Ileal prolapse through patent omphalomesenteric duct in a two year-old boy: a case report.

BACKGROUND: Patent omphalomesenteric duct is one of the birth defects included in the spectrum of vitelline duct abnormalities. It is a rare anomaly with estimated prevalence of 0.13-0.2% in the general population. The most common presentation of patent vitelline duct is yellowish or mucoid type umbilical discharge which is usually noted in neonatal age or infancy. The main stay of diagnosis is clinical and outcome is favorable as long as timely surgical correction is offered. Here we present a 2 years old male child who presented with ileal prolapse through patent vitelline duct which is an exceptional mode of presentation of this pathology. CASE PRESENTATION: 2 years old Ethiopian male child who was noticed to have umbilical discharge since early infancy presented with protrusion of pinkish mass per the umbilicus of 4 h duration. He had no signs and symptoms of bowel obstruction. Abdominal examination revealed a prolapsed bowel which was viable via the umbilicus which was about 6 cm long. Otherwise, he had no abdominal tenderness or rigidity. He was explored with a smiley incision just above the umbilicus. The prolapsed bowel was reduced gently to the abdominal cavity. The tract of the Patent vitelline duct was identified and completely resected along with a wedge of ileum at its base. Primary repair of the ileal end where the tract was inserted was done in two layers and abdomen was closed in layers. The child had smooth post op course and was discharged on the 4th post-operative day. CONCLUSION: Prolapse of a bowel through the umbilicus is unusual presentation of a rare anomaly namely patent vitelline duct. This presentation warrants early surgical intervention before bowel ischemia issues. Hence, all clinicians dealing with children should be aware of this rare pathology so that urgent surgical management can be offered.

Patent vitellointestinal duct with ileal prolapse in a newborn: A case report and literature review.

RATIONALE: Patent vitellointestinal duct is the most common omphalomesenteric duct anomaly to present with symptoms. PATIENT CONCERNS: A 10-day-old child presented with increase in the size of a polypoidal lesion into a large, "Y"-shaped reddish, prolapsing lesion, discharging gaseous, and fecal matter at her umbilicus. A laparoscopic exploration was performed, followed by wedge resection and anastomosis. No complications occurred during postoperative follow-up. DIAGNOSES: A patent vitellointestinal duct with ileal prolapse. INTERVENTIONS: The resection of extended intraperitoneal intestinal tube was performed. OUTCOMES: During the follow-up 3 months after surgery, the umbilical cord of the child healed well after surgery. LESSONS: Timely surgical treatment can minimize the occurrence of complications, and the overall prognosis is good after surgery.

Persistencia del conducto onfalomesentérico: otra causa de abdomen agudo / Persistence of the omphalomesenteric duct: another cause of acute abdomen

El conducto onfalomesentérico es una estructura embrionaria que comunica el saco vitelino con el intestino medio, generalmente desapareciendo entre la quinta y la novena semanas de vida intrauterina. La persistencia del conducto onfalomesentérico, presente hasta en un 2% de la población, es una anomalía congénita del tracto gastrointestinal que puede presentarse en forma de abdomen agudo, oclusión intestinal o hemorragia digestiva.(AU)

Ectopic pancreas, gastric, duodenal and colonic tissue in a case of persistent umbilical discharge: Report of two patients with review of literature.

One of the typical complaints in the pediatric population is umbilical discharge. Among the congenital causes, remnants of omphalomesenteric duct or patent urachus are often detected. On a few occasions, multiple types of ectopic tissue are present. We describe histopathologic findings of two cases reported recently at our center as pediatric umbilical lesions with associated ectopic tissue. Histopathology of the excised mass confirmed the patent omphalomesenteric duct with ectopic gastric, duodenal, and colonic mucosa and pancreatic tissue in two patients with the clinical presentation of umbilical discharge. There were no associated congenital anomalies in these patients. The presence of multiple ectopic gastrointestinal mucosa and pancreas in the umbilical mass is unusual. Herein, we report these cases because of its rarity, multiple ectopic tissues, and reviewing the literature of the reported cases of multiple ectopic tissues.

Next Generation Sequencing in Vitellointestinal Duct Adenoma: Existence of Adenoma-Carcinoma Sequence or too Early to Predict?

Patent vitello-intestinal duct with adenoma is rare presentation. We report a case of a 1-month-old boy presenting with intermittent passage of stool and blood from the umbilicus since birth. On local examination polypoidal mass measuring 1×1 cm was seen protruding from umbilicus with faecal discharge. Ultrasound was performed which revealed a tubular hyperechoic structure, extending from umbilicus to part of small intestine measuring 30 ×30 mm and clinical diagnosis of patent vitello-intestinal duct was given, exploratory laparotomy, excision with umbilicoplasty done, and send for histopathological examination. On histopathological examination, patent vitello-intestinal duct adenoma was rendered and next generation sequencing (NGS) was performed revealing somatic mutation of KRAS (NM_033360.4; c.38G>A; p.Gly12Asp). To our knowledge, this is the first report of the adenoma in patent vitello-intestinal duct with NGS analysis. This case emphasizes the importance of thorough microscopic examination of resected patent vitello-intestinal duct and mutational analysis of the early lesions.

Congenital ileal-mesenteric fibrous band remnant of the vitelline artery causing small bowel obstruction in an adult female: a case report.

The vitelline circulation connects the fetus and yolk sac in the first few weeks of fetal development. Its components are the vitelline duct, vitelline arteries and vitelline vein. This gradually breaks down as the placenta grows and overtakes the function of yolk sac as the primary nutrition source. In the event of persistence of these structures, multiple anatomical anomalies can arise such as a Meckel´s diverticulum, fibrous bands and others. We report on a rare finding of an ileal-mesenteric fibrous band remnant of a vitelline artery causing small bowel obstruction in an adult female. Our patient, a 40-year-old female presented with both clinical and radiological signs of small bowel obstruction. She had had no previous abdominal surgery or abdominal trauma. Intraoperatively we found an isolated ileal-mesenteric fibrous band situated at approximately 7 cm from the ileocecal junction. It spanned from the antimesenteric border of the terminal ileum to the border of the mesentery at a 15 cm breadth. In the snare-like loop that was created, part of the small bowel was trapped, creating a strangulated internal hernia which presented as a small bowel obstruction. The fibrous band was transected and the viable small bowel was freed during a laparotomy procedure. The post-operative period was uneventful and the patient was discharged on day 5. A fibrous band should be considered one of the rare causes of small bowel obstruction in a virgin abdomen in adults.

Persistent omphalomesenteric duct and urachus presenting as an umbilical hernia.

Vitellointestinal duct anomalies, although one of the most frequent malformations to be found (2%-3% in population), they are most unlikely to cause symptoms. A persistent Vitellointestinal duct can induce abdominal pain, bowel obstruction, intestinal haemorrhage and umbilical sinus, fistula or hernia which commonly occurs in children. Patent vitellointestinal duct or persistent omphalomesenteric duct is a very unusual congenital anomaly which occurs in 2% of the population related to the embryonic yolk stalk. Similarly, urachal anomalies remain a rare finding, with the most common being a cyst or sinus followed by patent urachus and rarely a urachal diverticulum. Presenting symptoms include periumbilical discharge, pain and a palpable mass.Here, we report a case of an adult patient with patent vitellointestinal duct and urachus identified intraoperatively on diagnostic laparoscopy when being operated for umbilical hernia repair.

Umbilical Appendix Masquerading as a Patent Omphalomesenteric Duct in a Neonate.

The umbilicus is the site of a number of well-recognized and unusual abnormalities. Well-known neonatal umbilical abnormalities include umbilical hernias, granulomas/polyps, and congenital remnants of development. In this article, we describe a rare case of an appendix draining through the umbilicus of a neonate. In the literature, there are only 15 cases with possible umbilical appendix. We describe this rare case along with a review of the literature and discuss the underlying pathophysiology.

Omphalomesenteric Duct Anomalies in Children: A Multimodality Overview.

The omphalomesenteric duct is an embryologic structure that connects the yolk sac with the primitive midgut of the developing fetus. Omphalomesenteric duct anomalies include a group of entities that result from failed resorption of the omphalomesenteric duct. These anomalies include Meckel diverticulum, omphalomesenteric fistula, fibrous bands, cysts, and umbilical polyps. Meckel diverticulum is the most common congenital anomaly of the gastrointestinal tract and is usually asymptomatic. Symptoms develop when Meckel diverticulum involves complications such as hemorrhage, inflammation, and perforation, or when it causes intussusception or bowel obstruction. Hemorrhage is the most common complication of Meckel diverticulum, and technetium 99m-pertechnetate scintigraphy is the imaging modality of choice for detecting acute bleeding. US and CT are commonly used for the evaluation of patients with other complications such as obstruction and inflammation. Nevertheless, the diagnosis of these complications can be challenging, as their clinical manifestations are usually nonspecific and can masquerade as other acute intraabdominal entities such as appendicitis, inflammatory bowel disease, or other causes of bowel obstruction. There are other umbilical disorders, such as urachal remnants and umbilical granuloma, that may present with symptoms and imaging findings similar to those of omphalomesenteric duct anomalies. An accurate preoperative diagnosis of omphalomesenteric duct anomaly is crucial for appropriate management and a better outcome, particularly when these anomalies manifest as a life-threatening condition. The authors review the anatomy, clinical features, and complications of omphalomesenteric duct anomalies in children, describing the relevant differential diagnoses and associated imaging findings seen with different imaging modalities. ©RSNA, 2021.

Histomorphology of the lesions of the umbilicus: Are we naïve about the navel?

CONTEXT: Twelve-year retrospective study of surgically excised umbilical lesions received for histopathology in a pediatric tertiary care hospital. AIMS: To study histopathology of the umbilical lesions and review pertinent literature on the embryological basis of these lesions. SUBJECTS AND METHODS: We reviewed cases of umbilical lesions and classified them as "developmental" and "others." Developmental cases were sub-classified based on the mechanism as those due to defect in the closure of body wall, defect in the closure of the umbilical ring, persistence of embryonic remnants, or failure of epithelization. Persistent embryonic remnants were subdivided into fistula, sinus, and cyst. Histology of all the cases was studied and the different types of tissue in omphalomesenteric ducts (OMD) remnants were identified. STATISTICAL ANALYSIS USED: Descriptive statistics were used as required. RESULTS: Seventy-one cases in the age range of 1 day to 13 years were studied and male preponderance was noted. The developmental lesions included 4 omphalocele sacs with dense acute inflammation, 2 umbilical hernial sacs with fibrocollagenous tissue, 30 OMD remnants, 10 allantoic duct remnants, 19 umbilical granulomas, and 2 cases showing more than one developmental mechanism. Four cases were classified as "others" including 3 epidermal inclusion cysts and 1 skin tag. Among OMD remnants, sinuses (arising from the distal tract) were found to be the most common. Histological examination of the OMD remnants showed enteric (18), enteric and gastric (5), colonic (4), enteric and colonic (2), and pancreatic and enteric and gastric mucosae (1). CONCLUSION: Accurate diagnosis is essential for definite treatment of these lesions.

Adenocarcinoma mucinoso uracal / Urachal mucinous adenocarcinoma

Introducción: El uraco o conducto onfalomesentérico es una estructura embrionaria que conecta el intestino medio y el saco vitelino, que involuciona antes del tercer trimestre de embarazo. Objetivo: Presentar un caso con diagnóstico de adenocarcinoma uracal, neoplasia maligna rara. Desarrollo: Paciente de 50 años de edad sin antecedentes patológicos, con cuadro clínico inicial de ascitis y dolor abdominal, al examen físico masa palpable en hipocondrio-fosa iliaca izquierda que en la tomografía abdominal se observa una masa adyacente a la cúpula vesical y pared anterior del abdomen. El resultado de la biopsia reportó un adenocarcinoma mucinoso del conducto onfalomesentérico (uracal). Al momento del diagnóstico, el paciente se encontró en un estadio IVB según el sistema Sheldon y otros y un IV según sistema Mayo, etapa avanzada de la enfermedad que determina mal pronóstico. Se instauró tratamiento con quimioterapia neoadyuvante, quirúrgico y continuación con quimioterapia adyuvante, el cual continúa, actualmente, con una evolución estable. Conclusiones: Esta es una neoplasia rara. Sospechar su diagnóstico llevará a un diagnóstico temprano, lo que mejorará el pronóstico y sobrevida de los pacientes afectados(AU)

Introduction: The urachus or omphalomesenteric duct is an embryonic structure connecting the midgut and the yolk sac, which regresses before the third trimester of pregnancy. Objective: To report a case with a diagnosis of urachal adenocarcinoma, a rare malignant neoplasm. Case report: A 50-year-old patient with no pathological history, who had an initial clinical condition of ascites and abdominal pain. At physical examination, we found a palpable mass in the left hypochondrium - iliac fossa, which revealed a mass next to the bladder dome and anterior wall on the abdominal tomography of the abdomen. The biopsy result reported a mucinous adenocarcinoma of the omphalomesenteric duct (urachal). At the time of diagnosis, the patient was in stage IVB according to the Sheldon et al system and IV according to the Mayo system, an advanced stage of the disease that determines poor prognosis. Treatment with neoadjuvant and surgical chemotherapy was established and adjuvant chemotherapy to follow, which currently continues with a stable evolution. Conclusions: This is a rare neoplasm. Suspecting its diagnosis will lead to an early diagnosis, which will improve the prognosis and survival of affected patients(AU)

[A neonate with soft tissue protruding through the umbilicus]. / Een neonaat met weefsel uit de navel.

A 6-week-old neonate presented with soft tissue protruding through the umbilicus due to a persisting ductus omphalo-entericus. This remnant was surgically removed the next day.

[Omphalomesenteric fistulas; epidemiological diagnostic and therapeutic features: study of four cases in the Department of Pediatric Surgery at the Aristide Le Dantec University Hospital in Dakar]. / Fistules omphalo-mésenteriques; aspects épidémio logiques, diagnostiques et thérapeutiques: à propos de quatre observations au Service de Chirurgie Pédiatrique du CHU Aristide Le Dantec de Dakar.

This study aims to determine the epidemiological, therapeutic and diagnostic features of omphalomesenteric fistulas (OMF). We conducted a study of four cases over a period of 10 years, from January 2004 to December 2013. The parameters studied were: frequency, age, sex, clinical and radiological signs, therapeutic and evolutionary features. Frequency was 0.4 cases per year. Patients were aged 11 days, 40 days, 45 days and 3 years respectively (three girls and one boy). Clinical examination showed intestinal fluid discharge from the belly button and belly button bud catheterisable in all the cases. The bud was prolapsed in the patient aged 45 days. Fistulography performed in two cases helped to confirm the diagnosis by showing a communication between the fistula and the small intestine. The assessment of malformations revealed congenital cyanogen heart disease with interventricular communication in the newborn aged 45 days, anorectal cloacal malformation associated with urachus fistula in the newborn aged 11 days. All patients underwent surgery. Semicircular periumbilical incision was performed in the absence of associated abdominopelvic malformations. A communication between the fistula and the ileum was found in the majority of cases. Bowel resection with termino-terminal anastomosis was performed in three cases. Cuneiform resection was performed in one case and was completed by complete resection of the urachal fistulous tract and bladder suture, with colostomy in newborn with urachal fistula and anorectal cloacal malformation. The postoperative course was marked by non-febrile seizures in the first child with good evolution and by superficial parietal suppuration followed by death due to cardiac decompensation in the third case. Omphalomesenteric fistula is rare. Diagnosis is based on clinical examination complemented by the fistulography. Surgical treatment using semicircular periumbilical incision gives good results. However, the assessment of malformations is necessary.