Abstract

To the Editor, Spondylocostal dysostosis (SCD) is a form of shorttrunked dwarfism resulting from a defect in somitogenesis. Both general (GA) and neuraxial anesthesia (NA) may present anesthetic challenges in these patients. A 40-yr-old multiparous woman (G6P3), 140 cm 59 kg, with SCD was examined in anticipation of a planned repeat Cesarean delivery (CD). She had one prior vaginal delivery and two CDs. All previous surgeries were performed under GA. Due to intraoperative awareness with a prior CD, she requested use of NA. The patient’s physical examination revealed a short trunk with disproportionately long extremities and the presence of scoliosis. Her airway examination revealed a restricted neck extension, a 3.5 cm thyromental distance, and a normal mouth opening with a Mallampati II classification. No evidence of cardiopulmonary dysfunction was noted and laboratory studies were unremarkable. A spinal magnetic resonance imaging showed multiple vertebral abnormalities along with cervical spinal stenosis and a C3–C4 disc herniation (Fig. 1). Termination of the thecal sac occurred at the level of the L5 vertebral body, with the conus medullaris ending near the L2–L3 interspace. The operating room (OR) was prepared with an assortment of difficult airway tools, in the event that tracheal intubation became necessary due to either failed or total spinal anesthesia. After aspiration prophylaxis with metoclopramide 10 mg po and sodium citrate/citric acid (30 ml po), the patient was taken to the OR and placed in the sitting position. Spinal anesthesia was achieved with some difficulty using 0.75% spinal bupivacaine 6 mg, fentanyl 10 lg, and preservative-free morphine 100 lg, by means of a 27-G Quinke needle at the L2–L3 interspace. The patient was then placed supine with left lateral uterine displacement, and a bilateral T3 level to pinprick was attained. A male infant was delivered by CD with Apgar scores of 8 and 9 (at one and five minutes, respectively). After approximately two hours of recovery time, the patient was discharged to the postpartum unit in stable condition. The patient consented to publication of this report and the related images. Spondylocostal dysostosis can be either a recessive or a dominant trait, and is characterized by multiple vertebrocostal anomalies without severe thoracic impairment. Costal anomalies include broadening, bifurcation, and fusion; whereas the vertebral defects include block-, wedge-, butterfly-, and hemi-vertebrae. These malformations may lead to progressive scoliosis. Other anomalies associated with SCD include facial dysmorphism, oropharyngeal abnormalities, torticolis, and patent foramen ovale. Despite the suggestion that, in the face of short-stature, catheter-based techniques are superior to single-shot techniques, we chose to use the latter. We thought a flexible spinal needle might be easier to manipulate through the patient’s disordered vertebral anatomy. It was unclear if an uninterrupted epidural space was present; therefore, the success of an epidural block was uncertain. Finally, since treating a spinal headache with a blood patch would likely be very difficult, continuous spinal anesthesia would be a poor choice. It is our practice to adjust the dosage of local anesthetic (LA) in a single-shot spinal, based on the height of the J. A. Dolak, MD, PhD (&) Emory Crawford Long Hospital, Atlanta, USA e-mail: jdolak@emory.edu

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