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Cesarean section for a patient with Pseudoachondroplasia
Abstract Number: F 66
Abstract Type: Case Report/Case Series
Patients with Pseudoachondroplasia can present with significant challenges to the OB anesthesiologist. We present here a case of a 23 years old G2P1001 at 39 weeks gestation with pseudoachondroplasia and a medical history significant for asthma scheduled for a repeat elective C section.
Pseudoachondroplasia is a rare rhizomelic type of skeletal dysplasia which develops secondary to a mutation within genes encoding for cartilage oligomeric matrix protein (COMP) on chromosome 19. COMP is found in the extracellular matrix of the cartilage, tendon, and ligaments.
Most families have demonstrated an autosomal dominant inheritance pattern, but few germline/somatic mutations have been suggested.
The children are invariably normal at birth, and they usually present either around 2 years of age with a delay in walking or a little later with an abnormal waddling gait or lower limb deformity. Over the years, the rhizomelic type of dwarfism becomes apparent with progressively increasing morbidity.Physical examination of these patients reveals normal facies and intelligence. The adult height usually ranges between 82–130 cm with marked shortening of limbs. Associated deformities include limited neck extension, foramen magnum stenosis and atlanto-axial instability. Diagnosis is by Radiology and clinical signs.
We present here a case of a 23 years old pseudoachondroplasic dwarf presenting at 39 weeks gestation for a repeat elective C section.
She had regular follow up with the OB /GYN team during her current pregnancy from which one of the progress notes documented a questionable history of subglottic stenosis. Her physical exam was essentially normal except for a height of 3 feet 11 inches and weight of 142 lbs.Airway exam was Mallampati 1, with free range of movement of the neck, no signs or symptoms of cervical instability,and no radiology records on file. Her prior anesthetic history revealed a somewhat difficult epidural for her previous C section performed for cephalopelvic disproportion a year ago. Keeping this in mind, our plan was to do what had been done before and had worked for her, i.e. an epidural anesthetic. Accordingly, an epidural was placed without much difficulty and was dosed incrementally with 2% lidocaine with 1:200,000 epinephrine administered via the epidural catheter. The sympathetic block did not progress beyond T 12 level. Subsequent attempts to place a subarachnoid block were unsuccessful and the plan to convert to general anesthesia was made. Keeping in mind the propensity of these patients for cervical instability, and despite the absence of any obvious clinical signs and symptoms, in-line stabilization during endotracheal intubation was done. The baby was delivered with Apgar scores of 8 and 9 at 1 and 5 minutes respectively. The patient had an uneventful anesthetic and was successfully extubated at the end of the procedure. The post operative course was uneventful as well and she was discharged home on post operative day 3.