Anesthetic management of a parturient with oculo-dento-digital (ODD) syndrome for emergency cesarean section

Dear Editor, Oculo-dento-digital (ODD) syndrome is a rare multisystemic autosomal dominant disease, with fewer than 300 known cases worldwide. It is characterized by facial dysplasia, skeletal anomalies, and ocular problems, and remains a challenge for the anesthetic management.[1] A 28-year-old primigravida at term gestation, of height 155 cm and weight 80 kg, was posted for a lower segment cesarean section, given non progress of labor. She was a known case of ODD and had a history of shallow anterior chamber glaucoma, and bilateral avascular necrosis of the hip, which was managed conservatively without any residual deformity. Her airway examination revealed a mouth opening of 4 cm with modified Mallampati grade II, restricted neck extension, and multiple brittle loose teeth. Thoracolumbar kyphoscoliosis was also observed. She was planned for a subarachnoid block (SAB), and a difficult airway cart was kept ready for backup in case of failed SAB. SAB was performed in the L3–4 interspace with 1.8 mL of 0.5% hyperbaric bupivacaine to achieve a T4 level of sensory blockade. Intraoperative hypotension was managed with intravenous phenylephrine boluses to maintain a targeted mean arterial pressure of 70 mmHg. A female child was delivered, whose preliminary examination revealed the presence of syndactyly, as the disease is autosomal dominant. Ultrasound-guided transversus abdominis plane block was performed for postoperative analgesia, and the patient was shifted to the post-anesthesia care unit for observation. She was discharged with advice to follow-up in the outpatient department. ODD is an autosomal dominant disorder with myriad skeletal abnormalities, including microcephaly, depressed columella, mandibular overgrowth, cleft palate, and characteristic features of brittle and loose teeth with loss of enamel. The multisystemic presentation along with the anesthetic concerns and management are given in Table 1 and depicted in Figure 1.Table 1: Summary of ODD implications and it's anaesthetic concernsFigure 1: (a) X-ray both hands – Antero-posterior (AP) view depicting fused fingers. (b) Syndactyly. (c) X-ray neck – lateral view depicting short neck, mandibular overgrowth. (d) Brittle teethEarly recognition and assessment including a comprehensive ocular, neurological, hearing, and dental evaluation is crucial. Those at risk of glaucoma and raised intraocular pressure should receive antiglaucoma treatment. Arrhythmias necessitate cardiology consultation. Plastic or orthopedic surgery is indicated for severe limb malformations.[4,5] Hence, it is imperative that meticulous pre-anesthetic evaluation and planning be done well in the antenatal period for improved parturient and baby outcomes. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.