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.

[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.

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.

[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.

An unexpected gangrenous duplication of ileum.

Alimentary tract duplication is a rare congenital anomaly which may involve any part of the alimentary tract extending from stomach to rectum. Clinical presentation may mimic an inflamed appendix as described in this case. A 9-year-old boy with a clinical diagnosis of perforated appendix was noted to have a normal appendix intra-operatively. On further search for an underlying pathology, a gangrenous ileal duplication was discovered. En-bloc resection with primary bowel anastomosis was done. Histopathology report revealed a gangrenous small bowel duplication. We discuss the preoperative diagnostic dilemma and management options in approaching this rare entity.

Duodenal atresia with apple-peel configuration of the ileum and absent superior mesenteric artery.

BACKGROUND: Embryologically, duodenal atresia results from inadequate recanalisation and proliferation of gut epithelius in the 6th week of gestation, while apple-pee atresia of small bowel is a consequence of a vascular accident in subsequent embryonic development, and the two are rather rarely manifested as a joint clinical entity. CASE PRESENTATION: We present here a 29 week preterm boy admitted to the intensive care unit due to breathing difficulties and low birthweight. Following clinical, radiographic and ultrasound examination, he was diagnosed with duodenal obstruction and subjected to surgical treatment. The exploration of abdominal cavity verified duodenal atresia in the second portion with the absence of third and fourth portions of duodenum, superior mesenteric artery, as well as apple-peel atresia of jejunum. Resection of the apple-peel segment of jejunum was done and the continuity of digestive tube was established by the end-to-end duodeno-ileal anastomosis. CONSLUSION: This rare case of ours questions the embryology of duodenal atresia suggesting that a mesenteric vascular disruption phenomenon in subsequent embryonic life might be the aetiological factor.

The use of renal replacement therapy in critically ill pediatric small bowel transplantation candidates and recipients: Experience from one center.

Outcomes for pediatric SBT patients requiring perioperative RRT in the PICU remain unknown. The objectives were to document our center's experience with PICU SBT patients receiving perioperative RRT and to identify variables predictive of survival to discharge. A retrospective chart review of patients (ages, 0-18 yr) between January 1, 2000 and December 31, 2011 that received RRT within a SBT perioperative period and were transplanted at our university-affiliated, tertiary care children's hospital was performed. Six SBT patients received perioperative RRT (ages, 5-12 yr). Three patients (50%) survived to hospital discharge. Among survivors, RRT was required for a total of 1-112 days (mean, 49.7 days). All three survivors survived to hospital discharge without renal transplantation and free of RRT. There was a trend toward increased survival among older patients receiving RRT (p = 0.05). Survivors had a higher I-125 GFR prior to PICU admission (p = 0.045). A higher I-125 GFR prior to PICU admission among survivors may support this test's utility during SBT evaluation. In our experience, a high survival rate and freedom from RRT at the time of discharge support RRT use in the SBT population.

Diagnostic Accuracy of Prenatal Ultrasound in Identifying Jejunal and Ileal Atresia.

INTRODUCTION: Small bowel atresia (SBA) is one of the most common causes of congenital intestinal obstruction. However, the accuracy of prenatal ultrasound in diagnosing this condition has not been entirely ascertained. The aim of this study was to analyse the predictive accuracy of ultrasound in detecting SBA prenatally. METHODOLOGY: Retrospective study of all cases with prenatal suspicion or postnatal confirmed SBA seen in a tertiary fetal medicine and pediatric surgery units from 2007 to 2013. Cases with duodenal atresia were excluded from the study. The predictive accuracy of ultrasound and different ultrasound signs, alone and in combination, was calculated. RESULTS: 65 fetuses with prenatal suspicion or postnatal confirmed SBA were enrolled. 58 cases had full data and were included in the analysis. Predictive accuracy of ultrasound in detecting the presence of SBA was poor, with a sensitivity of 50% (95% CI 26.0-74.0) and a specificity of 70.59% (95% CI 52.5-84.9). The presence of both bowel dilatation ≥17 mm and polyhydramnios after 32 weeks of gestation slightly increased sensitivity (66.67%, 95% CI 34.9-90.1) and specificity (80.00%, 95% CI 44.4-97.5). CONCLUSIONS: In case of suspicion of SBA before the 3rd trimester, an ultrasound after 32 weeks should be performed to confirm the presence of both polyhydramnios and bowel dilatation >17 mm.

Intestinal obstruction due to dual gastrointestinal atresia in infants: diagnosis and management of 3 cases.

BACKGROUND: Several types of congenital lesions can cause complete or incomplete obstruction of the intestine. Our purpose is to present 3 neonates with dual intestinal type I atresia, i.e., simultaneous obstructive lesions at 2 locations in which the atresia manifested as diaphragm-like tissue. CASE PRESENTATION: All 3 cases were female infants ranging in age from 2 to 14 months. The common symptom in all cases was intermittent persistent vomiting. In some cases the vomitus was bilious, and other symptoms included abdominal distention and delayed meconium passage. Prior surgeries at another hospital were unsuccessful at relieving the symptoms in one case. One case had dual lesions in the colon, one dual lesions in the duodenum, and one atresia at both the distal portion of the ileum and the descending colon. Surgical exploration and removal of the lesions at our hospital was successful in all cases, and the infants were discharged in good condition. CONCLUSIONS: Type I atresia can manifest as a diaphragm-like tissue obstructing the continuity of gastrointestinal tract, and in rare cases multiple areas may be present. Base on the intermittent nature of the associated symptoms, diagnosis can be difficult and is often delayed. Physicians should be aware of this condition during the work-up of an infant with persistent intermittent vomiting.

Terminal ileum and total colonic duplication associated with a rectovestibular fistula in a child.

The presence of terminal ileum and complete colonic duplication associated with a rectovestibular fistula, caecal diverticulum and multiple appendixes in a child presents an extremely rare diagnostic and management conundrum. We report our surgical approach to successfully correcting this anomaly.

Meconial peritonitis in a rare association of partial ileal apple-peel atresia with small abdominal wall defect.

Intestinal atresia type III B (apple peel) and gastroschisis are both congenital malformations who require early surgical correction in neonatal age. Their association is very rare. We present the case of a full term infant with partial apple peel ileal atresia and a small defect of the anterior abdominal wall, complicated by in utero intestinal perforation and subsequent meconial peritonitis. We observed a partial atresia of small intestine, with involvement of terminal ileus savings of jejunum and a large part of the proximal ileum, small anterior abdominal wall defect with herniation of few bowel loops, intestinal malrotation. Paralytic ileus and infections are the main causes of morbidity and mortality at neonatal age. In our case, in spite of the mild phenotype, prognosis has been complicated by the onset of functional bowel obstruction, caused by chemical peritonitis resulting from contact with either amniotic fluid and meconium.

Symptomatic ileal duplication.

A pathology well known by pediatric surgeons, ileal duplication is in rare instances a cause of acute surgical abdomen in adults; that said, its atypical presentation often leads it to be mistaken for other etiologies. Even though it is benign in children, the risk of malignant transformation in adults should be taken into account in surgical procedures.

Sonographic diagnosis of fetal intestinal volvulus with ileal atresia: a case report.

Fetal intestinal volvulus is a rare life-threatening condition usually manifesting after birth with most cases being associated with intestinal malrotation. It appears on prenatal sonography (US) as a twisting of the bowel loops around the mesenteric artery, leading to mechanical obstruction and ischemic necrosis of the bowel. We report a case of intrauterine intestinal volvulus with ileal atresia, suspected when US revealed a typical "whirlpool" sign at 37 weeks' gestation, with a segment of markedly distended bowel loops and small amount of fetal ascites.

A rare congenital anomaly, bridge-like appendiceal fistula to the terminal ileum, demonstrated by MDCT.

Although appendiceal anatomical anomalies are very rare, understanding of the anatomical details of these anomalies is important for surgery. In this case report, we present images from multi-detector row computed tomography (MDCT) and histological findings of a rare anatomical appendiceal anomaly originating from the cecum and opening into the terminal ileum like a bridge. These anatomical details were clearly depicted on MDCT with multi-planar reconstruction. MDCT demonstrated a communication between the appendix and terminal ileum. Histological analysis revealed that a normal mucosal layer was maintained from the appendix to the connected ileum, without any evidence of inflammatory or neoplastic changes, and only thickening of the muscular layer of the appendix was identified. Based on these histological findings, the appendix was considered to represent an anatomical anomaly rather than secondary fistula caused by inflammation or neoplasm, which has not yet been reported.

Terminal ileum duplication: an unusual case of small bowel obstruction.

Duplications of the alimentary tract are uncommon congenital anomalies that usually present during infancy and early childhood. The case of an adolescent presenting with small bowel obstruction secondary to a duplication cyst is presented and the challenges in the management described.

[Caudal-epidural injection of adjuvants and local anaesthetics in the newborns undergoing surgery].

In 45 newborns, operated on for the inborn failures of gut, the markers of stress and indices of central hemodynamics were studied up. General anaesthesia was applied together with caudal-epidural injection of local anaesthetics in 15 newborns, in 15--the adjuvants--klophelline and promedol--were added to local anaesthetics, in 15--a standard atharalgesia was used. The best and most durable effect was noted while application of anesthesia in combination with adjuvants.

Ileal dysgenesis coexisting with multiple enteric duplication cysts in a child--MR enterography, CT, and Meckel scan appearances.

There are numerous causes of iron deficiency anemia due to gastrointestinal tract bleeding in children. While a very thorough history may elucidate common etiologies, such as cow's milk protein-induced colitis and nonsteroidal anti-inflammatory drug-related gastritis or peptic ulcer disease, other less frequent causes often present a diagnostic challenge. We present the MR enterography (MRE), CT and Meckel scan findings of ileal dysgenesis coexisting with multiple enteric duplication cysts in a young child who presented with chronic iron deficiency anemia, recurrent gastrointestinal tract bleeding and unexplained bowel perforation. In this case, MRE was able to identify and characterize each individual lesion and directly guide appropriate surgical management.

Antenatal sonographic features of ileal atresia.

AIM: The purpose of this study was to determine the antenatal sonographic features of ileal atresia. MATERIAL AND METHODS: We identified neonates with surgically-confirmed ileal atresia who had antenatal sonography performed in our institution between 1 January 1999 and 30 June 2009. The antenatal sonography images and reports were reviewed. RESULTS: Sixteen neonates had surgically-confirmed ileal atresia in our institution in 11 years. Seven fetuses (43.7%) did not have any intestinal abnormalities detected antenatally on ultrasonography. Nine fetuses (56.3%) had various sonographic features of ileal atresia, including multiple dilated bowel loops, ascites, cysts, and polyhydramnios with or without associated anomalies. Six of nine fetuses had multiple dilated bowel loops and two fetuses had ascites. One fetus had a large, cystic, mixed, echogenic dilatation of bowel. Polyhydramnios was present in three fetuses. Heart anomalies, kidney anomalies, or hydrops were present in four fetuses. Eight of 16 fetuses (50%) had other intestinal problems, including intussusceptions in one fetus, small bowel malrotation, meconium pseudocyst volvulus, meconium peritonitis, and a congenital band. CONCLUSION: The prenatal sonographic features of ileal atresia are not simple. Various sonographic findings are shown and ileal atresia was detected in about 60% of cases.