Dystrophic epidermolysis bullosa is an inherited mechanobullous disorder caused by mutations in the type VII collagen gene and perturbations in anchoring fibrils.1 It has two genotypes: an autosomal dominant form and an autosomal recessive form. The autosomal recessive form of dystrophic epidermolysis bullosa is the most severe form of the disease. Healing occurs with scarring and results in characteristic deformities that include flexion contractures of the extremities, pseudosyndactyly, microstomia, esophageal strictures, and adhesions of various skin surfaces.2 We describe anesthetic management in six patients aged 3 to 14 years with recessive dystrophic epidermolysis bullosa.
Most of the surgical operations were for release of syndactyly and dressing changes. On physical examination, they had multiple bullae and erosions on their body surfaces. All patients had growth retardation caused by malnutrition and chronic anemia. Two patients had restricted mouth opening because of scarring around the mouth. Anesthesia was provided by axillary brachial plexus block in five patients. In addition to axillary block, spinal anesthesia was performed in three of them to harvest a split-thickness skin graft. In a patient undergoing circumcision, penile block was performed by the pediatric surgeon. In all patients, anesthesia was induced with ketamine, 3 mg/kg body weight, administered intramuscularly to facilitate securing an intravenous line and monitoring devices. For electrocardiographic monitoring, adhesive pads were removed and well-lubricated electrodes were placed beneath the patients. Petroleum jelly–impregnated gauze was laid under the blood pressure cuff to avoid skin trauma. A silicone pad was placed under the patients. Axillary block was performed with a 50-mm, 22-gauge needle using the nerve stimulator. The needle direction was adjusted until flexion of the wrist at the lowest current value. At this point, 0.25% bupivacaine, 2 mg/kg body weight, was injected. Spinal anesthesia was administered with the patient in the lateral decubitus position, and bupivacaine 0.5%, 0.3 to 0.4 mg/kg body weight, was injected intrathecally at the L4-5 interspace. Operating conditions were satisfactory for all patients, and sufficient analgesia was provided during surgery. The severity of pain was measured by means of a visual analogue scale in the postoperative period. The durations of analgesia provided by axillary block and spinal anesthesia were 8.7 ± 2.1 hours and 3.2 ± 1.3 hours, respectively.
The anesthetic management of patients with epidermolysis bullosa is particularly difficult because of oropharyngeal and cutaneous involvement. Perioral scarring restricts opening of the mouth, resulting in airway difficulty.3 Scarring of the oral cavity occurred in 51 percent of cases in the major series of epidermolysis bullosa patients reported, and 6 percent of these patients could not be intubated.4 The principal advantage of the use of regional anesthesia in patients with epidermolysis bullosa is the avoidance of manipulation of the airway. In addition, struggling during emergence from anesthesia is not seen during regional anesthesia. Postoperative pain and excessive movement of the patient in an attempt to eliminate the painful stimuli can easily be avoided.5
In summary, we have provided safe and effective anesthesia with sufficient postoperative analgesia by using regional techniques in our patients with epidermolysis bullosa. New bullae formation was not observed around the site of axillary block in any of the patients. When the surgical site, the procedure, and the condition of the patient are suitable, regional techniques should be considered.
Ozlem Serpil Cakmakkaya, M.D.
Fatis Altindas, Asst. Prof.
Guner Kaya, Prof.
Department of Anesthesiology and Reanimation
Semih Baghaki, M.D.
Department of Plastic, Reconstructive, and Aesthetic Surgery
Cerrahpasa Medical Faculty
University of Istanbul
None of the authors has a financial interest in any of the products, devices, or drugs mentioned in this article.
1. Chen, M., Costa, F. K., Lindvay, C. R., et al. The recombinant expression of full-length type VII collagen and characterization of molecular mechanisms underlying dystrophic epidermolysis bullosa. Biol. Chem.
277: 2118, 2002.
2. Crowley, K. L., and Shevchenko, Y. O. Anesthetic management of a difficult airway in a patient with epidermolysis bullosa: A case report. A.A.N.A. J.
72: 261, 2004.
3. Katz, J., and Steward, D. L. (Eds.). Anesthesia and Uncommon Pediatric Diseases,
2nd Ed. Philadelphia: Saunders, 1987. Pp. 384–387.
4. James, I., and Wark, H. Airway management during anesthesia in patients with epidermolysis bullosa dystrophica. Anesthesiology
56: 323, 1982.
5. Culpepper, T. L. Anesthetic implications in epidermolysis bullosa dystrophica. A.A.N.A. J.
69: 114, 2001.
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