SUMMER 2010

Extreme Photophobia with Overbite Greater than Thyromental Distance in a Patient for Ambulatory Eye Surgery

Gwendolyn L Boyd MD, Hayden Hughes MD, Myra Aultman CRNA, and Michael Callahan MD
University of Alabama at Birmingham, Callahan Eye Foundation Hospital
UAB Departments of Anesthesiology and Ophthalmology

Abstract

A 34 y/o male with xeroderma pigmentosa with extreme photophobia and painful eyes, presented for an evaluation of an amniotic membrane placed previously for a symblepharon (fibrous tissue connecting the conjunctiva to the eyelid). He had had numerous skin cancers removed since age 8. Currently, his protuberant overbite was greater than his thyromental distance, compounded by neck contractures, near absent nares and inability to open his mouth more than two finger breadths. During two recent MAC anesthetics airway obstruction occurred. One was converted to general with difficult insertion of LMA while the other was completed with ketamine and propofol with chin lift.

What is Xeroderma Pigmentosa?1-3
Persons with xeroderma pigmentosa are unable to repair ultraviolet ray induced damage to their cutaneous cellular DNA. Normally, the body repairs this damage. Consequently, the skin gets very thin and patches of varying color (splotchy pigmentation) appear. Xeroderma pigmentosa also causes telangiectasias, spidery blood vessels in the skin as well as skin cancer. Skin cancer often occurs before the child is 5 years old.

Symptoms include:

A. Sunburn that does not heal after minimal sun exposure, beginning in early infancy. Marked freckling occurs before the age of two years

B. Blistering also after minimal sun exposure
C. Spider-like blood vessels under the skin
D. Patches of discolored skin getting worse with time
E. Crusting of the skin
F. Scaling of the skin
G. Oozing raw skin surfaces
H. Photophobia

Exams and Tests

A. A family history of xeroderma pigmentosa. XP is inherited in an autosomal recessive manner. The parents of an individual with XP are obligate carriers of a mutation in one of the nine genes associated with XP.

B. The eye exam may show:

1. Corneal clouding
2. Keratitis
3. Lid tumors
4. Blepharitis Atrophy of the skin of the lids results in ectropion, entropion, or in severe cases, complete loss of the lids.

C. Prenatal evaluation may include

1. Amniocentesis
2. Chorionic villous sampling
3. Culture of amniotic cells

D. Postnatal testing may include:

1. Culture of skin fibroblasts
2. Skin biopsy

Treatment

A. Children with this condition require total protection from sunlight. Even the light coming through windows and fluorescent bulbs is dangerous. When the person must go in the sun, protective clothing should be worn. High protection (SPF 30 or greater) sunscreen and very dark, UV-protected glasses should be worn.

B. Recently reconstruction of the DNA repair-deficient xeroderma pigmentosum skin has been possible using a T4N5 liposome DNA repair lotion (DimericineT®). The occurrence of the most common form of skin cancer, basal cell carcinoma, has been decreased by 30% and actinic keratosis, a pre-cancerous form of skin cancer, was reduced by 68% with the use of this lotion.

C. Treat small, premalignant skin lesions such as actinic keratoses with topical 5-fluorouracil or freeze with liquid nitrogen; treat larger areas with dermatome shaving or dermabrasion to remove damaged superficial epidermal layers; treat skin neoplasms with electrodesiccation and curettage, surgical excision, or chemosurgery; use high-dose oral isotretinoin to prevent new neoplasms; treat neoplasms of the eyelids, conjunctiva, and cornea surgically; corneal transplantation for those with severe keratitis and corneal opacity.

Course and Prognosis           

Despite the above measures and also as a result of multiple removal of skin cancers, disfigurement occurs. Prognosis is poor and most persons with this condition die early in adulthood of skin cancer.

Case Report

This 34 year old male began having lesions surgically removed from around his eyes at the age of two years. His first skin cancers were removed at age 8. His sister also had XP and died several years prior to this admission from a sarcoma extending into her brainstem. The patient’s only other documented medical problems were depression and insomnia for which he took Zoloft, trazedone and Ambien.

The current series of surgical procedures began a few months previously for an EUA, RLL symblepharon repair, and placement of amniotic membrane. Following administration of propofol, the “airway was difficult to maintain” and then was “lost.” A #3 LMA insertion was difficult in this 6 foot, 160 pound male and was only successful after 30 mg succinylcholine had been given. A month later, he had returned for the left symblepharon repair and corneal scraping with amniotic membrane, as well as a bandage contact lens on the right eye. This time a MAC anesthetic was chosen. Again, airway obstruction occurred, necessitating a constant chin lift throughout the procedure. In neither of these two anesthetics did the oxygen sat fall below 90%.

Three months later the patient returned for corneal scraping and placement of a bandage contact lens on the left eye. His photophobia had worsened to the point that he wore both dark glasses and held a baseball cap to cover his entire face. Examination of the airway despite dimming the lights was exceedingly painful. A marked overbite was present approximately the same length as his thryomental distance. He was unable to open his mouth more than two fingerbreadths with a Mallampati of 4. In addition, neck extension was extremely poor. His near absent nares appeared friable.

Anesthetic Management

The patient was transported to the dimmed operating room with his cap over his face and dark glasses. The planned anesthetic was a general with LMA. Propofol 200 mg was administered IV in contrast to the 70 mg in the first mentioned anesthetic. When airway obstruction occurred, a nasal airway was inserted without relief of the obstruction. A #3 LMA was then inserted without difficulty and sevoflurane with nitrous oxide administered for the fifteen minute surgery. Upon awakening, the patient’s pain was relieved with morphine and the lights redimmed. Recovery was uneventful. Two months later, an EUA of both eyes, when photophobia and severe eye pain had been significantly ameliorated, was done with midazolam 2mg, four ten mg doses of propofol, and three ten mg doses of ketamine.

ASA Difficult Airway Algorithm

Step 1 of the ASA Difficult Airway Algorithm4 assesses the patient’s airway. In this patient, both ventilation and intubation were likely to be difficult compounded by the patient’s severe pain limiting his ability to cooperate. Anterior chamber eye surgery does not require paralysis and therefore LMA, unless otherwise, contraindicated can provide a good airway obviating the need for tracheal intubation while maintaining spontaneous ventilation. Due to the lack of tissue in the anterior neck with clearly visible landmarks for cricothyrotomy for either jet ventilation or tracheostomy, neither of these alternative airways were likely to be difficult to perform. Supplies for both alternatives were immediately available as well as fiberoptic bronchoscopy.

References

1. Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKena WG. Clinical Oncology. 3rd ed. Orlando, Fl: Churchill Livingstone; 2004.

2. Kraemer, KH (kraemerk@nih.gov) Xeroderma Pigmentosum (last updated 4-22-2008) www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene♂part=xp

3. Kumar V, Abbas AK, Fausto N. Robbins and Cotran Pathologic Basis of Disease. 7th ed. St. Louis, Mo: WB Saunders; 2005.

4. Practice guidelines for management of the difficult airway Anesthesiology 2003; 98:1269-1277.

The Xeroderma Pigmentosum Society is available as a support group. Its website is www.xps.org. Information regarding the disease as well as services such as on-line discussion groups for parents and summer camps are available.

The presentation of this case report was approved by the Institutional Review Board of the University of Alabama at Birmingham. Patient photo used with permission of patient and University of Alabama IRB.

Callahan Eye Foundation Hospital

University of Alabama
School of Nursing

UAB Departments of Anesthesiology and Ophthalmology