MIDAS is an acronym for microphthalmia, dermal aplasia,
and sclerocornea. This genetic disorder is characterized by irregular linear
skin erythema of head and neck, microphthalmia, corneal opacities,
diaphragmatic hernia, respiratory distress, and absent cerebral septum
pellucidum leading to seizures.
Microphthalmia-Linear Skin Defects Syndrome; MLS.
Approximately 20 cases
described. Transmitted as an X-linked dominant trait; lethal in males.
Mapped to the short arm of chromosome X (Xp22.31). It is considered
as an X-linked phenotype distinct from Goltz syndrome.
Midfacial hypoplasia and linear erythematous skin
lesions, narrow and deformed ears, unilateral or bilateral microphthalmia,
anterior chamber anomalies (corneal opacity, sclerocornea, orbital cysts,
unplanted palpebral fissures and chorioretinal abnormalities). Focal dermal
hypoplasia or aplasia affecting usually the face, scalp, and neck. Looks
like linear burns or cuts at birth but heals without scar, or the skin can
remain pigmented and depressed at those places. Mild short stature.
Diaphragmatic hernia and rare occurrence of cardiac defects (atrial septal
defect, ventricular septal defect, one
case of cardiomyopathy, conduction defect, supraventricular tachycardia, and
ventricular fibrillation). In one case, the autopsy showed persistent
gross trabeculation of the left ventricle and an arteria lusoria. Hydrocephalus, agenesis of corpus
callosum, seizures, absence of septum pellucidum, and ectopic gray and white matter.
Check for the presence of
diaphragmatic hernia and cardiomyopathy. Ventricular fibrillation seems to
result from polymorphic ventricular tachycardia and is refractory to medical
treatment. Perform echocardiography to eliminate cardiac malformations and
ECG to determine conduction defect. Elective surgery should be postponed
until complete electrophysiologic study of the heart has been performed.
Anesthetic considerations are those
associated with cardiac problems, potential peroperative threatening
arrhythmias, and raised intracranial pressure.
Use of anesthetic agents that
increase risk of arrhythmia should be avoided. Patients presenting with
seizures activities should receive their routine preoperative anticonvulsant
medications and intraoperative supplementation of an intravenous antiepileptic
agent may be needed for prolonged procedures.
Goltz Syndrome: Complex mesoectodermal hereditary disorder
characterized by focal dermal atrophy with herniation of fat-producing
multiple papillomas, in association with a skeletal, dental, ocular, and
Aicardi Syndrome: Rare disorder characterized by partial or
complete agenesis of the corpus callosum, infantile spasms (spasm-like
epilepsy), mental retardation, and an ocular abnormality called lacunae of the retina. Often
associated with other features such as microcephaly and porencephalic cysts.
Onset generally begins between the ages of 3 and 5 months. The disorder
affects only females.
Happle R, Daniels O, koopman RJ: MIDAS syndrome (microphthalmia, dermal
aplasia, and sclerocornea). An X-linked phenotype distinct of Goltz
syndrome. Am J med Genet
Paul T, Laohakunakorn P, Long B, et al: Complete elimination of incessant
polymorphic ventricular tachycardia in an infant with MIDAS syndrome. J Cardiovasc Electrophysiol
Temple IK, Al-Gazali LI: Microphthalmia ...