Anesthetic management of a child with xeroderma pigmentosum

Brunner, Thomas; Jöhr, Martin

doi:10.1111/j.1460-9592.2004.01367.x

Cited by 6 publications

(2 citation statements)

References 4 publications

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“…Perioperative management entails meticulous evaluation of airway, neurological abnormalities, shielding the skin from OT lights by using protective clothing, ultraviolet (UV) blocking film [7,8] and avoidance of drugs like paracetamol, halothane, isoflurane and sevoflurane which are known to derange Nucleotide Excision Repair (NER) [1,2,9]. Comet assay [10,11] (alkaline single cell gel electrophoresis) demonstrates peripheral blood lymphocyte DNA damage at 60 mins and at 120 mins of anaesthesia with 1 to 1.5% halothane, isoflurane and sevoflurane.…”

Section: Clinical Pearls In Anesthesia For Xeroderma Pigmentosummentioning

confidence: 99%

Clinical Pearls in Anesthesia for Xeroderma Pigmentosum: A Case Report

Shah¹,

Hariharan²,

Naithani³

et al. 2015

TOATJ

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Xeroderma Pigmentosum (XP) is a rare autosomal recessive (AR) disease characterized by hypersensitivity of the skin to ultra violet (UV) radiation, resulting in a high frequency of UV induced skin tumors and progressive neurological complications at an early age. Through the following case report we emphasize that perioperative management of xeroderma patients entails meticulous evaluation for neurological abnormalities, shielding the skin from OT (operation theatre) lights by using protective clothing, sunscreen and UV blocking film as well as avoidance of genotoxic drugs like volatile anaesthetics and paracetamol. One must be prepared for the possibility of difficult mask ventilation (we used a mask one size larger), difficult intubation and prolonged effect of muscle relaxants (as in our case) due to skin atrophy, neoplasia, joint contracture and neuronal dysfunction.

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Section: Clinical Pearls In Anesthesia For Xeroderma Pigmentosummentioning

confidence: 99%

Clinical Pearls in Anesthesia for Xeroderma Pigmentosum: A Case Report

Shah¹,

Hariharan²,

Naithani³

et al. 2015

TOATJ

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“…Anecdotally, the incidence in North Africa and the Middle East is substantially higher [2]. Little information exists on the optimal anesthetic management of these patients [3-6]. It has been reported that volatile anesthetics have a genotoxic side effect in the cells of patients with XP and may worsen the symptoms [5,7].…”

Section: Introductionmentioning

confidence: 99%

Postoperative neurological aggravation after anesthesia with sevoflurane in a patient with xeroderma pigmentosum: a case report

et al. 2013

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IntroductionXeroderma pigmentosum is a rare autosomal recessive disease that causes changes in skin pigmentation, precancerous lesions and neurological abnormalities. It is a defect in the nucleotide excision repair mechanism. It has been reported that volatile anesthetics has a possible genotoxic side effect and deranged nucleotide excision repair in cells obtained from a patient with xeroderma pigmentosum.We report an unusual case of postoperative neurological aggravation in a patient with xeroderma pigmentosum anesthetized with sevoflurane.Case presentationA 24-year-old African woman, who has had xeroderma pigmentosum since childhood, was admitted to our hospital for a femoral neck fracture. A preoperative physical examination revealed that she had a resting tremor with ataxia. She had cutaneous lesions such as keratosis and hyperpigmentation on her face and both hands. There was no major alteration of cognitive function, muscular strength was maintained and her osteotendinous reflexes were preserved. Surgical fixation was performed under general anesthesia after the failure of spinal anesthesia. All parameters were stable during surgery. When she woke up four hours later, the patient presented with confusion and psychomotor agitation, sharpened reflexes and the Babinski reflex was present. Her postoperative test results and a magnetic resonance imaging scan were unremarkable. It was suggested that sevoflurane had had a probable deleterious effect on the neurological status of this patient.ConclusionThe anesthetizing of a patient with xeroderma pigmentosum is associated with a risk of worsening neurological disorders. At present, there are no clear recommendations to avoid the use of volatile agents in the anesthetic management of patients with xeroderma pigmentosum. More clinical and experimental research is needed to confirm the sensitivity of patients with xeroderma pigmentosum to sevoflurane and other halogenated anesthetics.

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