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Delleman syndrome: anesthetic implications

S Sadhasivam¹, R Subramaniam

¹Department of Anesthesiology and Intensive Care, All India Institute of Medical Sciences, New Delhi.

Anesthesia and Analgesia

|September 5, 1998

Summary

This summary is machine-generated.

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Delleman syndrome, a rare condition, presents complex congenital anomalies. This case highlights crucial anesthetic considerations for managing patients with oculocerebrocutaneous syndrome.

Area of Science:

Pediatric Neurology
Medical Genetics
Anesthesiology

Background:

Oculocerebrocutaneous (Delleman) syndrome is an extremely rare congenital disorder with fewer than 30 reported cases.
The syndrome is characterized by a constellation of ocular, cerebral, and cutaneous anomalies.

Observation:

A neonate presented with a spectrum of Delleman syndrome features including alopecia, skin hypertrophy, orbital cyst, lid coloboma, cleft palate, neonatal seizures, cerebral hemiatrophy, and intracranial cysts.
The infant experienced significant preoperative challenges, including neonatal seizures and aspiration pneumonia.

Findings:

Surgical interventions for Delleman syndrome anomalies, such as orbital cyst drainage and cleft palate repair, frequently necessitate multiple anesthetic procedures.
Despite preoperative complications, the infant's perioperative course was uneventful, underscoring the importance of thorough preoperative assessment.

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Implications:

This case underscores the critical need for anesthesiologists to be aware of the potential anesthetic risks associated with Delleman syndrome.
Careful preoperative evaluation and planning are essential for optimizing patient outcomes in rare congenital disorders like Delleman syndrome.