Spinal anesthesia for cesarean section in a patient with achondroplasia: case report

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Abstract

Abstract Introduction: The perioperative management of achondroplastic pregnant patients that will undergo lower segment cesarean section represents a clinical challenge to the anesthesiologist. Objective: To describe the anesthetic management of a pregnant patient with achondroplasia, programed for lower segment cesarean section using single-dose regional subarachnoid anesthesia, and to review the general guidelines for anesthetic management emphasizing the role of anesthesia in these patients. Methods: Case report and subject review. Results: The case of a first pregnancy in an achondroplastic, 117-cm tall patient and 38.5 weeks of gestation, programed for lower segment cesarean section is discussed. The patient received single-dose subarachnoid regional anesthesia and adjuvant opioids, with satisfactory results. Conclusion: The ideal anesthetic technique is controversial and the decision shall be based on the individual patient characteristics. The spinal technique, as the anesthetic approach to lower segment cesarean section, was satisfactory and safe in this particular patient.

Translated abstract

Resumen Introducción: El manejo perioperatorio de pacientes acondroplásicas embarazadas que van ser llevadas a cesárea segmentaria representa un reto clínico para el anestesiólogo. Objetivo: Describir el manejo anestésico de una paciente gestante con acondroplasia programada para cesárea segmentaria utilizando anestesia regional subaracnoidea con dosis unica y realizar una revisión acerca de las pautas generales del manejo anestésico destacando el papel de la anestesia regional en este tipo de pacientes. Métodos: Reporte de caso y revisión de tema. Resultados: Presentamos el caso de una primigestante acondroplásica de 38.5 semanas y altura de 117 cm programada para cesárea segmentaria a la que se realizó una técnica anestésica regional tipo subaracnoidea con dosis única de anestésico local más coadyuvante opioide con resultados satisfactorios. Conclusiones: La técnica anestésica ideal para emplear es controversial y debe ser decidido con base en las características individuales de cada paciente. La técnica espinal fue satisfactoria y segura en esta paciente en particular como técnica anestésica para cesárea segmentaría.

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Achondroplasia: from genotype to phenotype.

Pascal Richette, Thomas Bardin, Chantal Stheneur (2008)

This review focuses on the rheumatological features of achondroplasia, which is the most common skeletal dysplasia and the most frequent cause of short-limbed dwarfism. It is inherited in an autosomal dominant manner but results in the majority of cases of de novo mutations. The disease is related to a mutation in the fibroblast growth factor receptor-3 (FGFR3) gene encoding one member of the FGFR subfamily of tyrosine kinase receptors, which results in constitutive activation of the receptor. Biochemical studies of FGFR3 combined with experiments in knock-out mice have demonstrated that FGFR3 is a negative regulator of chondrocytes proliferation and differentiation in growth plate. This mutation induces a disturbance of endochondral bone formation. The diagnosis of achondroplasia is based on typical clinical and radiological features including short stature, macrocephaly with frontal bossing, midface hypoplasia and rhizomelic shortening of the limbs. The most common rheumatological complications of achondroplasia are medullar and radicular compressions due to spinal stenosis and deformities of the lower limbs. Current treatment and future therapies are discussed.