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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.)

Competing Interests: The authors report no conflicts of interest.

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.)

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.)

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.

Gastrointestinal duplication cysts are rare congenital malformations, with esophageal and gastric duplication cysts being among the rarest. We report an 8-week-old female who presented to the emergency department with failure to thrive and was subsequently found to have multiple gastric and esophageal duplication cysts that had ruptured intrathoracically and intra-abdominally. We describe the diagnosis and management of this patient who underwent successful resection of 4 gastrointestinal duplication cysts. This report emphasizes the unexpected, and sometimes relatively benign, presentations of gastrointestinal duplication cysts. To our knowledge, this is the first reported occurrence of multiple duplication cysts that independently ruptured thoracically and abdominally.

Two children developed hepatoblastoma concurrent with congenital portosystemic shunts (PSSs) (Abernethy malformations). Both underwent operative ligation of their PSSs. One received concurrent tumor resection, whereas the other was deemed initially unresectable and underwent biopsy followed by neoadjuvant chemotherapy. Although benign hepatic masses, such as focal nodular hyperplasia and nodular regenerative hyperplasia, are common in patients with Abernethy malformations, malignant tumors have also been documented and should always be considered in the differential diagnosis of a patient with a congenital PSS and a hepatic mass.

Objective: Management of short bowel syndrome (SBS) has significantly evolved recently. We present our single-center, 25-year experience focusing on the implementation of a refined multidisciplinary SBS care protocol., Methods: This is a retrospective review of the patients with SBS treated at our tertiary center from 1988 to 2014, with either <25% short bowel remaining or duration of parenteral nutrition (PN) >3 months. Patients with primary intestinal motility disorders were excluded. Clinical characteristics, including intestinal anatomy, markers of cholestasis, and catheter-related infections (CRIs), were analyzed. The implementation of a refined modern uniform management protocol in 2009 divided the cohort into 2 subgroups, whose outcomes are compared., Results: Forty-eight patients with SBS were identified (median gestational age 33 weeks). Of them, 22 were born between 2009 and 2014. The main causes of SBS were necrotizing enterocolitis (46%) and midgut volvulus (23%). Median remaining small bowel length was 36 cm. The overall survival was 23 of 26 (88%) before 2009 and 21 of 22 (95%) thereafter, whereas none were transplanted. Duration of PN shortened from a median of 15 to 6 months (P = 0.0015) in the latter cohort, whereas frequency of autologous intestinal reconstruction surgery (31%) remained unchanged. Frequency of neonatal cholestasis was similar in both groups (75%), but cleared in all after 2009. Before 2009, 2 patients died of progressive cholestatic liver failure. The CRI rates decreased from 1.7 to 0.7 per 1000 catheter-days between 2000-2008 and 2009-2014, respectively (P = 0.0178)., Conclusions: Uniform refined multidisciplinary approach decreased the duration of PN and CRI rates with high transplant-free survival and avoidance of liver failure, although the frequency of transient neonatal cholestasis remained unchanged.

The complete Currarino triad (or syndrome) consists of anorectal anomalies, a sacral bony defect, and a presacral mass. Generally autosomal dominant in its inheritance, incomplete penetrance also characterizes this syndrome. We report the case of a 12-day-old patient with vomiting in whom a diagnosis of incomplete Currarino syndrome was made.

Objectives: We evaluated the frequencies and clinical consequences of mutations in the genes encoding cationic trypsinogen, serine protease 1 (PRSS1), and serine protease inhibitor Kazal type 1 (SPINK1) in children with acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP)., Patients and Methods: The study population consisted of 32 children with ARP or CP and 28 healthy controls. We analyzed clinical data and the sequences of the entire coding region and the intron-exon boundaries of the PRSS1 and SPINK1 genes from each patient., Results: Fifteen (46.9%) of the 32 patients had at least 1 PRSS1 or SPINK1 mutation. Four (12.5%) of the 32 patients carried the p.N29I, p.R122H, or p.N29T mutation or a p.G208A variant of the PRSS1 gene in a heterozygote state. Eleven (34.4%) of the 32 patients carried either the IVS3 + 2T>C or p.N34S mutation of the SPINK1 gene. No PRSS1 or SPINK1 mutations were identified in the control group. In particular, mutations were identified in 4 of our patients who experienced pancreas divisum with CP, whereas the remaining 2 patients with pancreas divisum and ARP did not have mutation., Conclusions: The frequencies of the PRSS1 and SPINK1 mutations are relatively high in Korean children with ARP or CP. Mutations in the PRSS1 and SPINK1 genes are highly associated with the development of childhood ARP or CP. Our findings suggest that patients with genetic mutations combined with pancreas divisum tend to develop CP early.

Objectives: The aims of this review were to summarize a) the consensus definitions of normal and pathologic intra-abdominal pressure (IAP); b) the techniques to measure IAP; c) the risk factors for intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS); d) the pathophysiology of ACS; and e) the current recommendations for management and prevention of ACS., Data Sources: PubMed was searched using the following terms: ACS, IAH, IAP, and abdominal decompression., Data Synthesis: ACS represents the natural progression of end-organ dysfunction caused by increased IAP and develops if IAH is not recognized and treated appropriately. Although the reported incidence of ACS is relatively low in critically ill children (0.6%-4.7%) it may be under-recognized and under-reported. The diagnosis of IAH/ACS depends on a high index of suspicion and the accurate and frequent measurement of IAP in patients at risk. Mortality from ACS remains high (50%-60%) even when decompression of the abdomen is performed early, which highlights the importance of detection and treatment of elevated IAP before end-organ damage occurs., Conclusions: A widespread awareness of the recognition and current approach to management and prevention of IAH and ACS is needed among pediatric intensivists, so outcome of these life-threatening disease processes might be improved.

Background: An infant born with pyloric atresia had development of intractable diarrhea and was found to have total epithelial detachment of gastric and small and large bowel mucosa. She had no skin abnormalities. Parental consanguinity and pyloric atresia in a sibling who died without autopsy suggest an inherited origin for this disorder. The purpose of this study was to examine defects in intestinal and skin cell adhesion., Methods: Histologic, immunohistochemical, and ultrastructural characteristics of the skin and gut of the patient were compared with that of normal control subjects. Distribution of adhesion molecules was determined., Results: Immunofluorescent analysis of the digestive mucosa showed alpha6beta4 integrin expression deficiency at the epithelial cell-lamina propria junction. Ultrastructural examination of the digestive mucosa revealed a complete epithelial detachment with a cleavage plane lying between the lamina densa and the basal pole of the enterocytes. Consistent with the absence of skin blistering, integrin alpha6beta4 was expressed at the dermal-epidermal junction. Electron micrographs of skin biopsy specimens showed the presence of normal hemidesmosomes and the absence of dermal-epidermal dysadhesion., Conclusion: It was postulated that this patient had protracted diarrhea related to epithelial detachment of the digestive mucosa as a consequence of a deficiency of an integrin alpha6beta4 isoform specific to the gut.

Background: This study defines the multiple characteristics associated with complex pediatric feeding problems and determines the relative frequency of each classification in a population referred to an interdisciplinary feeding team., Methods: The written reports from team evaluations on 103 children (64 males, 39 females; age range 4 months to 17 years) were reviewed. Prematurity and/or presence of developmental delay was coded. Identified factors related to current feeding problems were coded according to five categories: structural abnormalities, neurological conditions, behavioral issues, cardiorespiratory problems, metabolic dysfunction., Results: Interrater reliability for the classification coding was 88%. Thirty-eight percent of the children had a history of pre- maturity and 74% were reported to have evidence of developmental delay. The following five categories or combinations were coded most frequently: structural-neurological-behavioral (30%), neurological-behavioral (27%), behavioral (12%), structural-behavioral (9%), and structural-neurological (8%). Overall, behavioral issues were coded more often (85%) than neurological conditions (73%), structural abnormalities (57%), cardiorespiratory problems (7%), or metabolic dysfunction (5%)., Conclusions: Data analysis using this classification system revealed that the majority of children in this sample had a behavioral component to their complex feeding problem, regardless of concurrent physical factors. These findings suggest that complex pediatric feeding problems are biobehavioral conditions in which biological and behavioral aspects mutually interact, and both need to be addressed to achieve normal feeding.