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

Abstract

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.

Dexmedetomidine as an anaesthetic adjunct for total intravenous anaesthesia in patients with xeroderma pigmentosum.

“…3 In patients with XP, general anaesthesia using volatile agents is preferably avoided, as the inhalational agents may worsen the symptoms of XP. [4][5][6] So in our patients, total intravenous anaesthesia (TIVA) was provided using dexmedetomidine and propofol infusion. Dexmedetomidine reduces the requirement of propofol for induction and both of them can facilitate the intubationwithout the use of a muscle relaxant 7 and such patients are sensitive to muscle relaxants due to the neuronal dysfunction.…”

Anesthesia Management of a Patient with Xeroderma Pigmentosum: Case Report

“…Postoperatif dönemde ilaveten kognitif fonksiyonlarda bozulma, konfüzyon, ajitasyon ve piramidal sendrom gelişebilmektedir. 6 Miyazaki ve ark.nın aldıkları ve 23 yaşında KP'li olduğunu bildikleri hastanın daha önceki ameliyatında volatil ajanların kullanımı nedeni ile anestezi sonrası mevcut olan nörolojik semptomlarının kötüleştiği bildirilmiştir.…”

“…1 Yine femur boyun kırığı nedeni ile opere edilen 24 yaşındaki Afrikalı kadın hastanın operasyon öncesi mevcut nörolojik bulgularının arttığı bildirilmiştir. 6 Bu hastada anestezi idamesinde sevofluran kullanılmış ve nörolojik semptomların kötüleşmesinin sebebi olarak düşünülmüştür. Hastamızda, entübasyondan sonra anestezinin sürdürülmesinde propofol infüzyon ve remifentanil infüzyon uygulandı.…”

Dexmedetomidine and ketamine combination for a patient with xeroderma pigmentosa

“…Though the anesthetic implications are not clearly known, several case reports have shown worsening neurological conditions including delayed awakening and prolonged neuromuscular blockade [1][2][3]. We report a patient of XP with delayed recovery during previous exposure to anesthesia who was managed successfully with a combination of intravenous dexmedetomidine and ketamine.…”

“…In a report by Fjouji et al [2], a 24-year-old woman with XP underwent general anesthesia for fracture femur surgery after the failure of spinal anesthesia. She had confusion, agitation, and sharpened reflexes after extubation (MRI normal).…”