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.

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.

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.

Prevalence, Morphology, Embryogenesis, and Diagnostic Utility of Umbilical Cord Vitelline Vascular Remnants.

The objective of this study is to better understand embryonic vitelline vascular remnants in the umbilical cord, to assess their prevalence, to categorize their morphology, and then finally to describe and assess inflammation arising from these structures. During routine placental sign out, the author noted the presence or absence of vitelline vessel remnants for 1 year; when present, he assessed their histologic patterns and noted whether there were neutrophils marginating from the remnants and into the adjacent Wharton's jelly and whether there was any other evidence of amniotic fluid infection in sections of placental disc, membranes, or cord. All cord sections with vitelline vessel remnants were immunostained for CD15 to document any infiltrates, to highlight patterns of infiltration, and to evaluate whether mild cases of umbilical phlebitis were associated with these lesions and were at risk of being missed. Vitelline vessel remnants were present in 4.2% of placentas examined. There were 5 vitelline vessel remnant histologic patterns identified providing insight into the vitelline vessel circulation. Funisitis, primarily neutrophilic, arising from vitelline vessel remnants was present in 70.3% of the 37 cords with vitelline vessel remnants. The presence of vitelline vessel remnant funisitis documents continued active circulation in these vascular structures, and vitelline vessel remnant funisitis was associated with the presence of other placental histological evidence of amniotic fluid infection in 53.8% of cases. The author also reviews normal embryology and the pathology of vitelline vessel remnants.

Changes in the Prevalence of Embryologic Remnants in Umbilical Cord With Gestational Age.

The aim of this study was to examine the prevalence of embryologic remnants in umbilical cords of different gestational ages. Sections from 392 umbilical cords were examined using light microscopy. Of these, 52% contained at least 1 remnant, most commonly of the allantoic duct type. Although there was a significant decrease in vitelline duct remnants over increasing gestational age, from 11% at weeks 11-25 to 1.6% at weeks 36-42 (P = .009; χ2 test), the allantoic duct remnants remained constant in prevalence irrespective of gestational age.

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.

Male predominance in Meckel's diverticulum: A hyperacidity hypotheses.

The symptomatic presentation of Meckel's diverticulum (MD) depends on a person's age, sex, and presence or absence of ectopic gastric tissue. There are no differences in the prevalence of asymptomatic MD between males and females; however, symptomatic MD has a distinct male predominance with a male-to-female ratio ranging from 2:1 to 5:1 in children. Furthermore, if the ectopic tissue contains gastric mucosa, MD has a greater chance of becoming symptomatic. Studies have shown that acid secretion is more likely to occur in male infants compared to female infants. In adults, men are known to have a higher level of acid production compared to women. Peptic ulcers (PU) are more common in males due to high acid secretion, and gastric tissues are affected by gastrin secretion in both conditions. MD is typically accompanied by ectopic gastric tissue, and could therefore be affected by gastrin and acid secretion in a similar manner to PU. Some of the major complications of MD are diverticulitis, ulcers, and bleeding from adjacent ectopic gastric tissue, and such complications resemble PU. PU also have male to female ratios ranging from 2:1 to 5:1, which is again similar to MD. Since the secretion of both gastrin and acid decrease with age, symptomatic presentation of MD also declines with age. Therefore, we hypothesize that higher gastrin and acid levels in males affect the ectopic gastric mucosa and lead to an increase in MD symptoms, which result an increased incidence of MD in males.

Heterotopic pancreas in the omphalomesenteric duct remnant in a 9-month-old girl: a case report and literature review.

BACKGROUND: Heterotopic pancreas most commonly occurs in the upper gastrointestinal tract of adults, usually as an incidental finding. It seldom occurs at the umbilicus, and even rarely in the pediatric age group. CASE PRESENTATION: Here we present a case of heterotopic pancreatic tissue in the omphalomesenteric duct remnant of a 9-month-old baby girl. She presented with redness at the base of the umbilicus associated with occasional mild wetness. A urachal fistula was suspected by ultrasound. Histology from subsequent resection revealed fibrous tissue with heterotopic pancreatic tissue and accompanying small bowel mucosa. The patient's umbilical redness resolved after the surgery. CONCLUSIONS: Upon literature search, we found only 17 other cases of heterotopic pancreas reported in the umbilicus. They described a high male to female ratio, frequent association with omphalomesenteric duct remnant and presentation of umbilical discharge. The Heinrich system is frequently used to classify heterotopic pancreas into 3 types, based on the presence of acini, islets and ducts. Several mechanisms have been proposed on the pathogenesis of heterotopic pancreas, including misplacement, metaplasia and totipotent cell theories. Heterotopic pancreas can manifest clinically with diseases of the pancreas, including malignant transformation, reported as high as 12.7% in a series. Awareness of this finding in the biopsy aids the suitable treatment decisions for the patient.

Internal herniation due to an omphalomesenteric duct cyst in a 69-year-old man.

Previous abdominal surgery is the most common cause of mechanical small bowel obstruction. However, in patients with no history of abdominal surgery, the diagnosis and treatment of mechanical small bowel obstruction is difficult. A persistent omphalomesenteric duct remnant is a rare finding that typically presents in the pediatric population and is extremely rare in patients aged > 60 years. In the present report, we describe the case of an omphalomesenteric duct cyst causing small bowel obstruction in a 69-year-old man with no history of a surgical procedure.

Fetal MRI as a complementary technique after prenatal diagnosis of persistent vitelline artery in an otherwise normal fetus.

Prenatal ultrasound is the standard for the diagnosis of fetal anomalies. However, fetal MRI has emerged as a valuable diagnosis tool to complete the study of fetal malformations. Type II single umbilical artery results from the absence of both umbilical arteries and persistence of the vitelline artery. It has been described only in fetuses with sirenomelia or caudal regression syndrome. We report a favorable outcome in a normal fetus in which prenatal ultrasound and MRI showed a single umbilical artery arising from the aorta. The etiology of such a finding and its possible consequences are discussed.

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.

Portal hypertensive cardiovascular pathology: the rescue of ancestral survival mechanisms?

The portal system derives from the vitelline system, which is an extra-embryonic venous system. It could be suggested that this extraembryonic origin determines some of the characteristics attributed to portal hypertension, both compensated, i.e. prehepatic, and decompensated, i.e. fibrotic or cirrhotic. The experimental models most frequently used for studying both types of portal hypertension are portal vein ligation and common bile duct ligation in rats, respectively. We propose that in partial portal vein ligated rats, a low-grade inflammatory response, formed by the successive expression of three overlapping phenotypes - ischemia-reperfusion, vitellogenic-like and remodeling or gastrulation-like - is produced. The names of these inflammatory phenotypes developed in compensated portal hypertension are based on some metabolic similarities that can be established with the abovementioned phases of embryonic development. In bile-duct ligated rats, decompensation related to hepatic insufficiency would induce a high-grade inflammatory response. In this experimental model, the splanchnic interstitium, the mesenteric lymph and the peritoneal mesothelium seem to create an inflammatory axis that produces ascites. The functional comparison between the ascitic and the amniotic fluids would imply that, in the decompensated portal hypertensive syndrome, the abdominal mesothelium acquires properties of the amniotic membranes or amnion. In conclusion, the hypothetical comparison between the inflammatory portal hypertensive evolutive types and the evolutive phases of embryonic development could allow for translational research.

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.

Obliterated, fibrous omphalo-mesenteric duct in an adult without Meckel's diverticulum or vitelline cyst.

Vitello-intestinal [omphalo-mesenteric duct (OMD)] connects the developing mid-gut to the primitive yolk sac, provides nutrition to the embryo and remains patent and connected to the intestines until the fifth to ninth week of gestational period. Varied remnants of the vitello-intestinal duct have been reported. The present case-report describes a completely obliterated fibrous remnant of the duct. The remnant presented as a thick cord extending from the umbilicus towards the terminal part of the ileum and beyond. The terminal part of the cord showed a few ramifications that ended in the mesentery. This embryological entity was not found to be associated with any other anomaly usually related to non-regression of the vitello-intestinal duct. Though very rare, the occurrence of such innocuous band of fibrous cord across the abdominal cavity may cause entanglement of intestinal loops around it. Possibility of such a situation should be suspected in an acute abdominal condition. The structure reported in this study might not be detected by investigations used to uncover common anomalies of patent vitello-intestinal ducts.

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.

Atypical presentation of an omphalomesenteric duct cyst.

We describe a clinical case of an omphalomesenteric duct (OMD) cyst that presented as an umbilical nodule with a late and atypical clinical presentation. This report illustrates the possibility of OMD cyst resembling a keloid or dermatofibroma.

Escape of the yolk sac: a hypothesis to explain the embryogenesis of gastroschisis.

Gastroschisis is a significant birth defect that in many countries has shown an increased prevalence in recent decades, and the change has affected primarily younger mothers. Despite numerous epidemiological studies no other consistent associated risk factor has been identified. In this paper we review the five main theories related to the pathogenesis of this malformation and outline the reasons why we think none fully explains the embryogenesis of gastroschisis. We briefly present some clinical observations we have made that we consider germane to the pathogenesis and outline a hypothesis that we think can account for the origins of this malformation. Our proposal is that the determining defect in gastroschisis is failure of the yolk sac and related vitelline structures to be incorporated into the umbilical stalk. Otherwise, ventral closure of the lateral abdominal walls occurs normally, thus orphaning the vitelline duct and yolk sac outside both the main body stalk and the abdominal wall. Thus, in addition to the umbilicus, the abdominal wall has a separate perforation through which the midpoint of the gut is attached to the exteriorized vitelline structures. This connection through the ventral wall prevents normal egress of the gut into the umbilical cord during the second month of development and acts as the egress point for the gut resulting in gastroschisis.