J Dent Anesth Pain Med.  2017 Sep;17(3):215-217. 10.17245/jdapm.2017.17.3.215.

Anesthetic management of a patient with branchio-oto-renal syndrome

• Tsukamoto
• Yokoyama T

• KMID: 2390806
• DOI: http://doi.org/10.17245/jdapm.2017.17.3.215

Abstract

Branchio-oto-renal syndrome (BOR) is a rare autosomal dominant disorder. The features include branchial cysts, hearing loss, ear malformation, preauricular pits, retrognathia, congenital heart disease, and renal abnormalities. However, anesthetic management of these patients has seldom been reported. We report a case in which general anesthesia was performed for dental treatment in a patient with BOR. Airway management, renal function, and hemodynamic changes can be of critical concern during anesthetic management. A 13-year-old girl diagnosed with BOR had severe right hearing loss, right external ear malformation, renal abnormalities, and postoperative patent ductus arteriosus (PDA). Dental extraction under general anesthesia was scheduled for a supernumerary tooth. The procedure was completed with sufficient urine volume, adequate airway management, and stable hemodynamics.

Keyword

Branchio-oto-renal Syndrome; Congenital Heart Disease; Difficult Airway; General Anesthesia; Renal Failure

MeSH Terms

Adolescent
Airway Management
Anesthesia, General
Branchio-Oto-Renal Syndrome
Branchioma
Ductus Arteriosus, Patent
Ear
Ear, External
Female
Hearing Loss
Heart Defects, Congenital
Hemodynamics
Humans
Renal Insufficiency
Retrognathia
Tooth, Supernumerary

Reference

1. Taylor MH, Wilton NC. Bradycardia with sevoflurane in siblings with Branchio-oto-renal syndrome. Paediatr Anaesth. 2007; 17:80–83.

2. Senel E, Kocak H, Akbiyik F, Saylam G, Gulleroglu BN, Senel S. From a branchial fistula to a branchiootorenal syndrome: a case report and review of the literature. J Pediatr Surg. 2009; 44:623–625.

3. Chavan A, Shastri AR, Ross-Russell RI. Branchio-oto-renal syndrome with obstructive sleep apnoea. BMJ Case Rep. 2012; 2012.

4. Amer I, Falzon A, Choudhury N, Ghufoor K. Branchiootic syndrome--a clinical case report and review of the literature. J Pediatr Surg. 2012; 47:1604–1606.

5. Smith RJ, Schwartz C. Branchio-oto-renal syndrome. J Commun Disord. 1998; 31:411–420.

6. Misra M, Nolph KD. Renal failure and deafness: branchiooto-renal syndrome. Am J Kidney Dis. 1998; 32:334–337.