal visceral arrangement consistent with HS with polysplenia was observed, including midline liver, semi-annular pancreas, intestinal malrotation, preduodenal portal vein and other vascular anomalies. Accordingly, the patient was diagnosed with cholangitis secondary to obstructive choledocholithiases and HS with polysplenia. She was admitted to the hospital for endoscopic retrograde cholangiopancreatography after medical stabilization. Unfortunately, her condition rapidly worsened and died a few hours after hospitalization due to septic shock. Only one case of choledocholithiases in HS has been previously reported in the literature. It is unclear whether the abnormal arrangement of abdominal organs may have a significant role in the development of this complication. Although biliary drainage might be hindered in the presence of anatomical variants such as (semi-)annular pancreas, intestinal malrotation and preduodenal portal vein, further studies are needed to determine if there is an association between these anomalies and the development of choledocholithasis., (© 2021. Japanese Society of Gastroenterology.)

Background: Gastrointestinal duplication is a rare developmental anomaly that can be present anywhere along the GI tract, most often being found in ileum. The purpose of this study is to share our experience in evaluation of the presentation, investigations, management challenges and complications of patients with this very rare condition., Methods: This descriptive case series was conducted at the Department of Paediatric Surgery, National Institute of Child Health Karachi, Pakistan, from April 2018 to October 2019. Data was analysed with regard to age, clinical presentation, investigations, surgical procedures, site and type of lesion, histopathology, complications and outcomes., Results: A total of five patients were managed in one and half year. The patients' ages ranged from antenatally diagnosed foetus to 12 years old child. New-born who presented with antenatal diagnosis of abdominal cyst turned out to have duodenal duplication cyst. Among other four were thoracoabdominal duplication cyst, gastric duplication, jejunal duplication and ileal duplication, last two presented with perforation. Other presentations were abdominal pain, swelling and vomiting. Diagnosis was made on clinical ground, x-ray of abdomen, ultrasound and computed tomography. All cysts were resected successfully and patients remained asymptomatic till one year follow up except one patient who expired postoperatively due to liver failure., Conclusions: Enteric duplication can present in variety of ways depending on anatomical location. Prompt diagnosis and complete excision of cyst is the aim of treatment. However, these rare types of duplication are a challenge to operating surgeons.

Introduction: Intestinal malrotation is life-threatening and often presents during infancy with bilious vomiting. The prevalence and presentation among extremely premature infants are unknown., Methods: 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)., Results: 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., Conclusions: 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., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Aim: Children with congenital gastrointestinal malformations may be at risk of neurodevelopmental impairment due to challenges to the developing brain, including perioperative haemodynamic changes, exposure to anaesthetics and postoperative inflammatory influences. This study aggregates existing evidence on neurodevelopmental outcome in these patients using meta-analysis., Method: PubMed, Embase and Web of Science were searched for peer-reviewed articles published until October 2019. Out of the 5316 unique articles that were identified, 47 studies met the inclusion criteria and were included. Standardised mean differences (Cohen's d) between cognitive, motor and language outcome of patients with congenital gastrointestinal malformations and normative data (39 studies) or the studies' control group (8 studies) were aggregated across studies using random-effects meta-analysis. The value of (clinical) moderators was studied using meta-regression and diagnostic subgroups were compared., Results: The 47 included studies encompassed 62 cohorts, representing 2312 patients. Children with congenital gastrointestinal malformations had small-sized cognitive impairment (d=-0.435, p<0.001; 95% CI -0.567 to -0.302), medium-sized motor impairment (d=-0.610, p<0.001; 95% CI -0.769 to -0.451) and medium-sized language impairment (d=-0.670, p<0.001; 95% CI -0.914 to -0.425). Patients with short bowel syndrome had worse motor outcome. Neurodevelopmental outcome was related to the number of surgeries and length of total hospital stay, while no relations were observed with gestational age, birth weight, age and sex., Interpretation: This study shows that children with congenital gastrointestinal malformations exhibit impairments in neurodevelopmental outcome, highlighting the need for routine screening of neurodevelopment during follow-up., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY. Published by BMJ.)

We report a case of reversed rotation (RR) of the midgut, the rarest form of intestinal malrotation (IMR), revealed by a chronic volvulus, in a 42-year-old woman presenting with episodes of abdominal pain occurring after heavy meals. The complete preoperative diagnosis was obtained by contrast-enhanced computed tomography (CT). Classical signs of volvulus were associated with typical findings of RR comprising an unusual position of the third duodenum in front of the mesenteric vessels and a very unusual location of the transverse colon behind these vessels. Complete absence of fixation of the right colon allowed secondary volvulus. A detailed reminder of the embryology of IMR and RR is presented., (© 2021. The Author(s), under exclusive licence to Springer-Verlag France SAS part of Springer Nature.)

Background: Intestinal volvulus in a neonate, complete or segmental, is a true surgical emergency, which when the diagnosis is delayed can bring detrimental results. The aim of this study was to describe the clinical characteristics of intestinal volvulus during the neonatal period by comparing total midgut volvulus (TMV) and segmental volvulus (SV)., Methods: The medical records of 44 neonates who were operated on for intestinal volvulus from 1993 to 2019 were retrospectively reviewed. The patients were divided into TMV and SV groups, and clinical features were compared., Results: Operations were performed on 27 patients for TMV and 17 for SV. All cases of TMV were associated with intestinal malrotation, while those with SV were not. Gestational age, birth weight, and ratio of prematurity showed no differences between the groups. Preoperative diagnosis of TMV or SV was possible in 23 and 5 (85% and 29%) cases, respectively. Intestinal resection was required in 16/17 patients (94%) with SV, while it was required in 5/27 (19%) patients with TMV. When bowel resection was performed in TMV, all but one patient suffered from short bowel syndrome leading to two mortalities, while SV group showed good recovery., Conclusion: Diagnosis of SV before laparotomy can be difficult. Even though performing intestinal resection in SV neonates is highly likely, it shows a favorable outcome. TMV neonates are less likely to undergo intestinal resection; however, when bowel ischemia is present, significant morbidity can occur., Type of Study: Treatment study., Level of Evidence: Level IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Congenital myenteric hypoganglionosis is a rare developmental disorder characterized clinically by severe and persistent neonatal intestinal pseudoobstruction. The diagnosis is established by the prevalence of small myenteric ganglia composed of closely spaced ganglion cells with sparse surrounding neuropil. In practice, the diagnosis entails familiarity with the normal appearance of myenteric ganglia in young infants and the ability to confidently recognize significant deviations in ganglion size and morphology. We review clinical, histologic, and immunohistochemical findings from 12 patients with congenital myenteric hypoganglionosis in comparison with similar data from age-matched controls and clearly delineate the diagnostic features of the condition. Practical guidelines are provided to assist surgical pathologists, who are likely to encounter this condition only infrequently. The diagnosis typically requires full-thickness intestinal biopsy as the abnormality is confined to the myenteric plexus in many patients. Immunohistochemistry for Hu C/D may be used to confirm hypoganglionosis. Reduced staining for calretinin and NeuN implicates a selective deficiency of intrinsic primary afferent neurons in this disease., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Rationale: Gastrointestinal tract duplication is a rare congenial anomaly which can be found anywhere along the gastrointestinal tract. While many patients are incidentally diagnosed during operation, in some cases it can present with severe gastrointestinal symptoms. In this case report, the patient presented with signs of toxic megacolon leading to rapid aggravation of inflammatory shock., Patient Concerns: A 49-day old male infant presented with fever, poor feeding, and severe abdominal distension., Diagnosis: Abdominal ultrasonography was done. During the examination, a foley catheter was inserted through the anus to evaluate bowel patency and enable rectal decompression. The tip of the foley catheter was located in a separate narrower tubular lumen adjacent to the distended rectum. These findings suggested possibility of a tubular duplication cyst of the rectum as the culprit for the bowel obstruction., Interventions: The patient underwent emergency laparotomy. Findings showed multiple tubular intestinal duplications involving the ileum, appendix, cecum, descending colon, sigmoid colon and rectum. The true lumen of the rectosigmoid colon was completely collapsed while the adjacent tubular cyst remained severely distended and stool passage was not possible. Decompression of the sigmoid colon was done with loop colostomy with both the wall of the true bowel and enteric cyst forming the colostomy orifice., Outcomes: After 40 days of postoperative care, the patient was discharged with no immediate complications. Four months after the initial operation, colostomy take-down and transanal rectal common wall division was done. No complications were observed., Lessons: To our knowledge, this is the first case to be reported where a rare presentation of intestinal duplication resulted in an acute presentation toxic megacolon. Such emergency cases can be effectively treated with emergency surgical bowel decompression and elective common wall division., Competing Interests: The authors have no conflicts of interests to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Exstrophic rectal duplication and its association with bladder exstrophy and anorectal malformation is an extremely rare clinical entity. This is a report of the second case of an exstrophic rectal duplication associated with bladder exstrophy in English literature. However, it is the first case, where all these anomalies were accompanied by an anorectal malformation., Competing Interests: None

The review is devoted to the current classifications of common bile duct malformations, authors' opinion regarding surgical approach and reconstruction of the bile ducts. The authors describe the modern modifications of bile duct reconstructions and estimate their effectiveness. PubMed database, Google Scholar and National Scientific Electronic Library eLIBRARY.ru databases were used. We analyzed the complications in children depending on various reconstructions, anatomical form, timing of reconstructive surgery and early diagnosis of this pathology.

We present the case of a 25-year-old female who presented due to refractory chronic constipation and fecal incontinence. She had bowel movements every 7-30 days with an increased consistency (1-2 Bristol type stools), together with soiling and passive fecal incontinence (Wexner Scale: 12/20). She had previously undergone surgery shortly after birth for an anorectal malformation repair. The colonoscopy and histological study of the rectum were normal. A pelvic magnetic resonance imaging (MRI) was performed, which showed a right pararectal mass that compressed the rectum without invading it. This mass was compatible with a presacral teratoma or hamartoma. MRI also revealed coccyx agenesis and hypoplasia of the last sacral vertebrae (Image 1), which were consistent with Currarino syndrome (CS). The patient received 14 sessions of transcutaneous electrostimulation of the posterior tibial nerve, resulting in an increase in bowel movements (every 3 days) and a reduction in fecal incontinence. She was then referred to surgery for presacral mass removal.

Purpose: Midgut volvulus is associated with intestinal ischemia/reperfusion (IR) injury and can progress to severe intestinal damage. Remote ischemic conditioning (RIC) reduces IR-induced injury in distant organs. The aim of this study was to investigate whether RIC protects the intestine from IR injury., Methods: We investigated intestinal IR injury in 3 weeks old SD rats. Animals underwent: (i) sham laparotomy, (ii) intestinal IR injury, (iii) intestinal IR + RIC during ischemia, or (iv) intestinal IR + RIC after reperfusion. Intestinal IR injury was achieved by 45 min occlusion of superior mesenteric artery followed by de-occlusion. RIC was administered via four cycles of 5 min of hind limb ischemia followed by 5 min reperfusion. Animals were sacrificed 24 h after surgery and the ileum was harvested for evaluation., Results: Intestinal injury was present after IR. However, this injury was reduced in both IR + RIC groups. Expression of inflammatory cytokine IL6 was lower in IR + RIC groups compared to IR alone. Carbonyl protein was also significantly lower in IR + RIC compared to IR, indicating lower oxidative stress in both IR + RIC groups., Conclusion: Remote ischemic conditioning attenuated intestinal injury, inflammation, and oxidative stress in experimental intestinal ischemia/reperfusion injury. Remote ischemic conditioning may be useful in children with midgut volvulus to reduce the intestinal injury., Level of Evidence: Experimental study., Type of Study: Animal experiment.

Rationale: Duodenal atresia in association with situs inversus abdominus is extremely rare. Care should be taken when selecting appropriate surgical methods, and caution should be exercised during the surgery to avoid misdiagnosis and mistreatment. With prompt recognition of the condition, the surgical procedure should be performed in a timely manner to achieve positive results., Patient Concerns: A newborn affected by situs inversus abdominus associated with duodenal atresia, midgut malrotation, and volvulus., Diagnosis: Congenital duodenal atresia with situs inversus abdominis., Interventions: Diamond-shaped duodenoduodenostomy with appendectomy was performed, with the release of Ladd band and correction of the malrotation., Outcomes: The baby boy is thriving well with no abdominal complaints at 4 years of surgical follow-up., Lessons: Although several theories are put forward to clarify this matter, the proper cause of duodenal atresia is not well defined. Clinical symptoms and examinations can assist diagnosis, the definitive cause should be ascertained by surgical approach. And the operating surgeon must be aware of the "mirror anatomy" to prevent unnecessary injuries. Additionally, long-term prognosis for duodenal atresia are very good, therefore, careful attention in postoperative management are important in such a case.

&lt;b&gt;Introduction:&lt;/b&gt; Adult midgut malrotation is very rare. The presentation in adults is mostly subtle; the diagnosis is often made on imaging. &lt;br&gt;&lt;b&gt;Case report:&lt;/b&gt; A 32-year-old man presented with a 3-day history of an irreducible painful mass protruding per rectum, followed by abdominal pain and constipation. The patient was febrile and toxic, with marked signs of peritonitis and complete fullthickness irreducible rectal prolapse. Abdominal radiographs showed multiple air-fluid levels. A diagnosis of irreducible rectal prolapse with intestinal obstruction was made. Laparotomy revealed complete small bowel gangrene, abnormal rotation of the small bowel 180° around the mesenteric root, hypermobile duodenojejunal flexure and ileocaecal junction and an abnormally mobile caecum lying in the central abdomen. Abnormal mobility of the sigmoid colon with a large mesocolon, and a large irreducible rectosigmoid intussusception, was also noted. Our patient is probably the first case in literature, which may prompt awareness of simultaneously-occurring disorders of fixation, and preventive steps.

Right paraduodenal internal hernia, which has an embryonic origin, can be a rare cause of bowel obstruction, generally in young adults. We report the case of a young man who sought emergency care for acute abdominal pain with obstruction., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

This paper retrospectively analyzed 28 cases of children diagnosed with repeated intestinal malformations in our hospital from October 2014 to November 2016. The characteristics of ultrasound sonograms of children were evaluated. The images of intestinal repetitive malformations were classified and summarized to identify their common sites, the characteristics of various types of ultrasound sonograms, the main points of ultrasound diagnosis and identification, and the experience of ultrasound workers in daily inspection of children with repetitive malformations of the digestive tract. We analyzed the images of some common complications caused by repeated bowel malformations and identified the main points of ultrasound diagnosis. We then compared the results of ultrasound with other imaging findings, surgery, and pathologic results. At the same time, some special types of repeated bowel malformations were analyzed, discussed, and summarized to improve the understanding of ultrasound examination of gastrointestinal diseases in children, and show the superiority of children's abdominal intestine ultrasound. This study found that the ultrasound diagnosis of children with intestinal repetitive deformities has a high detection rate, clear images, clear anatomic levels, and clear blood flow conditions. Compared with other imaging methods, ultrasound has strong operability, real-time dynamics, strong specificity, and no radiation, and things are not easily misdiagnosed and missed. Therefore, ultrasound can be used as the preferred method for preoperative diagnosis, differential diagnosis, and postoperative follow-up review of repeated intestinal malformations in children., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Esophageal lung is a rare variety of communicating bronchopulmonary foregut malformation with anomaolous communication between an isolated portion of respiratory tissue and esophagus. Children present in early life with recurrent cough and pneumonia. Majority of the reported cases are associated with other anomalies like tracheoesophageal fistula. We report a case of a 7-month-old girl with right sided esophageal lung who was misdiagnosed as dextrocardia with right sided pneumonitis.

Introduction: Intestinal duplications are rare malformations and hail sites are the most common. Today, the diagnosis is made in antenatal because of the performances of antenatal ultrasound., Observation: This was a 15-month-old male infant received for constant crying, vomiting and increased abdominal volume that had been evolving for two weeks. An abdominal ultrasound was performed and objectified an intraperitoneal cyst formation of 30 x 27 mm, surrounded by a stratified wall and contiguous to a digestive loop. Surgery was performed and confirmed the existence of non-communicating ileal duplication., Conclusion: Intestinal duplication is a rare malformation. Ultrasound is often sufficient for diagnosis based on the presence of a characteristic double-walled cystic mass.