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.

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.

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.

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.

These umbilical lesions weren't granulomas after all.

Consider a VID remnant as part of the differential diagnosis for any patient who has what appears to be a granulomatous umbilical lesion. Order ultrasonography to evaluate a suspected VID, especially for lesions that fail to respond to 2 or 3 silver nitrate treatments. Surgical excision of a VID remnant is usually curative.

Latent vitellointestinal duct sinus presenting with massive lower gastrointestinal bleeding in an adolescent.

A 12-year-old boy with a history, at birth, of a weeping pink fleshy lesion after his umbilical cord detached, requiring repeated chemical cauterisation, presented with massive lower gastrointestinal bleeding and required resuscitation and blood transfusion. Augmented Tc99m nuclear medicine scan confirmed ectopic gastric mucosa. The lateral view suggested its attachment behind the umbilicus. At exploration, a latent vitellointestinal duct sinus with ectopic gastric mucosal mass was found. Segmental resection of the sinus and mass excision with primary anastomosis and incidental appendicectomy was curative. Pink fleshy mass discharging coloured fluid at the umbilicus following detachment of umbilical cord should be considered a remnant of vitellointestinal duct unless proved otherwise. A pink lesion with yellowish discharge resistant to chemical cauterisation should raise the suspicion of embryonic structures. Latent vitellointestinal sinus is a new lesion in the spectrum of umbilical anomalies. Lateral view of the nuclear medicine scan is helpful in locating the site.

Intestinal prolapse through a persistent omphalomesenteric duct causing small-bowel obstruction.

Persistent omphalomesenteric duct as a cause of small-bowel obstruction is an exceptional finding. A neonate presented with occlusion due to intestinal prolapse through a persistent omphalomesenteric duct. Remnants of the duct were successfully resected, and the postoperative course was uneventful. We discuss the presentation of omphalomesenteric duct and its management.

Coexistence of multiple omphalomesenteric duct anomalies.

The omphalomesenteric duct is an embryonic structure which connects the yolk sac to the midgut. The omphalomesenteric duct attenuates between the 5th and 9th week of gestation. Failure of the omphalomesenteric duct involution, either partial or complete, results in various omphalomesenteric duct remnants including Meckel's diverticulum, patent vitelline duct, fibrous band, sinus tract, umbilical polyp and cyst. Omphalomesenteric duct remnants are present in 2% of the population but related diseases have seldom been reported in adults. The simultaneous presence of sinus tract, omphalomesenteric cyst, fibrous ligament and Meckel's diverticulum has, according to authors' knowledge, never been reported. We present a case of a 23 years old male with persisting umbilical discharge for 2 years in whom there was coexistence of the above mentioned anomalies of the omphalomesenteric duct.

Postoperative ileo-ileal intussusception following surgical resection of patent omphalomesenteric duct.

A female neonate with patent omphalomesenteric duct was treated by ductal excision. Six days following surgery, she developed vomiting and abdominal distension. On plain radiographs, distended bowel loops with multiple, air-fluid levels consistent with an intestinal obstruction were evident. Sonography demonstrated an ileo-ileal intussusception. At surgery, a 3-cm, ileo-ileal intussusception was noted proximal to the previous Lembert suture site and was manually reduced. Awareness of the possibility of small-bowel intussusception following surgery was critical to ensuring prompt and successful treatment of this infant.

Prolapse of bowel via patent vitello intestinal duct--a rare occurrence.

This report describes the presentation of prolapse of small bowel through the patent omphalomesenteric or vitello intestinal duct in a child. In spite of diagnosing the anomaly earlier, there was delay in surgical intervention that led to prolapse of the small bowel through patent remnants, which was life threatening. The patient presented to us with questionable viability of prolapsed bowel. Early surgery is recommended for this entity. As this is a very rare occurrence, it is being reported with a brief review of the relevant literature.

Omphalomesenteric duct remnants: umbilical versus umbilical cord lesions.

The omphalomesenteric duct is an embryologic connection between the digestive tract and yolk sac, which typically involutes by the time of birth. Failure of the obliteration process can result in omphalomesenteric duct remnants. We present two cases of children with such remnants, one with an umbilical polyp requiring traditional excisional therapy and one more unusual case of an umbilical cord cyst resolving spontaneously without intervention. The differential diagnosis, which includes urachal remnants and umbilical pyogenic granulomas, is discussed, along with management and special considerations that practioners should be aware of when treating pediatric umbilical and umbilical cord lesions.

Pathogenic implications of remnant vitelline structures in gastroschisis.

PURPOSE: The pathogenesis of gastroschisis is unknown. It may be helpful in understanding its pathogenesis to know the structural relationships among umbilical components including umbilical vessels, urachus, and vitelline structures, and thus, the authors investigated the remnants of vitelline structures in a series of cases of gastroschisis. METHODS: Medical records of 41 cases with gastroschisis treated in our institute from 1979 to 2009 were retrospectively reviewed. RESULTS: Paraumbilical bands, possible remnants of vitelline structures, were observed in 4 cases (9.8%). All 4 bands were attached to the skin edge of the abdominal defect without incorporation into the umbilical cord. The band ended at the mesentery in 3 cases and at the antimesenteric site of the ileum in the remaining case. Histologic findings showed fibrous tissues in all cases. One was possibly associated with the development of colonic atresia. Another was noticed after silo reduction when herniated bowels became strangulated by the band. The other 2 cases were uncomplicated. CONCLUSIONS: Our findings may support the recently proposed hypothesis that the developmental failure of the yolk sac and related vitelline structures to merge with or to be incorporated into the umbilical stalk might be associated with the pathogenesis of the abdominal wall defect in gastroschisis. Paraumbilical bands derived from vitelline structures may possibly cause intestinal ischemia prenatally or postnatally.

Patent vitellointestinal duct.

During the 3rd week of intrauterine life there is a communication between the intraembryonic gut and the yolk sac. As the development proceeds this communication narrows into a tube known as the vitellointestinal duct (VID). With the establishment of placental nutrition this duct usually becomes obliterated by the end of the 7th week of intrauterine life. In about 2% of humans this duct persists and gives rise to a group of anomalies of which Meckel's diverticulum is the commonest and complete patency of the duct is the rarest.1 We report a case of a 4-month-old infant who presented with umbilical polyp, discharge from the umbilicus, significant dehydration and failure to thrive because of complete patency of the VID.

Umbilical cyst containing ectopic gastric mucosa originating from an omphalomesenteric duct remnant.

An umbilical cyst originating from an omphalomesenteric duct remnant is extremely rare, and to the best of our knowledge, it has scarcely been reported in medical literature. We present ultrasonographic manifestations and computed tomographic findings of an umbilical cyst originating from an omphalomesenteric duct remnant in a 6-year-old girl. In cases of umbilical cyst, radiographic evaluation alone may not differentiate an omphalomesenteric duct remnant with heterotopic gastric mucosa from a urachal remnant, and surgical intervention is required.

Persistencia del conducto onfalomesentérico / Patent omphalomesenteric duct

La persistencia del conducto onfalomesentérico es una de las formas de presentación más raras de los restos embrionarios derivados de este conducto. Se presenta el caso de un recién nacido en el que se constata salida de contenido intestinal através del ombligo. Se confirma el diagnóstico de persistencia del conducto onfalomesentérico mediante fistulografía umbilical, procediéndose a su corrección quirúrgica (resección de conducto onfalomesentérico). Se describe la metodología diagnóstica, su tratamiento y se revisa la bibliografía sobre esta patología.

Hemorrhagic Meckel's diverticulum in an older woman diagnosed by repeated angiographies.

Meckel's diverticulum is a remnant of the vitelline duct and a congenital anomaly of the gastrointestinal tract. Hemorrhage from a Meckel's diverticulum is common in children but extremely rare in adults over 50 years of age. Very few cases have been reported to date and all prior cases were in men. Meckel's diverticulum is commonly overlooked as a possible cause of a lower gastrointestinal hemorrhage in adults. Here, we present the rare case of a 58-year-old woman with massive hemorrhage from a Meckel's diverticulum that was diagnosed by repeated emergency angiographies and treated with elective laparoscopic surgery.

Persistent omphalomesenteric duct causing small bowel obstruction in an adult.

An extremely rare case of persistent omphalomesenteric duct causing small bowel obstruction is presented. A 20-year-old female patient without medical history presented with colicky abdominal pain, vomiting, absence of passage of gas and feces, and abdominal distension of 24 h duration. Physical examination and blood tests were normal. Abdominal X-ray showed small bowel obstruction. Computed tomography of the abdomen demonstrated dilated small bowel and a band originating from the umbilicus and continuing between the small bowel loops; an omphalomesenteric duct remnant was suspected. In exploratory laparotomy, persistent omphalomesenteric duct causing small bowel obstruction was identified and resected. The patient had an uneventful recovery and was discharged on the 5(th) postoperative day. Although persistent omphalomesenteric duct is an extremely infrequent cause of small bowel obstruction in adult patients, it should be taken into consideration in patients without any previous surgical history.

Patent omphalomesenteric duct and exomphalos minor: a rare and interesting association.

A completely patent omphalomesenteric duct was seen in association with two cases of exomphalos minor. Both the patients also had limb anomalies, and one patient had an ileal duplication and colonic atresia. Both babies underwent emergency surgery to correct the anomalies, and one survived. There have been few reports of this combination of anomalies, and there is increasing appreciation that these lesions occur together more often than by chance.

A neonate with an intact congenital umbilical appendix: an alternative theory on the etiology of the appendico-umbilical fistula.

Neonatal umbilical anomalies usually represent remains of the vitelline duct or the allantois. We describe a case of an umbilical appendix in a neonate. The vermiform appendix was found to be positioned in the umbilical cord. In a brief literature review we found eight other reports concerning umbilical appendices. In this article we describe a possible embryological explanation for the development of an umbilical appendix, and discuss whether or not the appendiceal umbilical fistulae reported are congenital or iatrogenic. The possible association between an umbilical appendix and different forms of malpositioning and rotation of the gut is also discussed. Protrusion of the neonatal appendix into the umbilical cord represents a different entity of congenital anomalies. It is important to realize that, in the case of an unrecognized umbilical appendix, medical procedures (e.g., canulation or clamping of the umbilicus) may produce an iatrogenic appendico-umbilical fistula. Careful inspection and palpation of the umbilical cord prior to these procedures may prevent a fistula being created. Furthermore, because the possible association between umbilical appendices and different kinds of malpositioning of the gut is so far not wholly elucidated, we recommend further (radiological) investigation in each case of an umbilical appendix. Correct positioning of the bowel needs to be confirmed in order to rule out possible future complications.

Ultrasonographic demonstration of a type 1 omphalomesenteric duct remnant.

The vitelline or omphalomesenteric duct malformations constitute a rare group and are seen in only 2% of people. The malformations can take the form of a patent duct, a cyst, a fistula, or a sinus. They may or may not be symptomatic depending on the type of malformation. We present ultrasonographic features of a patent omphalomesenteric duct remnant in a 3-week-old boy who presented to our hospital with a non-healing umbilical lesion. Ultrasound can prove beneficial in the work-up of such cases.