Sirenomelia with VACTERL association-a rare anomaly

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Sirenomelia: A Rare Presentation

We are presenting two cases of Sirenomelia (Mermaid Syndrome), which is an extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, imperforate anus, and renal agenesis or dysgenesis. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most o...

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Sirenomelia: A Rare Malformation

A twenty two year old primigravida, at thirty four weeks gestation delivered a 1.7 kg baby by emergency lower segment cesarean section. Cesarean section was done in view of severe intrauterine growth retardation and severe oligohydramnios. There was no history of diabetes mellitus or gestational diabetes in the mother, no history of any chronic medical illness, any drug intake or consanguinity ...

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VACTERL Association

VACTERL association [3] is a term applied to a specific group of abnormalities involving structures derived from the mesoderm [4]. Although the defects of this disorder are clearly linked, VACTERL is called an association rather than a syndrome because the exact genetic cause is unknown. ?VACTERL? is an acronym, each letter standing for one of the defects associated with the condition: V for ve...

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Neonate with VACTERL Association and a Branchial Arch Anomaly without Hydrocephalus

VACTERL (vertebral anomalies, anal atresia, cardiac defect, tracheoesophageal fistula, renal anomaly, limb anomalies) is an association of anomalies with a wide spectrum of phenotypic expression. While the majority of cases are sporadic, there is evidence of an inherited component in a small number of patients as well as the potential influence of nongenetic risk factors (maternal diabetes mell...

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Scrotal ectopia is a rare condition. Associated anomalies are common. We describe a neonate with ectopic scrotum with VACTERL association. This combination of anomalies is very rare.

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ژورنال

عنوان ژورنال: Pediatrics & Neonatology

سال: 2018

ISSN: 1875-9572

DOI: 10.1016/j.pedneo.2017.09.007