Segmental Dilatation of Ileum Involving Bronchogenic Cyst in a Newborn.

Objective: Congenital segmental intestinal dilatation (SID) and bronchogenic cyst in the abdomen are two uncommon and different pathologies. We report a bronchogenic cyst associated with segmental intestinal dilatation. Case: A 2-day-old 3300 g term infant developed bilious vomiting. A jejunoileal segment with a diameter of 10 cm was detected at surgery. Histologically, the wall musculature and enteric plexus of the segmentally enlarged small intestine stained normally for CD117 and negative for calretinin. A bronchogenic cyst of 3 cm in diameter was centered on the mesenteric border of the dilated intestine. Conclusion: SID has a normal staining pattern for CD117 (for interstitial cells of Cajal) and negative for calretinin. it would suggest that the innervation is defective, may be associated with a bronchogenic cyst in the newborn, causing obstruction, requiring surgery.

Rare paediatric case of agenesis of the vermiform appendix, ileal duplication and sickle cell disease.

This study reports an exceptional case of a 14-year-old girl with sickle cell disease that was diagnosed with agenesis of the vermiform appendix and ileal duplication. Both consist of extremely rare gastrointestinal malformations whose association has never been described. The preadolescent girl presented with abdominal pain and vomiting, and the ultrasound was suggestive of acute appendicitis. Surgical findings were agenesis of the vermiform appendix and a T-shaped ileal malformation with inflammatory changes. The patient underwent resection and ileal end-to-end anastomosis. Histopathological evaluation identified an ileal duplication, with small bowel and colonic mucosa, no communication to the adjacent ileum and ischaemic changes. At 8-month follow-up, the patient was asymptomatic.

[Ileal Atresia Due to Intrauterine Intussusception]. / Intrauterine Invagination als Ursache einer Dünndarmatresie.

Intussusception commonly affects children in the first year of life but it may rarely also appear in utero. We report a newborn with delayed passing of meconium, repeated vomiting, and abdominal distension in the first week of life. After radiological diagnosis of a small bowel obstruction, the newborn underwent an exploratory laparotomy where an ileal atresia proximal to an intussusception was found. After resection of the affected bowel, an end-to-end anastomosis was possible. The postoperative period was uneventful. This case shows that intrauterine intussusception can be a rare cause for ileal atresia. Fast diagnosis and effective interdisciplinary team work are essential for a satisfying outcome.

Obstruccion intestinal por Íleo biliar: reporte de un caso / Biliary Ileus intestinal obstruction. a case report

El ileo biliar es una rara complicación de la colelitiasis no tratada. Presentamos el cuadro clínico y el manejo de un paciente masculino de 72 años (AU)

The biliary or Gallstone ileus is a rare complication of the non-treated Cholelithiasis. We present the clinical features and the treatment of a 72 years old patient

Digestive Duplication: A rare cause of abdominal mass in an infant.

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.

Long-term surgical outcomes of apple-peel atresia.

PURPOSE: The purpose of this study was to investigate (i) postoperative course of apple-peel atresia (APA), (ii) long-term follow-up of APA children, and (iii) risk factors for poor prognosis. METHODS: We conducted a retrospective review of 39 APA neonates treated at our institution between 2008 and 2017. Patient characteristics, operative details, postoperative course, long-term outcomes, and prognostic factors were analyzed. RESULTS: Of the 39 APA neonates, 30 (76.9%) were born preterm, and 20 (51.3%) were diagnosed prenatally. All patients underwent primary anastomosis within the first week after birth: 10 laparoscopic-assisted (25.6%) and 29 open (74.4%). Postoperative complications occurred in 28 patients (71.8%), of which 20 (71.4%) developed cholestasis. Survival at hospital discharge was 94.9%. Median parenteral nutrition period was 59 days. Reoperation was required in 7 children (17.9%) owing to anastomotic obstruction (n = 3) and adhesive intestinal obstruction (n = 4). 32 children (82.1%) were followed up for an average of 5.7 years, of which 23 children (71.9%) showed normal growth and development. APA patients with low birth weight and associated anomalies had significantly worse outcomes. CONCLUSION: Most of the patients with apple-peel atresia have excellent long-term outcomes, though initial postoperative complications are common. Low birth weight and the presence of associated anomalies are independent prognostic factors in APA. TYPE OF STUDY: Prognosis study (case series). LEVEL OF EVIDENCE: Level IV.

Is Pouch Specific to Colon and Not Ileum?

BACKGROUND: Congenital Pouch Colon (CPC) is an anorectal anomaly with an incidence of 3.5:1 in males and females, respectively. We have earlier reported CPC to be quite prevalent in north Indian tertiary care centers. OBJECTIVE: In this article, we deliberate on the possible causes associated with CPC bringing the manifestation of the disease. In addition, we throw insights on the effective role of this congenital anomaly in Colon and provide systems genomic evaluation by comparing our recent analysis to that of Colon and Ileum based on Next-Generation Sequencing (NGS) studies. CONCLUSION: In this commentary article, we argue that a host of epigenetic factors could be the reason why the disease is manifested in colon alone. We further hypothesize on the few unmet challenges linking epigenetics to understand the genetic variants.

Outcomes of end-to-side oblique anastomosis as a surgical technique for jejuno-ileal atresia.

All of the anastomotic techniques used for jejuno-ileal atresia aim to overcome the size discrepancy between the resected bowel ends, lessen anastomotic complication and prevent development of short bowel syndrome. Aim of study was to evaluate the outcomes of end-to-side oblique anastomosis for jejuno-ileal atresia and verifying the risk factors for complications. A prospective study was conducted on patients with jejuno-ileal atresia managed by end-to-side oblique anastomotic technique over a 7-year period. Data were collected and analyzed to evaluate the outcomes of this surgical technique as morbidity and mortality. The study included 40 patients, 26 males and 14 females. The mean operation time was 105 minutes, the mean time for starting oral feeding was 5.7 days and the mean duration of hospital stay was 9.13 day. Postoperative complications occurred in 11 patients (27.5%). Death was recorded in six cases; survival rate was 85%. End-to-side oblique anastomosis is simple and effective surgical procedure for most types of jejuno-ileal atresia. The technique results in wide and early functioning anastomosis, relatively low morbidity and mortality. As with any neonatal surgery, the overall prognosis and outcomes is primarily dependent on prematurity, birth weight and associated anomalies in addition to the amount of residual bowel function that exist after surgery.

Complete evagination of a patent vitellointestinal duct and adjacent ileal limbs from an omphalocele sac: an extreme presentation.

Vitellointestinal duct (VID) anomalies have been described extensively in the literature. However, an everted VID with prolapse of ileum arising from an omphalocele is rare, and its appearance at birth can be alarming and can present a diagnostic challenge. We describe a baby born to a teenage diabetic mother who was noted to have a strange exophytic mass arising from the abdominal wall. Antenatal scans had revealed multiple other malformations but not an omphalocele. He was operated on early, and the diagnosis of a patent VID with prolapse of the ileum arising from an omphalocele was only confirmed intraoperatively. The duct was resected, the ileum closed primarily and primary closure of the abdominal wall was performed without tension. He recovered well postoperatively. A brief review of similar cases is included.

Umbilical Cord Ulcer and Intrauterine Death in Fetal Intestinal Atresia.

OBJECTIVE: To examine the incidence of umbilical cord ulcer (UCU) that causes intrauterine fetal death (IUFD) in fetal duodenal or jejunoileal atresia and the association between UCU and bile acid concentrations in amniotic fluid. METHODS: Perinatal outcomes were evaluated in cases of fetal intestinal atresia between 2003 and 2017. A pathological examination of the umbilical cord was performed, and bile acid concentrations in the amniotic fluid were measured. RESULTS: Among the 46 cases included in this study, there were 27 with duodenal atresia and 19 with jejunoileal atresia. There were 4 cases (8.7%) of IUFD and 1 (2.2%) neonatal death with multiple structural anomalies. UCUs were found in 37.5% (15/40) of cases, and severe UCUs with exposed vessels were significantly more common in IUFD (3/4) than in livebirth (0/42) cases (p < 0.01). The incidences of chromosomal abnormality and structural anomalies were not markedly different between livebirth (9/30 and 11/42, respectively) and IUFD (1/3 and 1/4, respectively) cases. Bile acid concentrations in amniotic fluid were significantly higher in cases of UCUs than in those without (p < 0.01). CONCLUSION: UCUs were not rare in fetal intestinal atresia and were associated with high bile acid concentrations in amniotic fluid. UCUs with exposed vessels were associated with IUFD in intestinal atresia.

Renal tubular dysgenesis and microcolon, a novel association. Report of three cases.

Renal tubular dysgenesis (RTD) is a developmental abnormality of the nephron characterized by fetal anuria, oligohydramnios, and severe postnatal hypotension. Genetic forms have an autosomal recessive inheritance and are caused by mutations in genes encoding key components of the renin-angiotensin pathway. We report three patients from two unrelated families with RTD due to pathogenic variants of the angiotensin-converting enzyme (ACE) gene, in whom RTD was associated with microcolon. We also detail key variations of the renin-angiotensin system in one of these infants. The severe intestinal developmental abnormality culminating in microcolon and early terminal ileum perforation/necrotizing enterocolitis is a novel finding not previously associated with RTD, which points to a role of the renin-angiotensin system in gut development.

[Ileal duplication: challenging differential diagnosis caused by a rare abnormality. Case report]. / Ileum duplex: egy ritka elváltozás okozta differenciáldiagnosztikai kihívás.

Gastrointestinal tract duplications (GSD) are rare congenital abnormalities. Eighty percent of GSDs are diagnosed before the age of two. These lesions can be seen anywhere from the oral cavity to the anus, but ileum is the most commonly affected site. Their clinical presentation is widely variable and unspecific, making the differential diagnosis really hard. Thus despite performing a long line of radiological scans, the diagnosis can be made during a surgery and by the pathologist. A 23-year-old female patient presented at the emergency room (ER) with abdominal cramps. Examinations revealed an unidentified intraabdominal mass. This could not been identified through the next years despite having tons of examinations: intravaginal and abdominal ultrasonographies, CT and MRI scans, colonoscopies, laparoscopies, surgical, gynecological and gastroenterological visits. Amongst the diagnoses were: ovarian cyst, bowel enlargement, Crohn's disease. Due to the latter, she received therapy which temporarily eased her symptoms. But after these, because of abdominal pain, fever and an ultrasonography that showed an intramural abscess in her abdomen, she went through a surgery having an ileocecal resection. Pathological examination showed a duplication of the ileum that might have caused her symptoms all through the years. Despite facing this rare abnormality, it is important to keep this in mind in differentiating abdominal symptoms. It is true that in no case there could be a diagnosis made without surgery, it raises attention to the importance of precise medical history taking and also cooperation between specialties. Orv Hetil. 2018; 159(52): 2217-2221.

Enteric duplication cyst as a cause for small bowel obstruction in adulthood.

We report a case of a patient presenting with small bowel obstruction secondary to an enteric ileal duplication cyst. Although common in infancy, they are rarely seen in adults. Radiologically they may be difficult to distinguish from a Meckel diverticulum and often the diagnosis is made retrospectively. Optimal management of the asymptomatic adult is unclear.

Total midgut duplication: a ticking time bomb.

A day-old neonate presented with bowel obstruction and an abdominal mass. Exploratory laparotomy revealed complete tubular midgut duplication from duodeno-jejunal junction up to terminal ileum which was communicating with the ileum distally. At the proximal end, another 5×5 cm duplication cyst was identified and excised. Postoperatively, complaints were relieved. During follow-up, 99m-Tc-pertechnetate-SPECT scan showed ectopic gastric mucosa in lower abdomen and in the right hemithorax. CECT-chest showed a 3×3 cm foregut duplication cyst, but there were no respiratory symptoms. While being planned for an elective surgery, he presented at 6 months of age in emergency with massive bleed per-rectum and shock. He underwent Wrenn procedure without any injury to the normal bowel. The thoracic foregut duplication cyst was excised later. Total midgut duplication, though benign, may present with life-threatening haemorrhage if left untreated. Presence of one such lesion warrants a search for others. Mucosal stripping is a simple and safe alternate to resection.

Presentation and outcome of treatment of jejunoileal atresia in Nigeria.

BACKGROUND: Intestinal atresia is a common cause of neonatal intestinal obstruction. Previous reports from Nigeria have indicated a high mortality rate. This is a report of current outcome review from one tertiary center. PATIENTS AND METHODS: A retrospective analysis of infants managed for jejunoileal atresia in 10 years (2005-2014). The information retrieved from patients' records was analyzed using SPSS 17. RESULTS: There were 38 patients (19 boys and 19 girls) aged 1-28 days (median 4 days). Twenty-four patients (63.2%) presented after 48 h of life. Twenty-five (65.8%) had jejunal atresia and 13 (34.2%) had ileal atresia. Six patients had associated anomalies. The most common atresia was type III (39.5%, 15 patients). Twenty-eight (73.7%) patients had a resection of the atresia and anastomosis and others had enterostomies. Total parenteral nutrition and neonatal intensive care support were not available during the period of the study. Bowel function was established within 1 week and 27 (71.1%) patients commenced oral feeding. Twenty-six (68.4%) patients had postoperative complications resulting in prolonged hospital stay of 2-44 days (median = 13). Mortality was 34.2% (13 patients). Factors that significantly affected mortality were intestinal necrosis at presentation, postoperative complications, and severe malnutrition. CONCLUSION: Intestinal atresia is still associated with unacceptably high morbidity and mortality, due to late presentation, and lack neonatal intensive care services and parenteral nutritional support. Efforts need to be intensified to address these factors to improve outcome.

A Case of Anomalous Congenital Band that Was Difficult to Differentiate from Omphalomesenteric Duct Anomaly.

Anomalous congenital band (ACB) is rare and difficult to identify preoperatively. Here we report a pediatric ACB case that was preoperatively suspected using computed tomography and was difficult to differentiate from omphalomesenteric duct anomaly. ACB should be considered in the differential diagnosis of acute abdomen.