Successful Expectant Management of the Anomalous Fetus with Sirenomelia in Twin Pregnancy: A Case Report and Literature Review

Tilahun, Temesgen; Desta, Dawit

doi:10.2147/imcrj.s300318

IMCRJ

2021

DOI: 10.2147/imcrj.s300318

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Successful Expectant Management of the Anomalous Fetus with Sirenomelia in Twin Pregnancy: A Case Report and Literature Review

Temesgen Tilahun¹,

Dawit Desta²

Abstract: Background Sirenomelia is a rare congenital defect. Its management during pregnancy and after delivery is becoming a controversial issue because of its complex nature and management outcome. The possibility of expectant management in the sirenomelia twin drove us to write this case report. Case Presentation We report a case of successful expectant management in twin sirenomelia which was diagnosed in the second trimester. Prenatal counseling of the couple by a multi-dis… Show more

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Six‐month survival of a monochorionic monoamniotic twin with sirenomelia

Hirata

Yamamura-Miyazaki

Kawaguchi

et al. 2022

Birth Defects Research

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Background Sirenomelia is a congenital malformation of the lower body characterized by a single midline lower limb and severe urogenital and gastrointestinal malformations. Sirenomelia is rare (estimated incidence of approximately 1/100,000) and usually lethal in the perinatal period. Case A 2,042 g Japanese male infant, one of monochorionic monoamniotic twins, was born at 34 weeks of gestation by elective caesarean section. Sirenomelia was prenatally diagnosed. Single midline lower limb, bilateral dysplastic kidneys, an omphalomesenteric fistula, colon atresia, imperforate anus, indiscernible genital structures, and myelomeningocele were detected at birth. The amniotic fluid volume was normal throughout the pregnancy course, which led to appropriate lung maturation of the twin with sirenomelia. Although renal replacement therapy was initiated soon after birth, stable peritoneal dialysis was difficult because of the limited intraperitoneal space, and the infant frequently developed peritonitis. He died of sudden cardiorespiratory arrest at 6 months of age. Postmortem examination showed bilateral dysplastic kidneys, agenesis of the ureters and urinary bladder, abnormal branching and agenesis of the distal colon, bilateral inguinal hernias, and small testes. Conclusion Infants with sirenomelia, even those with end‐stage kidney disease at birth, may survive if they have a stable cardiorespiratory status at birth and renal replacement therapy is appropriately initiated.

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Six‐month survival of a monochorionic monoamniotic twin with sirenomelia

Hirata

Yamamura-Miyazaki

Kawaguchi

et al. 2022

Birth Defects Research

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show abstract

Sirenomelia: A case report

Tamene

Molla²

2022

SAGE Open Medical Case Reports

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Sirenomelia also known as mermaid syndrome is a partial or complete fusion of lower extremities associated with visceral anomalies. It is a rare anomaly with a poor prognosis. Maternal age less than 20 years, maternal diabetes mellitus, and monozygotic twinning are some of the known risk factors. Diagnosis can be made via antenatal ultrasound or typical physical appearance after birth. A 2200 g baby was born from 18-year-old non-consanguineous mother via assisted breech delivery. The baby had fused lower limbs with 10 toes, absent external genitalia, and a single umbilical artery. It was small for gestational age. The baby was passed away after 30 min of birth. Young maternal age (18 years) was the identified risk factor for sirenomelia in this case. We recommend an early routine ultrasound anomaly scan in all pregnant women particularly for early detection and termination of pregnancy as the prognosis is poor. Avoiding teenager pregnancy and strict control of blood sugar in diabetic mothers can decrease this anomaly.

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Sirenomelia: Relato De Caso E Revisão Da Literatura

Hiar,

Neto

2023

jmrr

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Introdução: A sirenomelia é uma condição caracterizada pelo desenvolvimento anormal do corpo caudal de um feto, com fusão parcial ou completa da extremidade inferior, sendo também conhecida como Síndrome da Sereia. A anomalia é uma malformação grave multissistêmica, geralmente envolvendo os sistemas gastrointestinal, geniturinário, cardiovascular e musculoesquelético. A causa exata da sirenomelia é desconhecida, e acredita-se que fatores ambientais e genéticos possam desempenhar um papel no desenvolvimento da referida anomalia. O prognóstico da sirenomelia é ruim, com uma sobrevida média de menos de uma semana e mais da metade dos casos terminam em natimortos. Objetivo: Relatar um caso de sirenomelia atendido em nosso Serviço. Método: Trata-se do relato do caso de uma gestante atendida no Serviço de Ginecologia e Obstetrícia do Hospital Universitário São Francisco na Providência de Deus – HUSF, localizado na cidade de Bragança Paulista - SP. Relato do Caso: Paciente primigesta, 17 anos, encaminhada ao Serviço de Medicina Fetal após a realização de ultrassonografia morfológica durante a gravidez que demonstrou múltiplas malformações, incluindo agenesia renal e oligoâmnio severo. A criança nasceu prematura e com sirenomelia, falecendo quatro horas após o parto. Análises da placenta e do cordão umbilical revelaram anormalidades na implantação do feto, além de uma artéria umbilical única. Conclusão: A sirenomelia é uma anomalia congênita rara e fatal, caracterizada pela fusão das extremidades inferiores, frequentemente associada a malformações adicionais. Sua etiologia é pouco compreendida e os possíveis fatores de risco incluem diabetes materno, exposição a fármacos teratogênicos, suscetibilidade genética, infecções e idade materna. Com base no caso descrito, sugere-se que a realização de ultrassonografia é crucial para o diagnóstico precoce e tomada de decisão frente a casos de sirenomelia. No mais, a conscientização sobre a prevenção da gravidez na adolescência e o controle da glicemia em mães diabéticas são essenciais para reduzir a incidência da síndrome.

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Successful Expectant Management of the Anomalous Fetus with Sirenomelia in Twin Pregnancy: A Case Report and Literature Review

Cited by 4 publications

References 15 publications

Six‐month survival of a monochorionic monoamniotic twin with sirenomelia

Six‐month survival of a monochorionic monoamniotic twin with sirenomelia

Sirenomelia: A case report

Sirenomelia: Relato De Caso E Revisão Da Literatura

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