Septo-optic dysplasia/de Morsier's syndrome

• Type: Article in International Scientific Journal
• Date: 2017

Authors

Reis, P (Author) Other; J Mourão (Author) FMUP

Journal

Title: Saudi Journal of Anaesthesia

Vol. 11

Pages: 106-107

ISSN: 1658-354X

Publisher: Wolters Kluwer Health

Indexing

ISI Web of Knowledge - 2 Citations

Scopus - 1 Citation

Other information

Authenticus ID: P-00M-JKX

DOI: 10.4103/1658-354x.197350

Abstract (EN): Septo-optic dysplasia (SOD)/de Morsier's syndrome is characterized by optic nerve hypoplasia, pituitary endocrine dysfunction, and midline brain abnormalities. Hypopituitarism, hypothyroidism, hypogonadism, and adrenal insufficiency can lead to severe hypoglycemia, adrenal crisis, seizures, and sudden death. Anesthetic management of SOD was associated with high perioperative mortality. A 9-year-old male child proposed for dental treatments/extractions. Medical history of SOD with hypopituitarism, hypothyroidism, and delayed psychomotor development was observed. Anesthetic induction with sevoflurane and intravenous administration of hydrocortisone plus dexamethasone were given. An infusion of 5% glucose in sodium chloride 0.9% was started. Anesthesia with sevoflurane and air, combined with local infiltration with 2% lidocaine, was maintained. During the procedure, the patient was breathing spontaneously, hemodynamically stable, with normal glucose levels measured every 30 min. The patient received 750 mg of paracetamol for analgesia and was discharged from the hospital 24 h after the procedure without complications. The mortality related to general anesthesia in such patients put us some challenges. The procedure was imperative for improving the health and quality of life of the patient, so we opted for inhalational anesthesia combined with local infiltration. We think that combined anesthesia contributed to the abolition of pain and avoided adrenal suppression contributing for the success of the procedure.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 2