General anaesthesia for parturients with spondyloepiphyseal dysplasia: Risky but possible!

Abstract

Sir, A 28-year-old primigravida, 135 cm tall and a known case of spondyloepiphyseal dysplasia (SED), with 37 weeks of gestation, was scheduled for elective caesarean section. The patient was unable to walk and was wheelchair bound. The flexion deformity of the knee joint, as well as the hip joint, made it difficult for the patient to lie down straight [Figure 1]. In addition, she had flexion deformities of the elbow and wrist joints.Figure 1: Patient with fixed flexion deformities of the knee and hip jointsThe patient was the first child of her parents who had non-consanguineous marriage. The other siblings of the patient also had similar deformities. Patient's biochemical, haematological investigations, echocardiography and pulmonary function tests were unremarkable. The patient had limited neck movements but had modified Mallampati Airway Class 3. The patient's neck extension was two-thirds restricted and her mouth opening was about 40 mm. Other airway parameters were within normal limits. Examination of the spine revealed scoliosis, and intervertebral spaces were difficult to palpate. Radiographic examination (pre-pregnancy) revealed platyspondyly, reduced disc height as well as features of premature secondary arthritis in both hip joints [Figure 2]. She was unable to position herself for regional anaesthesia. The fixed flexion deformity of the knee prevented lateral supine positioning of the patient. She could sit with two pillows behind her back and a pillow below her knee, and slept in head-up position.Figure 2: X-ray of the hip joints showing early osteoarthritis resulting in fixed flexion deformityThe patient did not consent for regional anaesthesia. Hence, it was decided to conduct caesarean section under general anaesthesia. She was positioned with a pillow under the knees along with a wedge under the abdomen. The head was also supported on a head ring with two pillows below her head. Intravenous line was secured with an 18 gauge cannula and infusion of Ringer lactate was started. Routine monitors were connected and basal readings noted. Awake nasal fibre-optic intubation was performed with small size of endotracheal tube (ETT) using cuffed polyvinyl chloride 6.5 mm ETT uneventfully. The rest of the anaesthetic and post-operative period was uneventful. SED is an extremely rare genetic entity with incidence of 1–4 per million. Regional anaesthesia is the method of choice in parturients with SED although it is technically difficult and has variable effects. Administration of general anaesthesia in such patients is not without risks. The presence of kyphoscoliosis in SED may lead to significant respiratory compromise that gets exaggerated in pregnancy due to diaphragmatic splinting caused by gravid uterus.[1] Pregnancy further exaggerates the already existing respiratory compromise and enhances aortocaval compression. The presence of atlantoaxial instability due to odontoid hypoplasia, central anterior pointing of the cervical spine along with ligamentous laxity in SED may increase the risk of severe spinal cord compression. Tetraparesis has been reported after intubation in these patients due to cervical spine instability.[2] Pregnancy also poses an added risk for aspiration.[1] Patients with SED may have associated laryngotracheal stenosis, which may pose difficulty in airway management. There are reports of intubation being guided by lightwand.[3] Intubation with video laryngoscope could be another tangible alternative.[4] However, video laryngoscope was unavailable during intubation in the management of the present patient. Thus, patients with SED with pregnancy can pose a special challenge for the anaesthesiologist. However, while administering general anaesthesia, proper assessment and monitoring for cervical spine stability and respiratory status, positioning of the patient so as to prevent any untoward neurological injury and meticulous planning of airway management and perioperative care may go a long way in successful outcome. Declaration of Patient Consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Acknowledgement We would like to acknowledge Dr. P. N. Gupta, Associate Professor, Department of Orthopaedics, Government Medical College and Hospital, Chandigarh, for his help.