Persistent omphalomesenteric duct in an infant with trisomy 21.

We present the case of a term newborn with trisomy 21 who presented to the paediatric emergency department with periumbilical flare and green-brown discharge from a clamped umbilical cord, initially suspected to be omphalitis. However, it was noticed later, that when the infant strained or cried, a thick, bubbling and offensive green-brown discharge came out of the clamped umbilical cord with umbilical flatus. An ultrasound abdomen and umbilical cord confirmed the presence of a persistent omphalomesenteric duct (POMD). He was then transferred to the paediatric surgical unit. There, he underwent a laparotomy and surgical resection of the POMD and was discharged home 2 days later.

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.

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.

[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.

Paediatric Postoperative Intussusception.

Intussusception is one of the common causes of acute abdomen in early childhood, particularly in children younger than two years of age. The majority of cases in children are idiopathic. Pathologic lead points can be identified in only 25 percent of cases. Here. we present a case of 15 months female child with Ileoileal postoperative intussusception with an anastomotic margin as a lead point, following resection anastomosis done for patent vitello intestinal duct. Role of high clinical suspicion, investigations and judgement are highlighted in managing the case. Keywords: Anastomosis; ileoileal; postoperative intussusception; patent vitello-intestinal duct.

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.

Cutaneous presentations of omphalomesenteric duct remnant: A systematic review of the literature.

BACKGROUND: Disorders of the umbilicus are commonly seen in infancy, including hernias, infections, anomalies, granulomas, and malignancies. Meticulous inspection of the umbilicus at birth might reveal a persisting embryonic remnant, such as an omphalomesenteric duct (OMD), manifested by a variety of cutaneous signs, such as an umbilical mass, granulation tissue, or discharge. OBJECTIVE: To systematically review the available data regarding the presence and management of OMD remnant with cutaneous involvement to suggest a practical approach for diagnosis and treatment. METHODS: A systematic review of the literature evaluating OMD anomalies presenting with cutaneous symptoms was performed. In addition, an index case of an 11-month-old patient is presented. RESULTS: We included 59 publications reporting 536 cases; 97% of the patients whose age was noted were infants (mean age 11 months). In 7.5% of the cases, diagnosis was established only after treatment failure. In 6.4% of patients, nonlethal complications were reported, and in 10.3%, the outcome was death, partly due to delayed diagnosis or mismanagement. LIMITATIONS: Limited quality of the collected data, reporting bias. CONCLUSION: OMD is relatively rare; however, the clinician must consider this remnant while examining patients with umbilical abnormalities because mismanagement could cause severe morbidity and mortality.

Altered VEGF Signaling Leads to Defects in Heart Tube Elongation and Omphalomesenteric Vein Fusion in Quail Embryos.

Formation of the endocardial and myocardial heart tubes involves precise cardiac progenitor sorting and tissue displacements from the primary heart field to the embryonic midline-a process that is dependent on proper formation of conjoining great vessels, including the omphalomesenteric veins (OVs) and dorsal aortae. Using a combination of vascular endothelial growth factor (VEGF) over- and under-activation, fluorescence labeling of cardiac progenitors (endocardial and myocardial), and time-lapse imaging, we show that altering VEGF signaling results in previously unreported myocardial, in addition to vascular and endocardial phenotypes. Resultant data show: (1) exogenous VEGF leads to truncated endocardial and myocardial heart tubes and grossly dilated OVs; (2) decreased levels of VEGF receptor 2 tyrosine kinase signaling result in a severe abrogation of the endocardial tube, dorsal aortae, and OVs. Surprisingly, only slightly altered myocardial tube fusion and morphogenesis is observed. We conclude that VEGF has direct effects on the VEGF receptor 2-bearing endocardial and endothelial precursors, and that altered vascular morphology of the OVs also indirectly results in altered myocardial tube formation. Anat Rec, 302:175-185, 2019. © 2018 Wiley Periodicals, Inc.

Persistencia de conducto onfalomesenterico permeable / Patent onfalomesenteric conduct / Persistência do ducto onfalomesentérico permeável

La persistencia del conducto onfalomesentérico permeable es una de las formas de presentación menos frecuente, dentro de la patología, de los restos embrionarios derivados de este conducto. Se presenta el caso de un lactante de 30 días de vida a quien se le hace un diagnóstico de conducto onfalomesentérico permeable y se realiza cirugía resectiva, con una buena evolución postoperatoria inmediata y con alta a domicilio a los 8 días. Se revisan los datos de embriología así como de patología, la presentación clínica, los diagnósticos diferenciales y las opciones terapéuticas.

The persistence of permeable omphalosenteric duct is one of the less frequent forms of presentation, within the pathology, of the embryonic remnants derived from this duct. We present the case of a 30-day-old infant who is diagnosed with permeable omphalomesenteric duct, and resective surgery is performed, with a good postoperative evolution and with home discharge at 8 days. The embryology data as well as the pathology, the clinical presentation, the differential diagnoses and the therapeutic options are reviewed.

A persistência do ducto onfalossentérico permeável é uma das formas menos freqüentes de apresentação, dentro da patologia, dos remanescentes embrionários derivados desse ducto. Apresentamos o caso de um lactente de 30 dias que é diagnosticado comducto oncomumentérico permeável, sendo realizada cirurgia ressectiva, com boa evolução pós-operatória e com descarga domiciliar aos 8 dias. Os dados da embriologia, bem como a patologia, a apresentação clínica, os diagnósticos diferenciais e as opções terapêuticas são revisados.

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.

Hernia of the umbilical cord associated with a patent omphalomesenteric duct.

Congenital hernia of the cord is a different type of ventral abdominal wall defect in which the bowel usually herniates into the base of normally inserted umbilical cord through a patent umbilical ring. It is rare congenital anomaly with incidence of 1 in 5000. Although it was described as a distinct entity since 1920s it is often misdiagnosed as a small omphalocele. We present an unusal case of term male newborn with umbilical cord hernia associated with patent omphalomesenteric duct. The diagnose was made after birth despite antenatal ultrasound scans and it is managed successfully with uneventful recovery. If this is missdiagnosed, it could cause iatrogenic atresia of the ileum by clamping the umbilical cord after birth.

Unusual Ileal Anomalies Associated with Omphalomesenteric Duct Remnants.

The association between omphalomesenteric duct remnants and other digestive tract malformations is not rare. Most associated anomalies are reported with Meckel's diverticulum. We report two associated anomalies which were never reported: an ileal stenosis and an ileal duplication. Surgeons must be aware of associated anomalies to prevent post-operative complications.

Occult Adenoma in Patent Vitellointestinal Duct Presenting as an Umbilical Fistula: Cause for Concern?

INTRODUCTION: Prevalence of vitellointestinal duct anomalies varies from 2-4%. Though a completely patent vitellointestinal duct is a common symptomatic embryological defect with a prevalence of 0.0063-0.067%, other vitellointestinal abnormalities are rarely reported. Ours is the first case to describe histopathological features of an umbilical fistula harboring an occult tubular adenoma. CASE REPORT: We report a case of one-month old child, presenting with umbilical fistula for which excision and ileal anastomosis was performed. Routine histopathologic examination revealed an occult tubular adenoma in tip of the fistula. CONCLUSION: Adenoma arising in an umbilical fistula at such an early stage of infancy has never been reported before. Detection of occult adenoma warrants screening GI endoscopy, genetic testing for syndromic gastrointestinal adenomatosis and carcinomas and lifelong surveillance. Our case highlights the importance of routine histopathologic examination in detection of occult premalignant lesions as it significantly affects the patient management and prognosis.