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)

Persistencia simultánea del conducto uraco y onfalomesentérico en un paciente adulto, reporte de caso / Simultaneous persistence of the urachus and vitelline ducts in an adult patient, case report / Persistência simultânea de úraco e do ducto onfalomesentérico em um paciente adulto: relato de caso

Introducción. La patología umbilical es un tema frecuente en el ámbito pediátrico, la presentación clínica de una fístula umbilical hace pensar en dos patologías mencionadas en la literatura de manera independiente; la primera es la persistencia del conducto onfalomesentérico y la segunda es la persistencia del remanente del uraco producto del fracaso en el cierre de las estructuras embrionarias. Su presencia en adultos es infrecuente y no existen datos estadísticos acerca de su presentación conjunta en población pediátrica o adulta, solo algunos pocos reportes de caso. El diagnóstico se basa principalmente en la sospecha clínica, depende en gran manera del examen físico al evidenciar secreción a través del ombligo al realizar esfuerzos o maniobras de Valsalva. Objetivo. Mostrar un caso infrecuente de la presentación simultánea del conducto de uraco y onfalomesentérico en un paciente adulto. Reporte de caso. Paciente femenina de 24 años de edad con antecedentes de infecciones urinarias y celulitis periumbilicales a repetición. Se sospecha un conducto persistente onfalomesentérico por lo que es sometida a un procedimiento quirúrgico en el que se encontró incidentalmente la persistencia simultánea del conducto onfalomesentérico y persistencia del uraco. Discusión. La persistencia del conducto onfalomesentérico o la persistencia del uraco de forma individual es poco frecuente en adultos, y es aún más raro la persistencia simultánea de ambos conductos; la presencia simultánea de ambos conductos es reportada principalmente en menores de dos años. Conclusiones. La persistencia de estos conductos es rara en adultos y representa un reto diagnóstico para el clínico. Cómo citar: Escudero-Sepúlveda AF, Cala-Duran JC, Belén Jurado MB, Pinasco-Gómez R, Tomasone SE, Roccuzzo C, Domínguez-Alvarado GA. Persistencia simultánea del conducto uraco y onfalomesentérico en un paciente adulto, reporte de caso. MedUNAB. 2020;23(2): 288-293. doi: 10.29375/01237047.3826.

Introduction. Umbilical pathology is a common topic in the pediatric sphere. The clinical presentation of an umbilical fistula leads to the consideration of two pathologies independently reported in literature. The first is a persistent vitelline duct and the second is a persistent urachal remnant as a result of the embryonic structures' failure to close. They are uncommon in adults and there are no statistical data about their presentation together in the pediatric or adult population, only very few case reports. The diagnosis is mainly based on clinical suspicion. It largely depends on a physical examination noting secretion through the navel when straining or performing Valsalva maneuvers. Objective. Show an uncommon case of the simultaneous presentation of the urachus and vitelline ducts in an adult patient. Case report. Female patient aged 24 years with a background of repeated urinary tract infections and periumbilical cellulitis. A persistent vitelline duct is suspected. Therefore, the patient is subject to a surgical procedure in which the simultaneous persistence of the vitelline duct and the urachus was found incidentally. Discussion. The persistence of the vitelline duct or the persistence of the urachus individually is uncommon in adults, and the simultaneous persistence of both ducts is even rarer. The simultaneous presence of both ducts is reported mainly in infants aged under two years. Conclusions. The persistence of these ducts is rare in adults and poses a diagnostic challenge for clinicians. Cómo citar: Escudero-Sepúlveda AF, Cala-Duran JC, Belén Jurado MB, Pinasco-Gómez R, Tomasone SE, Roccuzzo C, Domínguez-Alvarado GA. Persistencia simultánea del conducto uraco y onfalomesentérico en un paciente adulto, reporte de caso. MedUNAB. 2020;23(2): 288-293. doi: 10.29375/01237047.3826.

Introdução. A patologia umbilical é um tópico frequente no cenário pediátrico; a apresentação clínica de uma fístula umbilical faz pensar em duas patologias mencionadas na literatura de forma independente; a primeira é a persistência do ducto onfalomesentérico e a segunda é a persistência do úraco como resultado da falha no fechamento das estruturas embrionárias. É pouco frequente sua presença em adultos e não há dados estatísticos sobre sua apresentação conjunta em população pediátrica nem adulta, apenas alguns poucos relatos de caso. O diagnóstico baseia-se principalmente na suspeita clínica, dependendo em grande parte do exame físico ao evidenciar uma secreção pelo umbigo quando realizar esforço ou manobra de Valsalva. Objetivo. Mostrar um caso infrequente de apresentação simultânea do úraco e ducto onfalomesentérico em um paciente adulto. Relato de caso. Paciente do sexo feminino, 24 anos, com histórico de infecções urinárias e celulite periumbilical recorrentes. Suspeita-se de um ducto onfalomesentérico persistente, portanto ela é submetida a um procedimento cirúrgico no qual encontrou-se a persistência do ducto onfalomesentérico e a persistência de úraco simultaneamente. Discussão. A persistência do ducto onfalomesentérico e a persistência de úraco individualmente é rara em adultos, e a persistência simultânea de ambos os ductos é ainda mais rara; esta presença simultânea é relatada principalmente em crianças menores de dois anos de idade. Conclusão. A persistência desses ductos é rara em adultos e representa um desafio diagnóstico para o profissional de saúde clínico. Cómo citar: Escudero-Sepúlveda AF, Cala-Duran JC, Belén Jurado MB, Pinasco-Gómez R, Tomasone SE, Roccuzzo C, Domínguez-Alvarado GA. Persistencia simultánea del conducto uraco y onfalomesentérico en un paciente adulto, reporte de caso. MedUNAB. 2020;23(2): 288-293. doi: 10.29375/01237047.3826.

A Case of a Patent Omphalomesenteric Duct Presenting with Meconium Discharge from the Umbilicus

The omphalomesenteric duct is a link between the primitive midgut and the yolk sac. Normally, the duct obliterates around 6 weeks of gestation, yet varying degrees of incomplete obliteration can take place in 1%–4% of infants. This study described the case of a newborn with a patent omphalomesenteric duct remnant fistula identified at birth with meconium in the umbilical cord. At birth, the infant presented meconium staining and meconium discharged within the umbilical cord. Physical examination and other examinations showed no other specific findings. The omphalomesenteric duct fistula was confirmed through the imaging study (abdominal ultrasonography, gastrografin enema). A surgery was carried out where the remnant was resected. The patient did well and was discharged soon after without complication.

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.

Diverticulo de meckel: reporte de casos / Meckel's diverticulum. Case reports

El divertículo de Meckel es el defecto congénito más frecuente del tracto gastrointestinal. Se trata de un remanente del conducto onfalome- sentérico, una estructura que conecta el saco vitelino primitivo al intestino medio durante el desarrollo del feto. El conducto onfalomesenté- rico se oblitera entre la quinta y séptima sema- nas de gestación. Generalmente se encuentra localizado en el último metro de intestino delga- do en el borde antimesentérico. Puede ser asintomático o manifestarse como un cuadro de abdomen agudo. Las complicaciones en un escaso número de pacientes pueden ser: infección, sangrado digestivo, obstrucción y raramente transformación maligna. A continuación, se presentan 4 casos de pacien- tes pediátricos con diagnóstico postoperatorio de divertículo de Meckel del Instituto Hondure- ño de Seguridad Social de San Pedro Sula, durante el período 2012-2014. El presente artículo se realizó con la nalidad de brindar una lección clínica a los colegas y permitir ampliar sus conocimientos sobre esta entidad...(AU)

Omphalocele with Double Prolapse of Ileum through Patent Vitellointestinal Duct: A Rare Presentation / 소아외과

Although Meckel's diverticulum is the most common vitellointestinal duct (VID) anomaly, patent vitellointestinal duct (PVID) is the most common symptomatic embryological defect. Patient may present with the anomaly itself or due to complications like intestinal obstruction secondary to volvulus, intussusception or adhesions. Prolapse occurs if the diverticulum is wide-mouthed enough to allow bowel to come out or due to increased intra-abdominal pressure like cry or cough. Bowel prolapse through PVID is rare and double prolapse of proximal as well as distal loop in a newborn is extremely rare. Omphalocele with prolapsing bowel through PVID as found in our index case is even rarer in literature. The pediatric surgeon should be familiar with these varied manifestations in the newborn because the prolapsed bowel can progress to gangrene and complications if not identified and operated upon early.

Persistent Vitellointestinal Duct Causing Small Bowel Obstruction - A Rare Entity.

Vitellointestinal {omphalo-mesenteric (OMD)} duct 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 vitellointestinal 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 ileum with a Meckel’s diverticulum. Herein, we report a case of 20 yr old male who presented with the features of small bowel obstruction due to completely obliterated fibrous remnant of the duct which is a rare entity. Although persistent omphalomesentric 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.

Heterotopic Pancreas in Omphalomesenteric Duct Remnant Results in Persistent Umbilical Discharge

No abstract available.

Omphalomesenteric duct cyst in an omphalocele: a rare association

Omphalomesenteric duct [OMD] remnants and omphalocele are not infrequently seen in paediatric patients. In most of the cases, OMD remnant in an omphalocele is a Meckel's diverticulum; however rarely there may be other lesions. A one-day old male baby underwent surgery for omphalocele. At exploration a 10 x 12 cm cyst containing gut contents was found as the content of the omphalocele, with proximal and distal ileal loops running in continuity with it. Resection of the cyst with end to end primary gut anastomosis was done. Baby also had complex associated cardiac anomalies and died few days after surgery due to sepsis

Adenocarcinoma arising from Meckel's diverticulum in the ileum with malrotation of the midgut / 대한외과학회지

Meckel's diverticulum (MD) is a true congenital diverticulum that is remnant by incomplete obliteration of the omphalomesenteric duct. It is the most common congenital anomaly of the gastrointestinal tract, with an estimated prevalence of 2% (0.3% to 3% in autopsy studies). About 90% of MD occurs within 100 cm of the ileocecal valve. A primary malignant tumor arising within an MD is extremely uncommon. Malignancies are reported to account for only 0.5% to 3.2% of the complications. Carcinoids are the most common malignant tumors occurring in MD. Adenocarcinomas are extremely uncommon and very poor prognosis has been reported. We report a case of radiographically diagnosed chronic inflammatory mass caused by adenocarcinoma arising from MD in the ileum with malrotation of the midgut incidentally discovered at exploration.

Umbilical Polyp / 대한피부과학회지

An umbilical polyp is a rare congenital lesion and it represents one of the developmental anomalies of omphalomesenteric duct remnants. Clinically, an umbilical polyp presents as a red, firm and round tumor with mucoid and sometimes bloody secretions. Umbilical polyps can be mistaken clinically for other umbilical disorders such as umbilical granulomas and granuloma pyogenicum. Hisopathologically, the umbilical polyp is usually composed of small intestinal mucosa or ectopic gastrointestinal mucosa. Herein, we report on two cases of umbilical polyps:one umbilical polyp was composed of small intestinal mucosa and the other consisted of gastric mucosa.

Obstrucción intestinal por remanente onfalomesentérico obliterado en paciente adulto: reporte de un caso / Bowel obstruction caused by an omphalomesenteric duct remnant: report of one case

Omphalomesenteric duct remnant is one of the least common congenital abnormalities due to vitelline sac persistence. Among the five types of this abnormalities, omphalomesenteric band or obliterated omphalomesenteric duct remnant is the least common. We report a 64 years old male presenting with vomiting, abdominal pain and absence of stools. The patient was operated with the diagnosis of intestinal obstruction and a persistent omphalomesenteric duct that caused the bowel obstruction was found. The patient had an uneventful postoperative period as is discharged 24 hours later.

El remanente del conducto onfalomesentérico es una de las anomalías congénitas más raras asociadas con la permanencia del saco vitelino. Dentro de los cinco subtipos que se describen en la literatura, la banda onfalomesentérica o conducto onfalomesentérico obliterado es el de menor frecuencia. Presentamos un caso muy infrecuente de persistencia del conducto onfalomesentérico, que ocasionó una obstrucción intestinal en un paciente adulto y que fue resuelto de forma quirúrgica.

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.

Congenital umbilical appendix

We report a case of a 3 years old female with umbilical appendix presenting with a fistula. Initial examination, which was done by a midwife, missed a congenital appendix protruding through the umbilicus. Ligation of this cord resulted in fistula formation. The patient was successfully managed by doing an appendectomy. Congenital umbilical anomalies are rare, only handful of cases have been reported so far. Most umbilical neonatal anomalies described in literature are derived from the vitelline duct. In this article we describe the clinical presentation and management of an umbilicus appendix presenting with a fistula following umbilical cord ligation. We also review the embryological origin, and aetiological facors

[Ileal prolapse through patent omphalomesenteric duct. A review of six cases]

Meal prolapse through the umbilicus is a severe complication of patent omphalomesenteric duct which exposes to the risk of intestinal necrosis in case of diagnostic or therapeutic delay. This is a retrospective study of six cases of ileal prolapse complicating a patent omphalomesenteric duct observed between 1986 and 2004 in the pediatric surgery department of Monastir. The mean age of the six patients was 67 days. The diagnosis was performed on the observation of an out-f low of intestinal liquid or stools through the umbilicus associated to an ileal prolapse. Five patients were treated by intestinal resection and anastomosis and the sixth had a wedge-shaped resection of the omphalomesenteric duct. One patient died from a severe sepsis. The evolution was favourable for the other patients. The ileal prolapse complicates 50 to 80 per cent of the umbilical enteric fistula. It exposes to the risk of occlusion and intestinal necrosis. So, the treatment must be urgent and consists on the resection of omphalomesenteric duct with ileal anastomosis.This treatment can be done by classic surgery or, more recently, by coelioscopy. The mortality can reach 33 per cent in the event of diagnostic or therapeutic delay. The ileal prolapse is a rare form of the patent omphalomesenteric duct which must be diagnosed and treated quickly to ovoid complications

Persistencia de Conducto Onfalomesentérico como causa de Obstruccion Intestinal en el Adulto. Reporte de un caso en el Hospital Nacional Hipolito Unanue / Persistence of Onfalomesentérico Conduct as a cause of intestinal obstruction in the Adult. A case report in the Hipolito Unanue National Hospital

La persistencia del conducto onfalomesentérico ha sido reportada en diversas publicaciones pediátricas ya sea a través de la aparición de un divertículo de Meckel que es la forma más común, o ya sea por la aparición de segmentos con permeabilidad parcial o total el mismo. Se han presentado casos esporádicos donde esta anomalía ha originado episodios de obstrucción intestinal en lactantes y niños, especialmente bajo la forma de unabanda fibrosa remanente. La presentación en el adulto, sin embargo, es extremadamente infrecuente. El caso presentado en este reporte muestra hallazgos compatibles con un conducto onfalomesentérico con permeabilidad parcial, que originó un cuadro de obstrucción intestinal en un adulto joven.

The persistence of the onphalomesenteric duct has been reported in several pediatricpublications either through the appearance of Meckel diverticulum that are commonest, orby the appearance of segments with partial or total permeability of itself. Sporadic caseshave appeared where this anomaly has originated episodes of intestinal obstruction ininfants and children specially under the form of a fibrous band. However, adult presentationis extremely infrecuent. The case presented in this report shows compatible findings witha onphalomesenteric conduit with partial permeability, that I originate an intestinal pictureof obstruction in a young adult.

Omphalomesenteric duct cyst as a content of omphalocele.

A rare occurrence of an omphalomesenteric duct cyst in an exomphalos minor sac is reported herein. The noteworthy points in this case were an unusual presentation of the rarest variety of umbilical cord cyst. The tapering intra-abdominal end of the cyst was found to be attached to the ileal mesentry, thereby simulating a herniating mesenteric cyst till the histolopathological report resolved the issue.

Spontaneous regression of patent omphalo-mesenteric duct.

We report a one-month-old male child with a patent omphalo-mesenteric duct that regressed spontaneously in the neonatal period and resulted in a Meckel's diverticulum.

Clinical Analysis of Vitelline Duct Anomalies in Children

A vitelline duct (VD) anomaly is a relatively common congenital abnormality of the umbilical area. The anomalies include patent vitelline duct (PVD), cyst, fistula or sinus. The incidence is approximately 2% of the populations, but development of symptoms is rare. Recently, we experienced two cases; PVD accompanied by a smallomphalocele and intestinal volvulus due to mesenteric band between Meckel's diverticulum and the mesentery. Thereafter,we evaluated the data of vitelline duct anomalies for 27 years. From 1980 to 2006, 18 cases of VD anomalies were reviewed based on the hospital records retrospectively. There were 15 boys and 3 girls and age ranged from 2 days to 15 years. Among the 18 cases, 15 cases were symptomatic and consisted of Meckel's diverticulum (10 cases), PVD (4 cases) and umbilical polyp (1 case). Three asymptomatic cases of Meckel's diverticulum were found incidentally were and were observed without resection. Ten cases of Meckel's diverticulum were presented with intestinal bleedings (4 cases), intestinal obstructions (5 cases) and perforation (1 case). Wedge resections and segmental resections of ileum were performed in 8 patients and 2 patients, respectively. Postoperative complications were adhesive ileus (1 case) and wound seroma (1 case). Small omphaloceles were accompanied in two of 4 PVD patients. There was 1 small omphalocele case which was accompanied by a prolapse of ileum. In summary, VD anomalies were more common in male and more than half of them were found in patients less than 1 year of age. PVD was diagnosed most frequently in neonates. Meckel's diverticulum presented with intestinal obstruction more frequently than bleeding.

Persistencia del conducto onfalomesentérico / Persistence of the conduit onfalomesentérico

Se presenta el caso de un recién nacido, cuyo nacimiento ocurrió en el Hospital ®Iván Portuondo¼, en San Antonio de los Baños y en quien, inmediatamente después del nacimiento, se constata salida de contenido fecal a través de la zona umbilical. Se realiza el estudio contrastado con lipiodol y se comprueba el diagnóstico clínico y radiológico en la intervención quirúrgica. Se revisa la bibliografía, se muestran las fotografías al respecto y se expone el caso