Epidermolysis bullosa

Epidermolysis bullosa is a hereditary rare condition characterized with local and generalized lesions and autosomal dominant trait with variable penetrance. It was decided to amputate the left leg under the knee of a female patient with epidermolysis bullosa with squamous cell carcinoma. The smallest trauma may cause formation of serious bullous in the skin in epidermolysis bullosa. Surgeons, dermatologists, and anesthesiologists must evaluate the cases. During preoperative, intraoperative, and postoperative periods, all interventions that may cause interruption in circulation of the tissues, cause irritation, or delay healing of the wounds must be avoided.

Introduction

Epidermolysls bullosa (EB) is a hereditary disorder with autosomal and recessive inheritance characterized with local and generalized lesions and incidence between 1/50,000 and 1/30,000. More than 20 subtypes have been described up until now. It has three different types according to the severity of the lesions: simplex, junction, and dystrophic.1,2

The disorder is characterized with bullous in the stratified squamous epithelium of oropharynx, lips, and esophagus. Patients with EB usually have malnutrition, infection, and a mucocutaneous type of squamous cell carcinoma.

In this study, we discuss a case of left amputation below the knee and anesthetic technique in an EB dystrophic patient, and we provide a literature review.

case Report

The patient was a 37-year-old woman with a weight of 62 kg, height of 157 cm, and a definitive diagnosis of dystrophic EB. Fingers were welded together in both hands. Local necrotic lesions were present in both extremities (Fig. 1). Blood circulation below the left knee was poor and tissues were necrotic in that region. She had squamous cell carcinoma on her left leg. The patient was in a state of excessive anxiety in the preoperative visit, and she refused our proposal of spinal anesthesia. Therefore, we planned a left amputation below the knee under general anesthesia. There were no abnormalities in biochemical tests or electrocardiogram (ECG) test thoracic radiograms.

There were widespread bullous around the mouth and all over the body. The patient's mouth could open only partly, and her neck movements were limited because of the scar tissue from the healed lesions. Her teeth were in a bad condition and most of them were decayed. Difficult intubation was considered likely and preparations were carried out as such.

We decided to induce the anesthesia with sevoflurane inhalation with minimal trauma. We gave her 10 mg of oral diazepam with the 0.5-mg/kg dose 30 minutes before the operation. The patient was monitored using a noninvasive pulse oximetry, which was connected to her ear as there were no fingers to use. Cotton wool was wrapped around the arm before placing a noninvasive sphygmomanometer's cuff. For ECG monitoring, adhesive parts of the leads were taken out; metal endings were coupled with leads and were kept in place by wrapping them with dressing material. Thus, it was possible to monitor the patient without adhering the electrodes to the thorax. The vital parameters were such: blood pressure, 130/85 mm Hg; heart rate, 80 beats per minute; and SpO^sub 2^, 99%. When these measurements were taken, the patient was in a state of composure, she opened her eyes when addressed, and showed a tendency to sleep. We started anesthesia induction when she was in this state with inhalation through a mask and using 3% sevoflurane, 50% oxygen, and 47% N^sub 2^O. We increased sevoflurane concentration gradually up to 6%. We cut a piece of dressing material just as big as the opening of her mouth and put it under the mouthpart of the mask to prevent contact and any irritation because of that contact.

An oropharyngeal airway no. 3 that was thoroughly lubricated was placed in the mouth because her teeth were not in a good position; thus, spontaneous breathing was kept under control. Having reached the adequate level of anesthesia, the maintenance of anesthesia was carried out with 1.5% sevoflurane, 40% oxygen, and 58.5% N^sub 2^O.

While she was under anesthesia, we opened an intravenous port with a catheter no. 20 on right vena jugularis externa. Adhesive bands were not used to fix the intravenous catheter in place; instead, we placed a cotton piece wetted with antiseptic on the insertion point of the catheter and dressed it (Fig. 2).

We discovered that it was impossible to place the laryngoscope and laryngeal mask airway upon examining the mouth opening. Therefore, we kept the mask on the patient. We gave 0.3 mg of atropine for premedication in the form of a bolus injection. No muscle relaxants, narcotics, and intravenous hypnotics were used. A dose of 1-mg/kg tramadol intravenous bolus was given for postoperative analgesia intraoperatively. Timing was planned for intravenous patient-controlled analgesia and tramadol (basal infusion with a rate of 2 mL/h, 4 mL of bolus, lock out 25 minutes). An intravenous cannula was taken out after 24 hours. Application of 50 mg of tramadol three times in a day was the planned pain oral treatment. A 5-mL/kg intravenous liquid was given intraoperatively.

We cleaned the skin of the left extremity up to the loin with antiseptic solution taking care not to irritate the skin. We did not use a tourniquet, again, to prevent irritation. We incised the skin and subcutaneous tissues on the left extremity 10 cm distal to the tuberositas tibiae with a fish-mouth incision. We tied and cut neurovascular structures. We cut tibiae and fibula with a Gigll saw. We established adequate soft tissue support under tibiae and fibula, achieved homeostasis, and then closed the tissues. We sutured the skin with sutures using 3-0 prolene without causing tension (Fig. 3). The whole operation took 75 minutes. Hemodynamic findings were normal during the operation. Visual Analog Scale was 3 after 30 minutes following awakening. Sutures were taken off on the 10th day after the operation. No serious complications were encountered on the follow-up.

Discussion

Great care must be taken in cases of EB, during preanesthesia and the preoperative, intraoperative, and postoperative periods. Anemia, hypoproteinemia, and electrolyte imbalances are common in these patients in the preoperative period.3'5 Parenteral nutrition can be necessary if there is severe malnutrition. The number of lesions (such as bullous, scar tissue, oropharyngeal adhesions) is an important factor too. In our case, lesions were widespread, there was extreme loss of tissue in the extremities, and there was significant scar tissue on the neck.

During the operation, soft pads must be placed on the operation table, ECG adhesive plates must not be used, and needle electrodes must be preferred for ECG monitoring. Intravenous and intra-arterial catheters can be fixed with elastic bandages or sutures.1'3,6 Cotton wool must be placed under the cuff of the sphygmomanometer to protect the skin. We fixed the intravenous catheter with dressing material in our procedure.

Pomades with 0.5% hydrocortisone can be used on joints (knees, wrists, ankles) in combination with supports to prevent pressure on these areas.

The slightest trauma may cause bullous formation in patients with EH.7,8 Even intramuscular injections are avoided because of this reason. We did not give any intramuscular injections to our case subject. However, there are some studies expressing that no problems arise with intramuscular injections.1

Regional anesthesia can be a good solution in cases with EB. However, we did not plan regional anesthesia, because the patient did not consent to it; although it is generally agreed upon that regional anesthesia is advantageous in these cases.1,6,9-11 Ketamine is announced as another good alternative in these cases.3,12 We did not use ketamine for anesthesia because our patient was not in good condition psychologically. Ketamine does not effect spontaneous respiration and respiratory reflexes adversely; however, it may cause hallucinations and a significant increase in secretions.13·14

When the literature is reviewed, it is seen that the most common anesthesia technique used is inhalation anesthesia. It is preferred in children and in cases if the maintenance of anesthesia is planned to be with inhalation.1 No publications were found on anesthesia with sevoflurane in EB cases, although there were many about anesthesia with halothane, cyclopropane, and isoflurane. However, sevoflurane is an inhaled agent with rapid and potent effect, and recovery after sevoflurane is quick.

Great care must be taken when using laryngoscope and intubating. Oropharyngeal bullous formation and bleeding may occur in patients with EB. The difficulties of establishing the airway arise from microstoma, bad structures of the teeth, adhesions in the oropharyngeal region, and limitations in the flexion of neck and extension of head. The blade, of the laryngoscope and endotracheal tube must be lubricated generously with petroleum jelly while intubating. James and Wark15 encountered difficulties of intubation in 17 of 31 cases and managed intubating in 2 cases. In our case, the patient's mouth opening was so small and movement of the neck was so limited that we decided to apply inhalation anesthesia with a mask. Intubation with oral or nasal fiber optic laryngoscope could be used, but we decided not to use them to avoid trauma. Air cushions of the mask must be inflated properly; wet cotton wool or dressing like we did must be placed between the skin and the mask if a facial mask shall be used for the airway.

Ketamine or penthotal can be given with potent inhalation agents. However, it must be taken into consideration that these cases can be with porphyria, and great care must be taken when using penthotal.16,17

It must not be forgotten that ketamine can cause tachycardia and hypertension when causing sedation, loss of conscious, and analgesia intraoperatively; and it may cause hallucinations postoperatively. Ketamine is contraindicated in patients with hypertension, myocardial insufficiency, and increased intracranial pressure and in psychotic patients. Benzodiazepins can prevent psychomimetic adverse effects.12 We did not use ketamine in our patient because she had psychological problems.

Muscle relaxants can be used in cases with EB. Depolarizing muscle relaxants must be avoided because atrophie muscles will release K+ as a result of contractions and hyperkalemia will result.5,18

Nondepolarizing muscle relaxants, on the other hand, will have diverse periods of effect because of hypoalbuminemia. ' Recurarization may happen, and emergent intubation may be necessary. Perhaps atracurium is the best choice because liver and kidneys may be affected from amyloidosis, which is common in patients with EB, and the metabolism of atracurium is not through the liver or kidneys.1 We did not use atracurium in our patient because we did not plan intubation and, furthermore, surgery would only be on one extremity.

Morphine must be used neither intraoperatively nor postoperatively because it causes histamine release.3 We used tramadol in our patient and did not encounter any problems.

As a result, we can argue that the surgeon, dermatologist, and anesthetist must evaluate these cases in the preoperative period; and regional anesthesia must be the first choice. If general anesthesia is to be applied, preparations for hard intubation conditions with a fiber optic laryngoscope must be made. No adhesive bands must be used in vein fixation of monitoring. Great care must be taken when choosing analgesics, and ketamine must be considered as an alternative. Inhalation anesthesia is a good alternative, and sevoflurane is one of the best agents in this respect. All interventions that may cause interruptions in blood circulation, irritation, or that may affect healing adversely in preoperative, intraoperative, and postoperative periods must be strictly avoided.

References

1. Griffin RP, Mayou BJ: The anaesthetic management of patients with dystrophic epidermolysis bullosa. Anaesthesia 1993; 48: 810-5.

2. Lin AN, Carter DM: Epidermolysis bullosa. Annu Rev Med 1993; 44: 189-99.

3. Hagen R, Langenberg C: Anaesthetic management in patients with epidermolysis bullosa dystrophica. Anaesthesia 1988; 43: 482-5.

4. Smith GB, Shribman AJ: Anaesthesia and severe skin disease. Anaesthesia 1984; 39: 443-55.

5. Lee C, Nagel EL: Anesthetic management of a patient with recessive epidermolysis bullosa dystrophica. Anesthesiology 1975; 43: 122-4.

6. Patch MR, Woodey RD: Spinal anaesthesia in a patient with epidermolysis bullosa dystrophica. Anaesth Intensive Care 2000; 28: 446-8.

7. Smith GB, Shibman AJ: Anaesthesia and severe skin disease. Anaesthesia 1984; 39: 443-55.

8. White JE: Minocycline for dystrophic epidermolysis bullosa. Lancet 1989; 1: 966-9.

9. Kaplan R, Strauch B: Regional anesthesia in a child with epidermolysis bullosa. Anesthesiology 1987; 67: 262-4.

10. Kelly RE, Koff HD, Rothaus KO, Carter DM, Artusio JF: Brachial plexus anesthesia in eight patients with recessive dystrophic epidermolysis bullosa. Aneslh Analg 1987; 66: 1318-20.

11. Sptelman FJ, Mann ES: Subarachnoid and epidural anaesthesia for patients with epidermolysis bullosa. Can Anaesth Soc J 1984; 31: 549-55.

12. Idvall J: Ketamine monoanaesthesia for major surgery in epidermolysis bullosa. Ada Anaesthesiol Scand 1987; 31: 658-60.

13. Loverme SR, Oropollo AT: Ketamine anesthesia in clermolytic bullous dermatosis (epidermolysis bullosa). Anesth Analg 1977; 56: 398-401.

14. Kelly AJ: Epidermolysis bullosa dystrophica: anesthetic management. Anesthesiology 1971; 35: 659-63.

15. James I, Wark H: Airway management during anesthesia in patients with epidermolysis bullosa dystrophica. Anesthesiology 1982; 56: 323-6.

16. Broster T, Placek R, Eggers GWN: Epidermolysis bullosa: anaesthetic management for Cesarean section. Anesth Analg 1987; 66: 341-3.

17. Spargo PM, Smith GB: Epidermolysis bullosa and porphyria. Anaesthesia 1989; 44: 79-80.

18. Tomlinson AA: Recessive dystrophic epidermolysis bullosa: the anaesthetic management of a case for major surgery. Anaesthesia 1983; 38: 485-91.

Guarantor: Maj Mahmut Komurcu, TAF

Contributors: Maj Mahmut Komurcu, TAF*; Maj Ferruh Bugin, TAF[dagger]; Col Ercan Kurt, TAF[dagger]; Col A. Sabri Atesalp, TNF*

* Department of Orthopaedic and Traumatology, Gülhane Military Medical Academy and Faculty, 06018, Ankara, Turkey,

[dagger] Department of Anesthesiology and Reanimation, Gülhane Military Medical Academy and Faculty, 06018, Ankara, Turkey.

This manuscript was received for review in January 2003 and accepted for publication in March 2003.

Reprint & Copyright © by Association of Military Surgeons of U.S., 2004.

Copyright Association of Military Surgeons of the United States Feb 2004
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