Abstract
Turner Syndrome results from the absence of a sex chromosome, resulting in a female with the 45XO karyotype and major anatomic and physiological changes. Anatomically from head to toe, features may include a short and wide neck which may be webbed, hearing changes, limited neck mobility, high-arched palate, mandibular and maxillary hypoplasia, coarctation of the aorta, bicuspid aortic valve, a smaller than normal trachea, hypothyroidism, and gonadal dysgenesis. Patients have short stature and a higher rate of scoliosis. Physiologically, patients are at higher risk of cognitive impairment. Cardiac anatomic differences result in a higher risk of aortic dissection and intraoperative hypertension. Hypothyroidism often results in delayed gastric emptying and subsequent increased risk of aspiration. There is a higher risk of type 2 diabetes and insulin resistance, as well as liver dysfunction. Kidney abnormalities such as horseshoe kidney may be present; however, they rarely affect kidney function. There are no contraindications to anesthesia in patients with Turner Syndrome, but the phenotypic changes do demand attention to management.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Divekar VM, Kothari MD, Kamdar BM. Anaesthesia in Turner’s syndrome. Can Anaesth Soc J. 1983;30(4):417–8.
Romano AA, Allanson JE, Dahlgren J, Gelb BE, Hall B, Pierpont ME, et al. Noonan syndrome: clinical features, diagnosis, and management guidelines. Pediatrics. 2010;126(4):746–59.
McLure HA, Yentis SM. General anesthesia for caesarean section in a parturient with Noonan’s syndrome. Br J Anesth. 1996;77:665–8.
Ingrande J, Brodsky JB, Lemmens HJ. Regional anesthesia and obesity. Curr Opin Anaesthesiol. 2009;22(5):683–6.
Taivainen T, Tuominen M, Rosenber PH. Influence of obesity on the spread of spinal analgesia after injection of plain 0.5% bupivacaine at the L3–4 or L4–5 interspace. Br J Anaesth. 1990;64(5):542–6.
Hodgkinson R, Husain FJ. Obesity and the cephalad spread of analgesia following epidural administration of bupivacaine for cesarean section. Anesth Analg. 1980;59:89–92.
Bomberg H, Albert N, Schmitt K, Gräber S, Kessler P, Steinfeldt T, et al. Obesity in regional anesthesia—a risk factor for peripheral catheter-related infections. Acta Anaesthesiol Scand. 2015;59(8):1038–48.
Ravenscroft A, Govender T, Rout C. Spinal anaesthesia for emergency caesarean in an achondroplastic dwarf. Anaesthesia. 1998;53:1236–7.
Wiesman T, Castori M, Malfait F, Wulf H. Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet J Rare Dis. 2014;9:109.
De Paepe A, Malfait F. The Ehlers–Danlos syndrome, a disorder with many faces. Clin Genet. 2012;82:1):1–11.
Ko JY, Leffert LR. Clinical implications of Neuraxial anesthesia in the parturient with scoliosis. Anesth Analg. 2009;109(6):1930–4.
Perlas A. Evidence for the use of ultrasound in Neuraxial blocks. Reg Anesth Pain Med. 2010;35(2):S43–6.
Wongprasartsuk P, Stevens J. Cerebral palsy and anaesthesia. Pediatr Anesth. 2002;12(4):296–303.
Van Dyke DC, Allen M. Clinical management considerations in long-term survivors with trisomy 18. Pediatrics. 1990;85(5):753–9.
Bos AP, Broers CJ, Hazebroek FW, van Hemel JO, Tibboel D, Wesby-van Swaay E, et al. Avoidance of emergency surgery in newborn infants with trisomy 18. Lancet. 1992;339:913–5.
Tucker ME, Garringer HJ, Weaver DD. Phenotypic spectrum of mosaic trisomy 18: two new patients, a literature review, and counseling issues. Am J Med Genet A. 2007;143A(5):505–17.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2021 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Thomas, DA., Missih, O., Zhu, R., Suchy, T., Vadivelu, N. (2021). Common Congenital Syndromes and Disease States Impacting Regional Anesthesiology Techniques. In: Narayan, D., Kapadia, S.E., Kodumudi, G., Vadivelu, N. (eds) Surgical and Perioperative Management of Patients with Anatomic Anomalies. Springer, Cham. https://doi.org/10.1007/978-3-030-55660-0_6
Download citation
DOI: https://doi.org/10.1007/978-3-030-55660-0_6
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-55658-7
Online ISBN: 978-3-030-55660-0
eBook Packages: MedicineMedicine (R0)