[An adult case of a small bowel volvulus with intestinal malrotation that resulted in postoperative short bowel syndrome after massive small bowel resection].

A 29-year-old man presented to the hospital with complaints of abdominal pain and vomiting. Abdominal computed tomography revealed intestinal malrotation, a whirl sign of the small intestine, and occlusion of the superior mesenteric artery and vein. Due to dilation of the small intestine and poor contrast enhancement of the intestinal wall, he was diagnosed with strangulating small bowel obstruction with intestinal necrosis. Emergency surgery was performed owing to the diagnosis of small bowel volvulus with intestinal malrotation, which necessitated massive small bowel resection. The remnant small intestine was approximately 100cm in length. The patient developed short bowel syndrome postoperatively but eventually resumed a normal diet.

Tubulocystic enteric duplication: a unique entity.

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.

Performance of pan-enteroscopy in children with intestinal failure due to short bowel syndrome: A single-center retrospective study.

OBJECTIVES: In pediatric patients with intestinal failure (IF) due to short bowel syndrome (SBS), we hypothesized that young children, those with shorter residual small bowel and those with congenital malrotation of the bowel would be more likely to undergo pan-enteroscopy. We aimed to determine the feasibility and diagnostic yield of pan-enteroscopy in this cohort. METHODS: We performed a single-center, retrospective study of pediatric patients with IF due to SBS who had undergone at least one GI endoscopic evaluation between January 1, 2018 and January 1, 2023. RESULTS: A pan-enteroscopy might have been possible in 381 of the 431 procedures (206 patients) reviewed. Forty-four (21%) patients underwent 54 pan-enteroscopies. Children with a residual bowel length <35 cm had higher odds of undergoing pan-enteroscopy (odds ratio [OR] 3.72, 95% confidence interval [CI] [1.32, 10.48], p = 0.01), as did patients with periprocedural glucagon-like peptide 2 (GLP-2) analog use (OR 4.30, 95% CI [1.24, 14.95], p = 0.02). Patients with diagnoses other than necrotizing enterocolitis (NEC) tended to be more likely to achieve a pan-enteroscopy (OR 2.73, 95% CI [0.95,7.88], p = 0.06). Evidence of gross and histopathologic abnormalities were found in 77.8% and 78% of the procedures, respectively. No complications were identified. CONCLUSION: In a large cohort of children with SBS, pan-enteroscopy was successfully performed in 14.2% of the procedures and microscopic abnormalities were common. Shorter residual bowel length, underlying diagnoses of non-NEC, and GLP-2 analog use were generally associated with successful pan-enteroscopy, independent of age and several other factors. These data suggest that pan-enteroscopy is feasible and of high-yield in a subset of patients with SBS.

Adult Female Patient with Untreated Truncus Arteriosus Type I and Torsion of Small Bowel Gastrointestinal Stromal Tumor: A Rare Case Report.

BACKGROUND Persistent truncus arteriosus is a rare congenital cyanotic heart defect characterized by a single ventricular outflow tract. Without surgical intervention, it has a poor prognosis in infancy. Here, we report an adult female patient with uncorrected truncus arteriosus type I, who presented with acute-onset abdominal pain due to torsion of a small bowel gastrointestinal stromal tumor (GIST). CASE REPORT A 41-year-old woman came to our Emergency Department with acute-onset lower abdominal pain for 2 days. Congenital heart disease, truncus arteriosus, had been diagnosed at birth, and there had been no surgical intervention. Abdominal computed tomography revealed a 10×9×12-cm mixed-density mass in the pelvic capacity. Transthoracic echocardiography revealed a 33-mm ventricular septal defect. The ascending aorta originated mainly from the right ventricle, and the pulmonary artery originated from the beginning of the aorta (type I truncus arteriosus, according to Collett and Edwards classification). After a quick and detailed preoperative workup, the patient underwent tumor resection by open surgery with general anesthesia. CONCLUSIONS This is the first case to report emergency surgery for a patient with uncorrected persistent truncus arteriosus due to torsion of a small bowel GIST. A multidisciplinary team with deep understanding of the disease entity was crucial. By considering the fixed hemodynamic and respiratory physiology, overtreatment and unrealistic goals were avoided. Eventually, the patient was discharged after being hospitalized for 2 weeks.

Analysis of Ileal Atresia from Prenatal Ultrasound to Postoperative Follow-up: Two Case Reports.

BACKGROUND: Congenital ileal atresia is a rare neonatal disease, the most common type of intestinal malformation in newborns, and one of the most common causes of congenital intestinal obstruction. It can cause various digestive system symptoms, including abdominal distension, vomiting, abnormal bowel movements, etc. In severe cases, it can be life-threatening. A prenatal ultrasound examination can assist clinical diagnosis of congenital ileal atresia, and those with a clear prenatal diagnosis should undergo surgical treatment after birth. CASE PRESENTATION: We have, herein, reported two cases of congenital ileal atresia, both of which showed fetal intestinal dilation (>7mm) and excessive amniotic fluid on prenatal ultrasound. Both newborns underwent surgical treatment after delivery and were confirmed to have congenital ileal atresia during surgery. Due to the different prenatal ultrasound manifestations of the two patients, they were divided into two different subtypes based on intraoperative manifestations. We observed significant differences in the prognosis of the two patients after surgery. CONCLUSION: Accurately locating and classifying ileal atresia using prenatal ultrasound is challenging; however, it plays an effective role in disease progression, gestational assessment, and prognosis. Accurately identifying intestinal diseases and/or the location of lesion sites through direct and indirect ultrasound findings in the fetal abdominal cavity is an important research direction for prenatal ultrasound.

Jejunal Diverticulosis Causing Small Intestinal Volvulus and Closed Loop Obstruction.

BACKGROUND Jejunal diverticulosis are false diverticula of the small bowel that form from outpouching of the mucosa and submucosa. They are pulsion diverticula that are often asymptomatic and can be found incidentally during surgery. In some instances, jejunal diverticula could result in intestinal obstruction. Small intestinal volvulus is an uncommon cause of small bowel obstruction that results in a closed loop obstruction and is an indication for emergent surgical intervention. CASE REPORT We report a case of an 84-year-old man who presented to the Emergency Department with abdominal pain and generalized weakness. A preoperative computerized tomographic scan demonstrated a closed loop small bowel obstruction with mesenteric swirling. The patient was taken for a diagnostic laparoscopy, which revealed extensive proximal jejunal diverticulosis and a volvulus of the involved jejunum. An exploratory laparotomy was warranted for safe detorsion of the small bowel and resection of the diseased segment. The small bowel was successfully detorsed, with resection of the involved jejunum. Intestinal continuity was established by a primary side-to-side anastomosis. CONCLUSIONS Jejunal diverticula have been reported in the literature as a cause of small bowel obstructions, and very few reports exist of concurrent small bowel volvulus. In very rare instances, both of these conditions can coexist. There should be prompt surgical intervention in all cases of closed loop small bowel obstructions to prevent intestinal ischemia, perforation, and sepsis.

Importance of Prenatal Diagnosis of Ileal Atresia in Gestational Diabetes Cases.

BACKGROUND Maldevelopment of the fetal bowel can result in the rare condition of intestinal atresia, which results in congenital bowel obstruction. This report describes a case of prenatal diagnosis of fetal ileal atresia at 22 weeks' gestation. CASE REPORT Here, we present a 24-year old woman who was 22 weeks into her first pregnancy when she underwent routine fetal ultrasound. She was diagnosed with gestational diabetes mellitus. Her body mass index was normal and she had normal weight gain. The ultrasonographic examination performed revealed a hyperechoic bowel and a small dilatation of the bowel. The couple was counselled for possible intestinal atresia and its postnatal implications. At 33 weeks of gestation, polyhydramnios appeared, and the intestinal distension was much more pronounced, with hyperechoic debris in the intestinal lumen (succus-entericus). After birth, surgery was performed and we concluded the patient had type II atresia, which was surgically treated. CONCLUSIONS This report has highlighted the importance of antenatal ultrasound in detecting fetal abnormalities, and has shown that rare conditions such as intestinal atresia can be accurately diagnosed and successfully managed. Surgical correction, if implemented promptly after stabilizing the general condition, can have a relatively good prognosis. Coexisting fetal ileal atresia and gestational diabetes mellitus are rare occurrences, which can make each condition even more difficult to treat.

Efforts to improve outcomes among neonates with complex intestinal atresia: a single-center low-income country experience.

PURPOSE: Intestinal obstruction caused by intestinal atresia is a surgical emergency in newborns. Outcomes for the jejunal ileal atresia (JIA), the most common subtype of atresia in low-income countries (LIC), are poor. We sought to assess the impact of utilizing the Bishop-Koop (BK) approach to JIA in improving outcomes. METHODS: A retrospective cohort study was performed on children with complex JIA (Type 2-4) treated at our national referral hospital from 1/2018 to 12/2022. BK was regularly used starting 1/1/2021, and outcomes between 1/2021 and 12/2022 were compared to those between 1/2018 and 12/2020. Statistical significance was set at p < 0.05. RESULTS: A total of 122 neonates presented with JIA in 1/2018-12/2022, 83 of whom were treated for complex JIA. A significant decrease (p = 0.03) was noted in patient mortality in 2021 and 2022 (n = 33, 45.5% mortality) compared to 2018-2020 (n = 35, 71.4% mortality). This translated to a risk reduction of 0.64 (95% CI 0.41-0.98) with the increased use of BK. CONCLUSION: Increased use of BK anastomoses with early enteral nutrition and decreased use of primary anastomosis improves outcomes for neonates with severe JIA in LIC settings. Implementing this surgical approach in LICs may help address the disparities in outcomes for children with JIA.

Diagnostic value of the microcolon using ultrasonography in small bowel atresia.

BACKGROUND: Microcolon helps diagnose small bowel atresia (SBA) using contrast enema. However, there are no ultrasonography (US) microcolon criteria for diagnosing SBA. Therefore, this study aimed to evaluate colon accuracy and other characteristics for diagnosing SBA by US, using surgical or clinical information as the reference standard. METHODS: US was performed on 46 neonates aged ≤ 7 days old. In the study group (n = 15), neonates with SBA were confirmed following surgery. In the study group without SBA (n = 15), neonates with other gastrointestinal problems besides SBA were confirmed by surgical or clinical follow-up. Sixteen neonates without gastrointestinal problems were classified as the control group. The colonic diameter was measured, and colonic gas was sought and observed. Statistical analysis was performed to compare US parameters between the study group and other two groups. The optimal cut-off value of the colonic diameter for SBA diagnosis was obtained using receiver operating characteristic analysis. RESULTS: Colonic diameters (0.5 cm) in the study group (interquartile ranges [IQR], 0.5-0.6 cm) was significantly smaller than that in the group without SBA (0.9 cm; IQR, 0.8-1.2 cm) (P < 0.001) and in the control group (1.2 cm; IQR, 0.8-1.35 cm) (P < 0.001). Optimum cut-off value for diagnosing SBA was 0.65 cm (sensitivity, 90.3%; specificity, 86.7%; accuracy, 89.1%) for the colonic diameter. Combining microcolon and gas-negativity showed the best performance in SBA diagnosis using US, with increased accuracy (91.3%). CONCLUSION: A colon < 0.65 cm in diameter should be called a microcolon; combining US with gas-negativity is an essential diagnostic basis for SBA.

Diagnostic laparoscopy to exclude malrotation following inconclusive upper gastrointestinal contrast study in infants.

PURPOSE: We report the use of diagnostic laparoscopy as an alternative to laparotomy in the investigation of infants with clinical features concerning for malrotation and inconclusive upper gastrointestinal contrast study. METHOD: Case note review of all infants in whom laparoscopy was performed during 2016-2020 to investigate for possible malrotation. RESULTS: Eight infants were identified. All presented with acute clinical features of malrotation (bilious vomit) without evidence of an alternate explanatory diagnosis. All underwent upper gastrointestinal contrast study, with three also undergoing abdominal ultrasound. The radiological examinations could not exclude malrotation and all proceeded to laparoscopy. At laparoscopy, the small intestine was run to exclude the presence of midgut volvulus. In six cases, normal rotation was confirmed and no abnormal pathology was found. Two proceeded to laparotomy and underwent correction of malrotation. All infants recovered without complication. CONCLUSION: Laparoscopy is an excellent modality for further investigation of infants presenting acutely in whom intestinal malrotation cannot be formally excluded radiologically. The positive identification of the DJ flexure and cecum in correct anatomical sites, both fixed to the posterior abdominal wall, provides adequate reassurance of low risk of volvulus and avoids a full laparotomy. We recommend diagnostic laparoscopy in cases of inconclusive upper gastrointestinal contrast study.

Multiple Small Bowel Diverticula Were an Unexpected Finding During Laparoscopic Enterectomy for Crohn's Disease.

INTRODUCTION: Small bowel diverticulosis (SBD) is a rare entity. Although it is usually an asymptomatic condition, clinical manifestations may vary from non-specific clinical signs to severe and complicated disease. The coexistence of SBD and Crohn's disease (CD) is rarely reported in the current literature. AIM: We present a rare case of concomitant Crohn's disease (CD) and SBD in a male patient, where multiple jejunal diverticula were an incidental intraoperative finding. Preoperative evaluation with magnetic resonance enterography (MRE) failed to recognize the coexistence of these two entities. Surgeons should be aware of the possibility of this rare situation. CASE REPORT: A 52-year-old Caucasian male diagnosed with CD was referred to our department for surgical intervention due to an ileal stricture. The patient reported no past medical history, except for a few episodes of bloody diarrhoea during a three-year period. The index colonoscopy revealed luminal narrowing in the ileum at approximately 70 cm proximal to the ileocaecal valve, and biopsies revealed findings compatible with CD. Clinical examination and laboratory tests were unremarkable one day before surgery. The patient underwent laparoscopic segmental resection of the affected part of the ileum. Intraoperatively, multiple non-inflamed diverticula along the jejunum extending from the Treitz ligament to the proximal ileum were recognized. Our patient had an uncomplicated post-operative course and was discharged on the fifth post-operative day. Pathological examination revealed features compatible with CD in the active phase. The patient was referred to his gastroenterological team for further consultation regarding the appropriate post-operative management. CONCLUSION: Concomitant CD and SBD is a rare condition, and the differential diagnosis may be challenging due to overlapping symptoms.

Diagnostic performance and role of the contrast enema for low intestinal obstruction in neonates.

PURPOSE: We aim to evaluate the diagnostic performance and relationship between clinical characteristics, imaging findings, and final diagnosis for the neonatal contrast enema (CE). METHODS: Retrospective 10-year review of all neonatal CEs including imaging findings, clinical information, indication, and final diagnosis from discharge summaries, surgical reports, and pathology (reference standard). Two blinded pediatric radiologists reinterpreted 366 CEs for obstruction, microcolon, rectosigmoid index (RSI), serrations, meconium, ileal cut-off, transition zone, diagnosis, and level of confidence. CE diagnostic performance was calculated versus reference standard. RESULTS: Diagnoses included Hirschsprung disease (HD) (15.8%), small left colon syndrome (14.8%), small intestinal atresia/colonic atresia (SIA/CA) (12.6%), meconium ileus (MI) (4.4%), and normal (48.9%). CE had a moderate specificity (87.7%) and low sensitivity (65.5%) for HD; abnormal RSI and serrations showed high specificities (90.3%, 97.4%) but low sensitivities (46.6%, 17.2%). CE showed high specificity (97.4%) and low sensitivity (56.3%) for MI blinded to cystic fibrosis status. Microcolon was specific (96.6%) but not sensitive (68.8%) for MI. CE showed highest PPV (73.1%) (specificity 95.6%, sensitivity 82.6%) for SIA/CA. Microcolon with an abrupt cut-off was specific (99.1%) but not sensitive (41.3%) for atresias. CONCLUSION: Neonatal CE demonstrates high specificities and low to moderate sensitivities across all diagnoses, with lowest performance in HD. CLINICAL TRIAL REGISTRATION: None.

A rare case of intrauterine intussusception causing ileal atresia.

Intrauterine intussusception is a rare but evident cause of intestinal atresia and is usually detected intraoperatively. We report on a term neonate who presented to the Department of Paediatric Surgery, Sabah Women and Children's Hospital, Malaysia with delayed passage of meconium and intestinal obstruction, wherein the lower contrast showed a claw sign. This was a good clue that this neonate had intrauterine intussusception and this suspicion was confirmed upon laparotomy. We found an ileo-ileal intussusception causing ileal atresia, requiring resection and primary anastomosis.

[Therapeutic effect of different surgical methods for congenital small intestine atresia].

Objective: To compare and analyze the curative effect of three surgical methods in the treatment of small intestine atresia, and to provide evidence for individualized surgical treatment of children with small intestine atresia. Methods: The clinical diagnosis and treatment of 168 children with small intestine Ⅱ, Ⅲ, Ⅳ type atresia in our hospital from January 2008 to September 2017 were retrospectively analyzed and they were divided into different types according to the operation. The three groups were end-to-end anastomosis group (EEA, n=58), end oblique anastomosis group (EOA, n=68), and proximal end-end anastomosis group (PEA, n=42). The EEA group and the EOA group were further divided into group a (EEA-a/EOA-a) with a proximal intestinal tube diameter greater than 4.0 times the distal end and a group b ((EEA-b/EOA-b) with a diameter less than or equal to 4.0 times the distal intestinal tube diameter. The gender, gestational age, birth weight, type of atresia, and postoperative defecation time, postoperative feeding time, postoperative hospital stay and postoperative follow-up complications were compared. Results: There was no significant difference in gender, gestational age and birth weight between the groups (P>0.05). The PEA group was better than EEA-a group and EOA-a group in postoperative defecation time, postoperative feeding time, postoperative hospital stay and complications (P<0.05). The postoperative defecation time, postoperative feeding time, postoperative hospital stay and complications of the EOA-a group were better than those of the EEA-a group (P<0.05). There was no statistically significant difference in postoperative defecation time, postoperative feeding time, and complications between the EEA-b group and the EOA-b group (P>0.05), but the postoperative hospital stay in the EEA-b group was longer than that in the EOA-b group (P<0.05). Conclusion: PEA is recommended for children with a proximal intestinal canal diameter greater than 4.0 times greater than the distal end because of the rapid recovery and fewer complications; EOA is recommended for children with a proximal intestinal canal diameter of 4.0 or less because of its advantage of shorter hospital stay than EEA surgery. Congenital intestinal atresia has a better effect according to the specific conditions of the child.

Study on the Application of Ultrasonic Image Analysis Technology Combined with the Analysis of Intestinal Nervous System in the Treatment of Intestinal Duplication in Children.

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.

Malrotation in Congenital Diaphragmatic Hernia: Is It Really a Problem?

INTRODUCTION: Congenital diaphragmatic hernia (CDH) has been associated with other congenital malformations, such as intestinal rotation abnormalities. However, there is no standard evaluation and treatment of intestinal rotation during initial CDH repair. This study evaluates intestinal malrotation in CDH patients. MATERIALS AND METHODS: All patients with a CDH treated in a high-volume center between 2000 and 2015 were retrospectively evaluated. Demographics, gastrointestinal characteristics, surgical treatment, and abdominal outcomes (acute surgery, small bowel obstruction, and volvulus) were described. RESULTS: A total of 197 CDH patients were surgically repaired. In 76 (39%) patients, a malrotation was described at initial CDH repair, in 39 (20%) patients, a normal rotation, but 82 patients (42%) had no report on intestinal rotation. During follow-up (range: 2-16 years), 12 additional malrotations were diagnosed, leading to a prevalence of at least 45% (n = 88). These were mostly diagnosed due to acute small bowel obstruction, of which three had a volvulus. Of the 12 later diagnosed malrotations, 58% required acute surgery, compared to 3% of the 76 initially diagnosed patients (p < 0.001). CONCLUSION: Malrotation is associated with CDH, with a prevalence of at least 45% and in 21% a normal intestinal rotation. "Missed" malrotations have a higher risk on need for acute surgery later in life.

Jejuno-ileal atresia: its characteristics and peculiarities concerning apple peel atresia, focused on its treatment and outcomes as experienced in one of the leading South African academic centres.

PURPOSE: Jejuno-ileal atresia remains the most common form of intestinal obstruction in the neonatal and infantile age group and has an incidence of 1:300 to 1:1500. Apple peel atresia (APA) is the rarest of the five described types. The morbidity and mortality of patients with APA managed at our institution are high, and we review our experience with this paper. We compared our outcomes with other developed and developing countries. In addition, we looked at factors that affect outcome and how we can change them. METHODS: The study was a retrospective review of all patients treated with APA at IALCH between January 2002 and December 2010 and includes 34 patients. RESULTS: The results revealed a mortality in excess of 70%. There were poor antenatal screening, a high rate of prematurity and often delays in transfer to our institution. Relaparotomy and sepsis rates were high. CONCLUSION: This review represents a significant number of patients with APA from a single institution. Although survival rates have improved significantly over the years, APA remains a life-threatening malformation and results in significant morbidity and mortality in our setting.

Is Tapering Enteroplasty an Alternative to Resection of Dilated Bowel in Small Intestinal Atresia?

BACKGROUND: Intestinal atresia is a congenital defect resulting in intestinal discontinuity and can be associated with significant morbidity related to intestinal failure. The bowel proximal to the atresia is often significantly dilated and dysfunctional. The treatment approaches of this dilated bowel include resection with primary anastomosis versus tapering enteroplasty with preservation of bowel length. The purpose of this study was to compare these two approaches in regard to bowel function as characterized by the time to full enteral feeding. METHODS: A retrospective review was performed of intestinal atresia repair performed at a tertiary referral pediatric hospital from 2007 to 2017. Length of stay, time to full enteral feeds, and complications were assessed in patients who underwent repair with tapering enteroplasty (n = 8) and compared with those who underwent resection and anastomosis (n = 39). RESULTS: The median age at surgery, gender distribution, weeks gestational age (WGA), location of the atresia, and comorbidities were similar between the two groups. Overall, there was no statistically significant difference in length of stay and time to full enteral feeds between groups. Three of eight (38%) patients in the tapered group and five of 39 patients (13%; P = 0.12) in the nontapered group underwent further surgical exploration because of bowel dysmotility. Factors associated with longer length of hospital stay were abdominal reoperation and WGA, and factors associated with longer time to full enteral feeds were WGA, abdominal reoperation, and gastroschisis. CONCLUSIONS: Tapering enteroplasty at initial operation for intestinal atresias preserves bowel length and has statistically equivalent outcomes to resection and anastomosis in regard to the length of stay and time to full enteral feeds.