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- DOI 10.18231/j.jsas.2025.018
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CrossMark
Perioperative management of a patient with tetra-phocomelia undergoing anterior cervical decompression and fixation: A case report
- Author Details:
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Prajakta N Patil
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Vaibhavi Baxi
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Yasaswini Jami
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Naba Mateen Madoo *
Naba Mateen Madoo *
Phocomelia is a rare congenital condition characterized by severe limb malformation, where the limbs are either partly or completely underdeveloped. Phocomelia can occur as a syndrome or as a limb-specific abnormality. It is characterized by high rates of stillbirth, intrauterine demise, and early neonatal mortality. Administering anaesthesia to these patients is challenging because of anatomical and physiological variabilities. Limited experience exists regarding the management of this condition in surviving adults, and no standardized perioperative or anaesthetic guidelines are available. We present the perioperative management of a 22-year-old female with isolated non-syndromic phocomelia, with no history of intrauterine teratogenic exposure, who underwent anterior cervical decompression and fixation at the C3–C5 level. Key anaesthetic challenges included difficult intravenous access, complex arterial and venous catheterization due to anatomical variation, and positioning difficulties.
Keywords: Phocomelia, Tetra-phocomelia, Anaesthetic Management, Cervical Decompression
References
- Hecht F, Gorlin RJ. Phocomelia: an analysis of sixty-two cases and delineation of the syndrome. Am J Hum Genet. 1970;22(3):298-305.
- Stevenson RE, Hall JG, Goodman RM. Human Malformations and Related Anomalies. Oxford: Oxford University Press; 1993.
- Vargesson N. Thalidomide-induced limb defects: resolving a 50- year-old puzzle. Bioessays. 2009;31(12):1327-36.
- Aase JM. Diagnostic Dysmorphology. New York: Springer; 2005.
- Ahmed S, Shankar H. Invasive Blood Pressure Monitoring. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2023.
- Sousa SB, Abdul-Rahman OA, Bottani A, Graham JM, Manouvrier- Hanu S, Fryns JP. et al. Phenotypic and genotypic variability in Roberts syndrome. Am J Med Genet A. 2010;152A(6):1470-9.
- Brown L, Morgan CJ. Central venous access: techniques and complications. Anesthesiol Clin. 2017;35(4):735-52.
- Langeron O, Birenbaum A, Francon D. Handling the patient with a difficult airway. Minerva Anestesiol. 2009;75(7-8):447-52.
- Tosi LL, Warman ML. Primary limb abnormalities. J Am Acad Orthop Surg. 2004;12(6):409-17.