Enteric Duplication Research Articles

Antenatal diagnosis of microvillus inclusion disease

Microvillus inclusion disease is a rare autosomal recessive disorder due to defective apical surface of the enterocytes presenting with severe watery diarrhea starting at birth. We describe a female infant who had antenatal diagnosis of microvillus inclusion disease. At 36th gestational week of a 32-year-old woman ultrasound examination revealed dilatation of fetal sigmoid colon. The amniotic fluid level was normal. An amniocentesis was performed to rule out congenital sodium and chloride diarrhea in the prenatal period. The patient didn't prefer to undergo genetic tests. In conclusion, prenatal ultrasonographic identification of dilated bowel loops without polyhydramnios suggests differential diagnosis of microvillus inclusion disease in addition to congenital chloride diarrhea, jejunoileal atresia, volvulus, meconium ileus, Hirschsprung disease, enteric duplications, anorectal atresia.

Cecal Duplication

Background: Enteric duplication cysts are rare congenital malformations that are most commonly found in the ileum and ileocecal junction, followed by the esophagus, stomach, and duodenum. Although most present in the 1st 2 years, they are often difficult to diagnose as both presentation and radiological findings can be variable and nonspecific. Clinical Description: We present the case report of an 11-month-old child who presented with nonbilious vomiting and diarrhea. There was no fever, abdominal distension, blood in stools, or crying on defecation. A provisional diagnosis of acute gastroenteritis was kept and management started as per the standard protocol. Management: A series of investigations were carried out when the child did not improve and developed bilious vomiting. Ultrasound findings were suggestive of an ileal duplication cyst which was confirmed by magnetic resonance imaging. The child then underwent surgery where cecal duplication cyst was confirmed, which was previously missed due to a malpositioned cecum. Conclusion: Gastrointestinal duplication cysts are rare developmental anomalies that may present as an acute abdomen in a young child. The possibility of the duplication cyst should be kept as a differential diagnosis in a child presenting with unexplained vomiting, abdominal distension, or pain. Early suspicion, investigation, and intervention help in faster recovery with minimal complications.

Role of Imaging in the Diagnosis of an Enteric Duplication Cyst of the Esophagus: A Case Report

Aims: Show the importance of imaging in the diagnosis of enteric cysts of the esophagus and the main differences with other mediastinal cystic lesions. Presentation of case: We report a case of an enteric cyst of the esophagus, in a 25 year old patient, with dyspnea on exertion. X-ray shows an oval opacity in the posterior mediastinum and the CT scan and MRI show a cystic mass of the posterior mediastinum, presenting intimate contact with the esophagus, with discreet wall contrast enhancement. Discussion and Conclusion: Enteric duplication cysts are rare congenital malformations formed during the embryonic development of the digestive tract. They are usually detected prenatally or in the first years of life. Their size, location, type and presence of complications produce a varied clinical presentation and different imaging findings. They most frequently occur in the small intestine, particularly the ileum, but can occur anywhere along the gastrointestinal tract. In the case of an enteric cyst of the esophagus, the first imaging method for diagnosis is the chest x-ray because of the breathing difficulty patients can experience due to compression of the airway. Magnetic resonance (MR) and computed tomography (CT) can help the radiologist in the diagnosis and the surgeon by evaluating more precisely the mass measurements and describing its anatomical relationship with the vessels and surrounding organs. Confirmation remains histological.

English

BACKGROUND Abdominal cystic lesions are not so uncommon in the paediatric age group. Ultrasonography (USG) is the initial investigation of choice for detection of lesions. Computed tomography (CT) and magnetic resonance imaging (MRI) further compliment the findings of USG and help in the final diagnosis of various abdominal lesions in this age group. Because of the overlap in imaging features, histologic analysis is usually necessary to establish a diagnosis. The major role of the radiologist is to document the cystic nature of these abdominal masses and to determine the origin. Our aim was to study the incidence of cystic abdominal lesions in the paediatric age group and role of imaging in the diagnosis of various types of cystic lesions. METHODS This is a cross sectional study of 60 children who have been referred to radiology department with abdominal symptoms, over a period of 18 months (from April 2018 to September 2019) in Niloufer hospital, Hyderabad. Children suspected to have abdominal lesions have been referred to radiology department. USG is the initial investigation done and further CT / MRI has been done according to the findings on USG. Paediatric patients of age day 1 to 12 years, both male and female suspected to have abdominal cystic lesions either clinically or sonologically have been included in the study. Retroperitoneal lesions are excluded from study. RESULTS Out of the 60 patients studied, 44 patients were females and the rest were males. The most affected age group was between 0 - 1 year which constituted to 50 percent of the total cases studied. The most common pathologies are ovarian cyst and duplication cyst which constituted about 49 % of the cases. Statistical significance has been observed between the gender and incidence of cystic lesions. CONCLUSIONS Imaging plays a key role in the evaluation of various types of cystic lesions in the paediatric age group and arrive at a particular diagnosis based on specific imaging features. The radiologist must consider patient age, clinical parameters and imaging findings to formulate the likely diagnosis of cystic lesions. USG being cost effective, widely available and with no risk of radiation has been chosen as the first modality for investigation. KEYWORDS Cystic Lesions, USG, CT / MRI, Ovarian Cyst, Enteric Duplication Cyst

Triplication of small bowel with perforated peptic ulcer: Report of a rarity with literature review

AbstractTriplication of small bowel is an extremely rare variant of enteric duplication with only one previously reported case in a neonate. Most enteric duplications are presented in neonates as intestinal obstruction. Herein we present a case of 3‐year‐old girl with acute abdomen due to perforation of triplicated ileum aiming to highlight the characteristic presenting features, the diagnostic difficulties and challenges of surgical intervention for this condition.

İntestinal Volvulusu Taklit Eden Y Şekilli Kolonik Duplikasyon: Olgu Sunumu ve Literatür İncelemesi

Enteric duplications are rare congenital anomalies found anywhere from mouth to anus. Colonic duplications constitute about 13% of all enteric duplications. In this report a 6-year-old boy with chronic abdominal pain for a duration of last 2 years requiring intermittent hospital admissions was diagnosed as colonic duplication mimicking intestinal volvulus. Clinical findings are nonspecific and definitive diagnosis can only be made during surgical intervention and surgical treatment is advocated for all duplications. The topic is discussed under the light of relevant literature with a brief a brief literature review.

Ileocolic Intussusception in an Infant with Ileal Duplication Cyst as a Lead Point- A Case Report

Gastrointestinal duplication cysts are uncommon congenital malformations, with small intestine being the most common site, followed by colon and stomach. It can have variable presentations such as intestinal obstruction, bleeding, palpable mass, or rarely volvulus. Here, the authors report a case of intussusception in a two-year-old child, presented with complaints of bilious vomiting and abdominal distension for three days with X-ray features suggestive of bowel obstruction. Ultrasonography revealed intussusception with ileal duplication cyst as the lead point, which was confirmed on exploratory laparotomy. In a paediatric patient, enteric duplication cyst should be included in the differential diagnosis of a cystic lesion as the lead point in intussusception.

A Rare Case of Retroperitoneal Isolated Enteric Duplication Cyst with Malignant Transformation

Abstract Introduction/Objective Isolated enteric duplication cysts (IEDC) are rare congenital abnormalities that do not have a communication with the normal intestinal lumen. They are usually recognized before the age of 2 years. They are rarely encountered in adults and very seldom undergo malignant change. These cysts are predominantly located within the abdominal cavity. They histologically demonstrate gastrointestinal mucosal lining and smooth muscle in the wall. Methods We report a case of a 45-year-old African American female who was evaluated for a pain in the left-sided abdomen and pelvis. Magnetic resonance imaging revealed a large multilocular cystic retroperitoneal mass, extending into the left iliac fossa and anteriorly displacing the colon. No other mass lesion was radiologically identified. The main radiologic impression was sarcoma. A biopsy was performed, and a pathologic diagnosis of adenocarcinoma was established with a consideration of primary retroperitoneal mucinous cystadenocarcinoma. An en bloc surgical resection of the retroperitoneal mass with adjacent segment of the descending colon was accomplished. Gross examination revealed an 11 x 5 x 4 cm cystic mass, containing a necrotic fluid and adherent to a 4 cm long segment of descending colon. Microscopically, the mass was lined by neoplastic cells arranged into tubules, villi and glands. Areas of necrosis were present. The wall of the cystic mass showed extensive hyalinized fibrosis and focal invasion. The neoplastic cells had positive immunoreactivity for CK7, CK20 and CDX2, but were negative for PAX8, PR and ER. Desmin immunostain and trichrome special stain highlighted a focus of smooth muscle bundle within the wall of the mass. These findings led to the pathologic diagnosis of adenocarcinoma arising in retroperitoneal IEDC. Conclusion In conclusion, we present this case because of its unusual presentation and location. Recognition of this entity and its rare malignant potential is important as it can pose a diagnostic challenge to pathologists.

Tubular enteric duplication presenting as jejuno-colic fistula in a 38 years old adult

Colo-enteric fistula is a rare entity, malignancy being the commonest cause. Both adenocarcinoma and rarely lymphoma have been known to cause it. Benign jejuno-colic fistulas are mostly secondary to tuberculosis or inflammatory bowel disease. Here we present a case of a young adult male, who presented with altered bowel habits. Colonoscopy and oesophago-gastro-duodenoscopy (OGD) revealed a fistulous tract between the proximal jejunum to the splenic flexure of colon. Surgery revealed a small fistulous connection between the above-mentioned parts of the bowel about 1cm long. Histopathology demonstrated it to have all the layers of the intestinal anatomy, hence indicating a possibility of congenital aetiology, probably enteric duplication of communicating tubular variety.

Acute neonatal volvulus with congenital enteric duplication, presenting with “currant jelly” stool

Abstract We present a clinicopathologic review of a 36-week gestation neonate who acutely presents with currant jelly stool, abdominal tenderness without distension, and dark-colored emesis, found to have an enteric duplication cyst with secondary jejunal volvulus and associated bowel wall ischemia and necrosis. Enteric duplication cysts may occur at all levels of the gastrointestinal tract, and may manifest a range of clinical presentations. Acute presentation with currant jelly stool, usually described with intussusception, has not been previously reported with neonatal volvulus.

Respiratory epithelium in an enteric duplication cyst at the ileocecal valve

Abstract Although a duplication cyst of the alimentary tract is rare, it can occur anywhere in the alimentary tract. The inner epithelial lining of a duplication cyst mostly consists of the gastrointestinal epithelium. However, studies have reported that foregut duplication cysts can have a respiratory epithelium lining. It is rare, however, that respiratory epithelium covers midgut duplication cysts. We herein report an enteral duplication lined with respiratory epithelium, occurring in an 18-month-old girl. Our study highlights the need for more studies about the embryonic development of duplication cysts are needed.

Enteric duplication cyst in a neonate: A case report

Duplication cysts of intestine are rare congenital malformations formed due to some developmental anomaly of gastrointestinal tract during embryonic period. They can occur in any portion of the gastrointestinal tract but are more commonly seen in small intestine. They have a variable clinical presentation depending on the size, site and type, or they may remain asymptomatic. In most of the cases they are detected during infancy or early childhood and uncommon in adults. Antenatal ultrasound can detect the condition in fetus and helps in follow- ups subsequently. Treatment of asymptomatic case is controversial, however early intervention prevents complications. Herein, we share our experience of managing a neonate with enteric duplication cyst, detected antenatally.

Retroperitoneal isolated enteric duplication cyst

Abstract Enteric duplication cysts are rare congenital malformations of the gastrointestinal tract with an estimated incidence of 1:100,000 live births. Fewer still are isolated enteric duplication cysts (IEDC). Accurate diagnosis and timely excision of IEDCs can help in avoiding possible complications including bleeding from gastric mucosa and malignant transformation later in life. Currently, in the paediatric population, there are twenty reported cases in the literature worldwide. Of these, only three have been described in the retro peritoneum. We present a retroperitoneal IEDC, which was juxtaposed to the inferior vena cava. To the best of our knowledge, this has not been reported before in literature.

Laparoscopic resection of ileocaecal duplication in children (report of 15 cases).

Background:Enteric duplication is a congenital anomaly with varied clinical presentation that requires surgical resection for definitive treatment. Ileocaecal (IC) duplications are duplications located at the IC junction, not clearly identified in all the published series. The reported treatment is IC resection and ileocolic anastomosis. The purpose of our study was to present our experience in successfully resection of IC duplication by laparoscope, thus avoiding bowel resection in children.Materials and Methods:A retrospective review was conducted of medical records of 15 patients with diagnosis of IC duplication, treated in the Department of Paediatric Surgery of our hospital, within the period from November 2013 to September 2018.Results:Laparoscopic resection of IC duplication was successfully performed in all children without bowel resection. The operation time was 50–90 min (55 ± 10 min), and the post-operative hospitalization time was 5–7 days (average, 6 days). The 15 patients were followed up for 6–12 months (average, 10 months). No recurrence was found by abdominal ultrasound examination. The wound had small scars with good appearance of umbilicus.Conclusions:The laparoscopic approach allows for confirming the diagnosis and accurately defining the exact site of duplication, as well as for effective and safe treatment. Laparoscopic excision of IC duplication without bowel resection is a safe option and is worth promoting.

Minimally Invasive Surgery for Congenital Abdominal Cystic Lesions in Newborns and Infants.

In the variety of congenital abdominal cystic lesions (CACL) of different origin, ovarian cyst is the most common intra-abdominal pathology in female neonates. The prognosis and timing of treatment varies depending on the nature of CACL. This study aimed to assess the results of diagnostics and treatment of CACL. A retrospective analysis was performed of 39 cases of CACL, with the spectrum including ovarian, enteric, mesenteric and pancreatic origin. Outcome of minimally invasive surgery, open surgery or conservative approach was analyzed. Twenty-eight neonates underwent surgery, while 11 were treated conservatively. Twenty patients were treated with a laparoscopic technique and eight with laparotomy combined with laparoscopy. Final diagnosis included: Fifteen cases of ovarian pathology (ovarian torsion in 11 cases), 12 treated laparoscopically and three with laparotomy, six enteric duplications (four laparoscopic and two laparotomic), three mesenteric cysts (one laparoscopic and two laparotomic), two pancreatic cysts (both laparoscopic only), two duodenal stenoses, including duodenal septum (both laparotomies with Heineke-Mikulicz plasty). No blood transfusion apart from two cases requiring re-laparotomy and no early complications were observed in any case; no death occurred. With the strategy of management based on ultrasound and laboratory data, a laparoscopically assisted minimal access approach resulted in minimal risk of complications and complete recovery in all patients, leading to exclusion of oncological risk.

A case report of enteric duplication cyst in adult

A case report of enteric duplication cyst in adult

Pyloric Duplication in Ten Years Old Girl: A Case Report

Gastrointestinal duplication cysts are rare congenital anomalies. Pyloric duplication represents 2.2% of all gastrointestinal tract duplications. The presentations merely depend on the site of occurrence, size and type of the cyst, and presence of ectopic mucosal lining. A ten years old girl vomited for two months, accompanied with stomach ache and difficulty in defecation. The color of stool sometimes was black. The body weight decreased twelve kilograms in two months. On physical examination, no abdominal distention and no palpable mass was found, bowel sound was decreased and there was tenderness in epigastric area. Nutritional status of patient was severe malnutrition. Laboratory test revealed mild hypochromic microcytic anemia, severe hyponatremia, severe hypokalemia, and hypoalbuminemia. Abdominal ultrasound revealed thickening of the gastric wall and upper gastrointestinal contrast study revealed partial stenosis with thickening of pyloric wall. Esophagogastroduodenoscopy revealed multiple gastric ulcers and gastric outlet obstruction. Cyst duplication and stricture in the pylorus were found during surgical procedure. The pediatric surgeon performed an excision of duplication cyst and gastroduodenostomy side to side anastomosis. Histopathologic examination from cyst confirmed the enteric duplication cyst. The patient was discharged in good condition. We concluded that pyloric duplication is considered as one of the differential diagnosis in children with symptoms of gastric outlet obstruction. Appropriate surgical procedures should be undertaken to avoid complications.

Gastric duplication cyst with multiple ectopic pancreatic tissues: A case report and review of literature

A gastric duplication cyst (GDC) is a type of enteric duplication cyst. It can co-exist with an ectopic tissue. This was a female toddler with a GDC at the greater curvature. An abdominal ultrasound and a contrast-enhanced computed tomogram suggested the cyst. She had laparotomy, complete cyst and partial gastric excision with the removal of extragastric pancreatic tissue. The histology report came out as a cyst with associated intracystic and an extracystic pancreatic tissue. She made a clinical improvement. GDC can be associated with both intracystic and extracystic ectopic pancreatic tissues. This should be kept in mind when choosing the modality of treatment.

Dynamic Compression-A New and Practical Technique for the Sonographic Diagnosis of Enteric Duplication.

This study aims to examine the utility of dynamic compression and other sonographic signs in diagnosing enteric duplication. This study reviewed real-time sonographic findings from ultrasound examinations performed by the author between April 2015 and March 2017, including 4 consecutive cases of enteric duplication and 7 cases of other abdominal cysts (control group). The ability of dynamic compression to separate the lesion from the adjacent intestine was analyzed, as were other sonographic signs, including the 5-layer sign, peristalsis, and the split-wall sign. The 5-layer sign and peristalsis were seen in only 1 of 4 cases of enteric duplication. The split-wall sign was positive in all 4 cases of enteric duplication. In a case of gastric duplication, the split-wall sign was not detected during the first examination. One case showed an atypical white split wall, and 3 cases showed a pseudo-split wall; however, this pseudo-split wall was differentiated from the true split-wall sign using dynamic compression. The dynamic compression technique correctly differentiated enteric duplication from other abdominal cysts in all but 2 cases (82%). The 5-layer sign and peristalsis were not sensitive for enteric duplication. The split-wall sign may have the highest sensitivity and specificity for enteric duplication, but finding this sign is technically difficult and interpretation is complicated by atypical variations. Dynamic compression differentiated enteric duplication from other abdominal cysts with 82% accuracy, and this technique was simple to perform and easier to interpret than the split-wall sign.

Clinical characteristics of gastrointestinal tract duplications in children: A single-institution series review.

Due to the various presentations of gastrointestinal tract duplications (GTD), diagnosing and management for this disease might be varied and difficult. We intend to improve the experiences for these difficult, in terms of the clinical presentations, diagnostic investigations, management.We reviewed recent literature and retrospectively analyzed 72 pediatric patients with enteric duplication. Diagnosis was confirmed by surgery and pathological examination for imaging characteristics and clinical and pathological features.The ages of patients ranged from one month to 12.5 years. The clinical presentations of the patients included 57 cases with abdominal pain, followed with nausea or vomiting, abdominal distension, etc. All of the patients were diagnosed by ultrasonography, and most of them presented as intra-abdominal cystic masses. Four cases were diagnosed with the cysts other than GTDs, like, mesenteric cyst, chledochal cyst and abscess, and so on. Computed tomography was performed on 65 patients. X-rays and barium meal showed the outline of the cyst structure, with intestinal displacement due to the pressure from the cyst. Among the 72 cases of enteric duplication, 45 were located with ileocecal area, 41 were ileal and 8 were colonic duplications.Enteric duplication is very rare in children and is prone to misdiagnosis. The preoperative diagnosis of enteric duplication can be improved through comprehensive analysis of various imaging exams and closely related clinical presentations.