MONOZYGOTIC TWINS WITH STRUCTURAL ANOMALIES. (CONGENITAL PYLORIC STENOSIS & OCCIPITAL MENINGOMYELOCELE)

Monozygotic twinning occurs in about 1 in 200 births & represents the most common aberration of morphogenesis noted in humans. Structural defects occur 2-3 times more commonly in live born monozygotic twins than dizygotic twins or singletons. Anomalies may be due to:
  • Uterine compression deformities from crowding - Hip dislocations
  • Vascular communications with Embolisation - Ileal atresia, Porencephaly, cutis Aplasia
  • Vascular communications with out Embolisation - acardiac twin
  • Unknown factors - conjoint twins, anencephaly, meningomyelocele

Here we have a pair of monozygotic twins born to a non-consanguineous couple aged 2 months, one child had an occipital meningomyelocele. Second child had a pyloric stenosis.

The first child underwent surgery for the meningomyelocele, in our hospital.


CONGENITAL GIANT MELANOCYTIC NAEVUS WITH STURGE-WEBER SYNDROME
This two month old child presented with refractory type of seizure since birth. Not associated with fever or ear discharge, child was born to a non-consanguineous couple with a normal uneventful birth history.

On examination, child had microcephaly and port-wine stain involving the Trigeminal area of face bilaterally.

Another bluish-black Giant nevus was seen covering the entire back, trunk and Lower limbs.

C.T. Scan of Brain showed well defined curvilinear calcification along the left Temporo parietal Gyri, with diffuse cerebral atrophy suggestive of Sturge-Weber syndrome. This child had an interesting association of Giant melanocytic nevus with Sturge-Weber syndrome.











CONGENITAL MELANOCYTIC NEVI are present in 1% of new born infants. Giant congenital pigmented nevi (> 20 cm in diamteter) occur in < 1 in 20,000 births. They occur most commonly on the posterior trunk, head and extremities. The incidence of malignant melaroma arising in a gaint nevus is 5 - 10%. Management of the nevus remains controversial. Periodic follow-up and serial photographs of the nevus may aid in detecting the changes.

STURGE-WEBER DISEASE is a constellation of symptoms and signs including a facial nevus (port-wine stain), seizures, hemiparesis, intracranial calcification and mental retardation. It occurs sporadically at 1 in 50,000 live births. Seizures develop in most patients during the 2nd - 7th months of life and are usually refractory to anticonvulsants and associated with slowly progressive hemiparesis. CT scan shows typical gyral calcifications with tram track appearance, associated with cortical atrophy and ipsilateral dilatation of lateral ventricle. Treatment is again controversial and may be medical or surgical.
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