MIDAS Syndrome

At a glance

MIDAS is an acronym that stands 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. The association of MIDAS Syndrome and severe congenital heart defects/cardiomyopathy has been reported. Sudden death in the young child should lead toward this diagnosis.

Synonyms

Microphthalmia-Linear Skin Defects Syndrome; MLS.

Incidence

The exact incidence remains unknown. Approximately 20 cases described.

Genetic inheritance

It is believed transmitted as an X-linked dominant trait. It is lethal in utero in males. Mapped to the short arm of chromosome X (Xp22.31). It is considered as an X-linked phenotype distinct from ☞Goltz Syndrome.

Clinical aspects

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). The presence of focal dermal hypoplasia or aplasia affecting usually the face, scalp, and neck is part of the diagnosis. The skin lesions show linear burns or cuts and are present at birth. They heal without leaving a scar. The skin can remain pigmented and depressed at the location of the lesions. Affected patients present mild short stature. Other features include diaphragmatic hernia and in rare occurrence 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. There are reports of associated hydrocephalus, agenesis of corpus callosum, seizures, absence of septum pellucidum, and ectopic gray and white matter.

Precautions before anesthesia

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

Anesthetic considerations are those associated with cardiac problems, potential peroperative threatening arrhythmias, and raised intracranial pressure.

Pharmacological implications

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.

Other conditions to be considered

• ☞Goltz Syndrome: Complex mesoectodermal hereditary disorder characterized by focal dermal atrophy with herniation of fat-producing multiple papillomas, in association with skeletal, dental, ocular, and other anomalies.

• ☞Aicardi Syndrome:...