Anaesthetic Dilemma in Paediatric Patients with Xeroderma Pigmentosum: A Case Report .


Vol 5 | Issue 3 | Sep-Dec 2019 | page: 16-17  | Shilpi Sethi, Prashant Gupta

Authors: Shilpi Sethi [1], Prashant Gupta [1]

Address of Correspondence
1 Department of anaesthesia, Sitapur Eye Hospital and Regional Institute Of Ophthalmology, Sitapur, Uttarpradesh, India.

Dr. Shilpi Sethi,
Sitapur Eye Hospital and Regional Institute Of Ophthalmology, Sitapur, Uttarpradesh, India.

Introduction: Xeroderma Pigmentosum (XP) although a rare disease, characterized by deficiency in Nucleotide Excision Repair (NER) mechanism, making one susceptible to UV induced hypersensitivity of skin at an early age. Thereby children present with neoplasm of skin, mucous membrane and ocular surfaces often requiring surgeries.
Case Presentation: We report a 10 year old female patient with Xeroderma pigmentosum who presented with scleral nodule requiring excision under general anaesthesia. Inhalational anaesthetics, paracetamol and opioids may cause genotoxic side effects in patients with defects in Nucleotide excision repair. Airway is often compromised due to buccal cavity scarring. We adopted a Total intravenous anaesthesia technique (TIVA) technique using propofol, dexmedetomidine, I gel and medial canthal peribulbar block for analgesia.
Conclusion: The aim of this case report is to highlight the anaesthetic implications like anticipation of difficult venous access, meticulous airway evaluation and judicious use of total intravenous anaesthesia. The psychosocial aspect of Xeroderma pigmentosum should also be dealt with in educating the parents in avoiding sun exposure, using UV creams and need for repeated surgeries.
Keywords: Xeroderma Pigmentosum, Nucleotide Excision Repair, TIVA.


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How to Cite this Article:Sethi S, Gupta P | Anaesthetic Dilemma in Paediatric Patients with Xeroderma
Pigmentosum: A Case Report | Journal of Anaesthesia and Critical Care Case Reports | Sep – Dec 2019; 5(3): 16-17


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