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Obstetric Anesthetic Considerations for Epidermolytic Hyperkeratosis: A case report.
Abstract Number: S-01
Abstract Type: Case Report/Case Series
A 30-year-old G1P0 with epidermolytic hyperkeratosis (EHK) at 38 weeks gestation was admitted and induced with oxytocin in the anticipation of spontaneous vaginal delivery after premature rupture of membranes. She had significant epidermal dehiscence with generalized scaling. Intravenous catheter placement was difficult due to the thickness of her skin and inability to visualize the veins and required non-adhesive gauze for securing. A decision was made to perform an emergent cesarean delivery due to non-reassuring fetal heart tones. In operating room, the EKG leads were selectively placed in areas with the least wound dehiscence and the blood pressure cuff and pulse oximetry were placed without any difficulty or damage to the skin. Routine spinal anesthesia was performed after skin preparation with povidone-iodine and local infiltration with lidocaine and an appropriate level was obtained. The surgical field was prepared with chlorhexidine and cesarean delivery was completed without maternal complications. The male newborn required an emergency dermatology consultation due to evidence of the same skin condition and required immediate treatment with antibiotics and moisturizer to reduce the chance of infection.
The patient was monitored closely for wound healing. She was discharged home on the third post-operative day but returned two days later with complaints of fever up to 40 degrees Celsius and purulent drainage from the incision. Spinal anesthesia was performed in the same fashion for wound exploration. A massive wound infection was identified and general anesthesia with endotracheal intubation was performed upon the decision to remove the infected uterus. The wound healed well after the hysterectomy and she was discharged on post-operative day eight.
EHK is a rare autosomal dominant disorder of keratinization (1). EHK usually presents with generalized erythema, blisters, and erosions, which could become sources of infection. Patients later develop hyperkeratotic scaling (2,3). Anesthesia challenges include the difficulty to find and secure intravenous catheters, as well as, the decision of whether or not to proceed with regional anesthesia due to the fact that patients with this disease are prone to delayed wound healing and infection. Ultimately, in this case there were no complications regarding the spinal anesthesia most likely due to the meticulous antiseptic technique completed during each placement. However, the risks of general versus regional anesthesia in a patient with EHK must be weighed carefully on an individual basis.
1.Arin MJ et al. Expanding the keratin mutation database: novel and recurrent mutations and genotype-phenotype correlations in 28 patients with epidermolytic ichthyosis. Br J Dermatol. 2011. 164:442.
2.Kwak J et al. Epidermolytic hyperkeratosis. Dermatology Online Journal. 2006. 12(5):6.
3.DiGiovanna JJ et al. Epidermolytic hyperkeratosis: applied molecular genetics. J Invest Dermatol. 1994; 102: 390.