Sirenomelia: A Case Report

Kattel, Pramod

doi:10.31729/jnma.3884

Cited by 3 publications

(1 citation statement)

References 4 publications

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“…Males are affected more as compared to female in the ratio of 2.7:1. [ 3 ] The affected infant has features like mermaid: human-like head and trunk with fish like tail. [ 4 ] These are associated with single umbilical artery, severe deformities of the urogenital and lower gastrointestinal system, renal agenesis, and caudal regression syndrome.…”

Section: Introductionmentioning

confidence: 99%

Sirenomelia- A rare congenital anomaly: Case report

Thombare,

Dixit,

Chavan

et al. 2023

Journal of Education and Health Promotion

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Sirenomelia is an extremely uncommon congenital deformity in which neonate has fused lower limbs associated with various visceral malformations including urogenital and gastrointestinal tract, pulmonary hypoplasia, and potter's facies. The prevalence is 0.8 to 1 per 100,000 newborns. About 300 cases were recorded till date. We describe a case of 31-year G4P1L1A2 with previous lower segment caesarean section with gestational age of 22 weeks 5 days with anomaly scan suggestive of single live intrauterine pregnancy of 21 weeks 5 days with bilateral renal agenesis with placenta previa and no demonstrable amniotic fluid pocket seen. Her pregnancy was uneventful without any medical disorder, drug history, and no congenital anomaly in the family. Termination of pregnancy done by emergency hysterotomy and delivered anomalous foetus with fused lower limb with absent toes, low set ears, absent left ear ostium, imperforate anus, and absent external genitalia. Sirenomelia is fatal congenital anomaly with unclear etiology. Early antenatal diagnosis and termination of pregnancy is the treatment.

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Section: Introductionmentioning

confidence: 99%

Sirenomelia- A rare congenital anomaly: Case report

Thombare,

Dixit,

Chavan

et al. 2023

Journal of Education and Health Promotion

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Sirenomelia—Challenges and Treatment Approach in a Rare Case

Sosińska,

Gołębiewski,

Czauderna

2024

Birth Defects Research

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IntroductionSirenomelia is a very rare congenital structural anomaly characterized by abnormal development of the caudal region of the body with varying degrees of fusion of lower limbs. Mostly, the condition is lethal for the baby. Most babies do not survive even after surgery. Fifty percent of cases are seen as stillbirths, and it is much more common in identical twins.Case ReportWe present a case of a boy born in 38th week of gestation (hebdomas graviditatis—HBD) with diagnosed sirenomelia, microcephaly, bilateral renal agenesis, duodenal atresia, imperforate anus, and agenesis of external genitalia. We provide a brief review of the literature and discussion concerning similar cases, determinants, pathogenesis and suspected genetic factors.ConclusionsDue to accompanying malformations, there is often no treatment for sirenomelia. Avoidance of risk factors, early diagnosis, multidisciplinary approach and psychological preparation of parents to help them cope emotionally and mentally with the challenges, seem to be the key factors of management. It is very important to diagnose this condition by ultrasonography prenatally so that termination of pregnancy can be carried out.

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Probability of Breech Presentation in Fetuses with Sirenomelia at Birth

Maričić,

Podgorac,

Kesić

et al. 2024

Clin. Exp. Obstet. Gynecol.

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Background: The 50% maximum probability of breech presentation (BP) implies that the same number of fetuses/newborns with the same characteristics take either BP or cephalic presentation (CP) with equal probability. Sirenomelia offers the possibility to investigate the incidence of BP in the simultaneous presence of several medical entities, each associated with an increased incidence of BP (Potter anomaly, lower limb fusion, prematurity, additional medical entities). The aim of the study is to investigate the probability of BP at delivery in sirenomelic singleton gestations to confirm the rule that the maximum probability for BP is 50%. Methods: A systematic retrospective literature search using the keywords: sirenomelia, sympus apus, sympus monopus, and sympus dipus, in the SCOPUS database. Inclusion criteria: presentation of singleton fetuses at delivery in the second and third trimester of pregnancy. Comparison with chi-square test (level of significance p < 0.5) of overall incidence of BP and CP as well as depending on the type of sirenomelia, prematurity, presence of additional medical entities associated with increased incidence of BP. Results: In total, 67 articles were identified, and 83 cases were found. The number of cases in BP vs. CP with a statistical difference to expected 50%:50% distribution of presentations: All cases—BP44 CP39 p = 0.583; Premature birth—BP25 CP20 p = 0.456; Term birth—BP17 CP18 p = 0.866; Sympus apus—BP15 CP14 p = 0.853; Sympus monopus—BP6 CP9 p = 0.439; Sympus dipus—BP21 CP14 p = 0.237; With additional entities—BP16 CP10 p = 0.239; Without additional entities—BP23 CP26 p = 0.668. Also, there were no statistically significant differences in the distribution of presentations between premature and term birth (p = 0.589), three types of sirenomelia (p = 0.601), with and without additional entities (p = 0.228). Conclusions: This study supports the rule that the maximum probability for BP in any medical entity, including sirenomelia, is 50%. The outcome of the BP should be evaluated with the case-control matching procedure: subtract the number of fetuses/newborns from the CP group that is identical to the number of fetuses/newborns in the BP group, with identical characteristics, and add this group to the BP group before comparing them to the rest of the CP fetuses/newborns.

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Sirenomelia: A Case Report

Cited by 3 publications

References 4 publications

Sirenomelia- A rare congenital anomaly: Case report

Sirenomelia- A rare congenital anomaly: Case report

Sirenomelia—Challenges and Treatment Approach in a Rare Case

Probability of Breech Presentation in Fetuses with Sirenomelia at Birth

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