Hernia of the cord with patent omphalomesenteric duct and ileal prolapse in two preterm neonates: case series

@#Hernia of the cord (HOC) is a rare condition that results from failure of the viscera to return to the abdominal cavity by the 10th week of fetal development. It sometimes presents together with a patent omphalomesenteric duct (POMD), another rare condition, which occurs earlier in fetal development. A proportion of POMD cases may also have ileal prolapse (IP) through the POMD lumen. Neonates diagnosed with the combination of these rare clinical conditions require immediate surgical intervention to resect the POMD and non-viable bowel segments, reduce the hernia, and repair the umbilical defect. In this case series, we report two neonates diagnosed with HOC with POMD and IP. One patient who had complete IP was not fit for immediate surgery and died of respiratory distress and sepsis. The other patient had a successful surgical correction of the congenital defects, but succumbed to sepsis postoperatively. Treatment of patients with this combination of clinical conditions should focus mainly on both surgical correction, and infection control and management.

New clues to understand gastroschisis. Embryology, pathogenesis and epidemiology / Nuevas pistas para comprender la gastrosquisis. Embriología, patogénesis y epidemiología

Abstract gastroschisis is a congenital structural defect of the abdominal wall, most often to the right of the umbilicus, through which the abdominal viscera protrude. Its developmental, etiological and epidemiological aspects have been a hot topic of controversy for a long time. However, recent findings suggest the involving of genetic and chromosomal alterations and the existence of a stress-inducing pathogenetic pathway, in which risk factors such as demographic and environmental ones can converge. . We have conducted a review of the medical literature that gathers information on the embryonic development of the ventral body wall, the primitive intestine, and the ring-umbilical cord complex, as well as on the theories about its origin, pathogenesis and recent epidemiological evidence, for which we consulted bibliographic databases and standard search engines

Resumen La gastrosquisis es un defecto estructural congénito de la pared abdominal, localizado con mayor frecuencia a la derecha del ombligo, a través del cual sobresalen las vísceras abdominales. Durante mucho tiempo, sus aspectos evolutivos, etiológicos y epidemiológicos han sido un tema candente de controversia, aunque hallazgos recientes sugieren la participación de alteraciones genéticas, cromosómicas, y la existencia de una vía patogénica inductora de estrés, en la que factores de riesgo como los demográficos y ambientales pueden converger. Con el objetivo de ampliar la frontera del conocimiento sobre una malformación que ha mostrado una creciente prevalencia global, hemos efectuado una revisión que incluye información, del desarrollo embrionario de la pared corporal ventral, el intestino primitivo, el complejo anillo-cordón umbilical, y de las teorías acerca de su origen, patogénesis e información epidemiológica reciente.

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.

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.

Clinical analysis of residual vitelline duct in adults / 中华普通外科杂志

Objective To summarize the clinical characteristics of adult patients with residual yolk duct,and to explore the diagnosis and treatment strategy of residual vitelline duct in adults.Methods A retrospective analysis on 11 adult cases with residual vitelline duct in our hospital between June 2012 and May 2017 was carried out.Results 8 cases were males,3 cases were females,and median age was 50 years (18-57 y).2 cases were vitelline cyst,9 cases were Meckel diverticulum.2 cases were with ectopic tissue,3 cases with ulcer bleeding,1 case with secondary intra-abdominal hernia and intestinal obstruction,2 cases with secondary infection.The pathological diagnosis of Meckel diverticulum was consistent with preoperative diagnosis.There were no major postoperative complications.The patients were followed up from 6 months to 2 years.Conclusion Most of the residual vitelline duct in adults are Meckel diverticulum and vitelline duct cyst.Resection of residual vitelline duct is the main treatment method.

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.

Divertículo de Meckel: revisão de literatura / Meckel's diverticulum: a literature review

O divertículo de Meckel (DM) é considerado a anormalidade congênita mais comum do trato gastrintestinal. Resulta de falha na obliteração do ducto onfalomesentérico (ducto vitelino) e, geralmente, apresenta-se como divertículo curto, de base larga, localizado na borda antimesentérica do íleo a aproximadamente 90 cm da válvula ileocecal. É assintomático na maioria dos casos e diagnosticado acidentalmente em laparotomia/laparoscopia indicadas por outras causas. Manifesta-se em crianças pela hemorragia digestiva e nos adultos pelo desenvolvimento de processos abdominaisobstrutivos ou inflamatórios. Suas principais complicações são: hemorragia, intussuscepção, volvo, formação de enterólitos, inflamação, perfuração, obstrução e neoplasia. A confirmação diagnóstica é definida por exames de imagem como a ultrassonografia abdominal, tomografia computadorizada, cintilografia e angiografia. A conduta em pacientes assintomáticos é controversa, entretanto, em pacientes sintomáticos, a indicação cirúrgica é consenso. Sua abordagem requer a diverticulectomia simples ou a enterectomia segmentar com reconstrução pela anastomose primária término-terminal.

Meckel's diverticulum (MD) is considered the most common congenital abnormality of the gastrointestinal tract. It results from a failed obliteration of the omphalomesenteric duct (vitelline duct), and generally presents as short diverticulum of wide base, located in the antimesenteric border of the ileum, about 90cm from the ileocecal valve. It is asymptomatic in most cases and diagnosed accidentally by laparotomy/laparoscopy indicatedfor other causes. It manifests as gastrointestinal bleeding in children and in adults by developing abdominal obstructive or inflammatory processes. Its main complications are bleeding, intussusception, volvulus, enterolith formation, inflammation, perforation, obstruction, and neoplasia. Diagnostic confirmation is defined by imaging studies such as abdominal ultrasound, computed tomography, scintigraphy, and angiography. Clinical conduct in asymptomatic patients is controversial; however, in symptomatic patients,surgical indication is a consensus. The approach requires simple diverticulectomy or segmental bowel resection with primary reconstruction by end-to- end anastomosis.

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.

A Clinical Study of Vitelline Duct and Vessel Remnants

Of 72 cases with vitelline duct and vessel remnants, 45 (62.5%) had symptomatic lesions (mean age, 27.9 months) with male preponderance (4.6: 1). Among the 45 symptomatic lesions, there were 22 cases of Meckel's diverticulum, 6 cases of Meckel's diverticulum with fibrous band attached to the umbilicus, 6 cases of patent vitelline duct, 5 cases of vitelline artery remnant as fibrous band, 2 cases of umbilical sinus, 2 cases of umbilical polyp, and 2 cases of vitelline cyst. Twenty three cases (51%) presented with intestinal obstruction, 6 (13%) with rectal bleeding, 4 (9%) with perforated Meckel's diverticulum, 5 with intestinal fluid drainage through umbilicus, 5 with umbilical lesion, and 1 with abdominal mass. Intestinal obstruction due to fibrous band developed at infancy (average age, 4.6 months). About 82% of complicated Meckel's diverticulum (n=28) presented less than 4 years of age. Seventeen Meckel's diverticulums, 8 obliterated vitelline artery remnants, and 1 vitelline vein remnant as fibrous band were found incidentally at laparotomy.