Anesthesia Management in a Patient with Patau Syndrome Who Underwent Bilateral Vitrectomy

Patau syndrome was the first described by Patau et al. and associated with trisomy 13. The syndrome is characterized by microphthalmia, polydacty-ly, and cleft lip-palate. In this case report, we discuss the anesthesiology ap-proach and management in a 2-year-old girl with Patau syndrome undergo-ing bilateral vitrectomy surgery.A 2-year-old girl weighing 12.5 kg was undergoing bilateral vitrectomy sur-gery. There was no additional pathology related to the cardiovascular, cen-tral nervous, and respiratory systems. Following anesthesia induction, the patient was intubated using a # 4.5 uncuffed endotracheal tube. At the end of the operation, 3 mg/kg sugammadex was given to the patient and she was transferred to the remission unit without any problems. During her fol-low-up in the unit, nurses called for help after her oxygen saturation level dropped to 90%. Mask ventilation support was supplied with a chin lift ma-neuver. As re-curarization was thought to be associated with the situation, an additional sugammadex dose was given to the patient. After 20 minutes of follow up, she was transferred to the pediatric ward in a steady state.The doses of anesthetic and analgesic drugs should be titrated carefully be-cause intraoperative opioid and neuromuscular blocker use can lead to post-operative respiratory problems. Therefore, close follow-up of patients is es-sential in the postoperative period, as in the intraoperative period.

___

Patau K, Smith DW, Therman E, et al. Multiple congeni-tal anomaly caused by an extra autosome. Lancet 1960; 1: 79 0 -3.

Duarte AC, Menezes AIC, Devens ES, et al. Patau syndrome with long survival. A case report. Genetics and Molecular Research 2004; 3(2): 288-92.

Fogu G, Maserati E, Cambosu F, et al. Patau syndrome with long survival in a case of unusual mosaic trisomy 13. Eur J Med Genet. 2008; 51(4): 303-14.

Magenis RE, Hecht F, Milham S Jr. Trisomy 13 (D1) syn-drome: Studies on parental age, sex ratio, and survival. J Pediatr. 1968; 73(2): 222-8.

Demir HF, Erakgün A, Çertuğ A. Patau sendromu ön tanılı pediyatrik olguda anestezik yönetim: Olgu sunumu ve literatüre kısa bir bakış. Ege Tıp Dergisi / Ege Journal of Medicine 2013; 52(4): 205-207.

Baum VC, O’Flaherty JE. Anesthesia for genetic, metabol-ic and dysmorphic syndromes of childhood. Lippincott Williams and Wilkins 2007; 374.

Roberts R, Verghese ST, Bauman N. Failure to intubate a child with Patau (Trisomy 13) Syndrome with fiberoptic via the LMA and retrograde wire insertion through the crico-thyroid with the ENT surgeon in the ER. CR39 2010 SPA/AAP Pediatric Anesthesiology. Winter Meeting; April 15-18, 2010.

Bellod Jr A, March X, Hernandez C. Et al. Delayed recu-rarisation after sugammadex reversal. Eur J Anaesthesiol 2014; 31: 708–721

Corre FL, Nejmeddine S, Fatahine C, et al. Recurarization after sugammadex reversal in an obese patient. Can J Anesth 2011; 58: 944–947[

Carollo DS, White WM. Postoperative Recurarization in a Pediatric Patient After Sugammadex Reversal of Rocuronium-Induced Neuromuscular Blockade: A Case Report. A&A Practice. 2019 Apr 11 (Publish Ahead of Print).

Murata T, Kubodera T, Ohbayashi M, et al. Recurarization after sugammadex following a prolonged rocuronium in-fusion for induced hypothermia. Can J Anesth 2013; 60: 508–509.

Kotake Y, Ochiai R, Suzuki T, et al. Reversal with sugamma-dex in the absence of monitoring did not preclude residu-al neuromuscular block. Anesth Analg. 2013; 117: 345–51.

Iwasaki H, Renew JR, Kunisawa T, et al. Preparing for the unexpected: special considerations and complications after sugammadex administration. BMC Anesthesiology 2 017; 17: 14 0 .