OJAnes  Vol.5 No.2 , February 2015
Anesthetic Management of a Child with Simpson-Golabi-Behmel Syndrome—A Case Report
Abstract: Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked recessive condition associated with pre-natal and postnatal overgrowth with visceral and skeletal anomalies. Abnormal airway anatomy may complicate the perioperative management of SGBS. However, there have been few reports regarding the anesthetic management of children with SGBS. We present the preoperative evalua-tion and intraoperative management of a 1.5-year-old child with SGBS undergoing plastic surgery for cleft palate. Sevoflurane-fentanyl anesthesia without a neuromuscular blocker was planned, due to the preoperative evaluation that airway management would be complicated by the macroglossia and short neck characteristic of SGBS. Fortunately, there was no difficulty in endotracheal intubation in the present patient due to careful planning of general anesthesia and meticulous airway management. Appropriate preoperative preparation for a difficult airway by the anesthe-siologist can ameliorate the difficulties inherent to the anesthetic management of patients with SGBS.
Cite this paper: Morita, Y. , Naito, K. , Kimoto, N. , Kimoto, E. , Omata, T. , Morita, N. and Nakagawa, M. (2015) Anesthetic Management of a Child with Simpson-Golabi-Behmel Syndrome—A Case Report. Open Journal of Anesthesiology, 5, 23-26. doi: 10.4236/ojanes.2015.52005.

[1]   Simpson, J.L., Landey, S., New, M. and German, J. (1975) A Previously Unrecognized X-Linked Syndrome of Dysmorphia. Birth Defects Original Article Series, 11, 18-24.

[2]   Xuan, J.Y., Hughes-Benzie, R.M. and MacKenzie, A.E. (1999) A Small Interstitial Deletion in the GPC3 Gene Causes Simpson-Golabi-Behmel Syndrome in a Dutch-Canadian Family. Journal of Medical Genetics, 36, 57-58.

[3]   Neri, G., Gurrieri, F., Zanni, G. and Lin, A. (1998) Clinical and Molecular Aspects of the Simpson-Golabi-Behmel Syndrome. American Journal of Medical Genetics, 79, 279-283.<279::AID-AJMG9>3.0.CO;2-H

[4]   Tsuchiya, K., Takahata, O., Sengoku, K., Hamada, I., Suzuki, A. and Iwasaki, H. (2001) Anesthetic Management in a Patient with Simpson-Golabi-Behmel Syndrome. Masui, 50, 1106-1108.

[5]   Behmel, A., Plöchl, E. and Rosenkranz, W. (1984) A New X-Linked Dysplasia Gigantism Syndrome: Identical with the Simpson Dysplasia Syndrome? Human Genetics, 67, 409-413.

[6]   Golabi, M. and Rosen, L. (1984) A New X-Linked Mental Retardation-Overgrowth Syndrome. American Journal of Medical Genetics, 17, 345-358.

[7]   Garganta, C.L. and Bodurtha, J.N. (1992) Report of Another Family with Simpson-Golabi-Behmel Syndrome and a Review of the Literature. American Journal of Medical Genetics, 44, 129-135.

[8]   Vora, N. and Bianchi, D.W. (2009) Genetic Considerations in the Prenatal Diagnosis of Overgrowth Syndromes. Prenatal Diagnosis, 29, 923-929.

[9]   Gurkowski, M.A. and Rasch, D.K. (1989) Anesthetic Considerations for Beckwith-Wiedemann Syndrome. Anesthesiology, 70, 711-712.

[10]   Taniyama, T., Kitai, N., Iguchi, Y., Murakami, S., Yanagi, M. and Takada, K. (2003) Craniofacial Morphology in a Patient with Simpson-Golabi-Behmel Syndrome. The Cleft Palate-Craniofacial Journal, 40, 550-555.<0550:CMIAPW>2.0.CO;2

[11]   Morita, Y., Kimoto, N., Ogawa, H., Omata, T. and Morita, N. (2011) Simpson-Golabi-Behmel Syndrome Associated with Cleft Palate. Journal of Craniofacial Surgery, 22, 1917-1918.

[12]   Filippi, G. and Mckusick, V.A. (1970) The Beckwith-Wiedemann Syndrome. Medicine, 49, 279-298.

[13]   Norioka, M., Kitamura, S., Sumikawa, K., Nagai, H., Amakata, Y., Ota, S., Kosugi, K. and Harano, Y. (1981) EMG Syndrome and Anesthesia (Author’s Translation). Masui, 30, 1243-1248.

[14]   Walker, R.W.M. and Ellwood, J. (2009) The Management of Difficult Intubation in Children. Pediatric Anesthesia, 19, 77-87.

[15]   Xue, F.S., He, N., Xu, Y.C. and Liao, X. (2009) Anesthesia and Airway Management for Children with Macroglossia. Pediatric Anesthesia, 19, 275-277.

[16]   Inomata, S., Maeda, T., Shimizu, T., Satsumae, T. and Tanaka, M. (2010) Effects of Fentanyl Infusion on Tracheal Intubation and Emergence Agitation in Preschool Children Anaesthetized with Sevoflurane. British Journal of Anaesthesia, 105, 361-367.