Anesthesia Management in An Adult Patient with Desbuquois Syndrome

Anesthesia Management in An Adult Patient with Desbuquois Syndrome

Desbuquois syndrome (DS) is a rare disease that can be accompanied by a difficult airway. In the literature, there are usually case reports about the mortal forms in childhood. We wanted to share our anesthesia experience in an adult patient with DS who underwent orthopedic surgery. We used videolaryngoscopy for intubation with coronavirus precautions. Adult patients with DS can be managed safely with adequate difficult airway preparation.

___

  • [1] LeMerrer M, Young ID, Stanescu V et al. Desbuquois syndrome. Eur J Pediatr. 1991; 150(11): 793-6.
  • [2] Lloyd AR, Ragosta KG, Bryke CR et al. Desbuquois syndrome in three sisters with significantly different lengths of survival. Am J of Med Genet Part A. 2006;140(11): 1253-5.
  • [3] Szmuk P, Matuszczeak M, Carlson RF et al. Use of CobraPLATM for airway management in a neonate with Desbuquois syndrome. Case report and anesthetic implications. Pediatric Anesthesia. 2005;15(7): 602-5.
  • [4] Desbuquo G, Grenier B, Michel J et al. Nanisme chondrodystrophique avec ossification anarchique et polymalformations chez deux soeurs. Arch Fr Pediatrie. 1966; 23(5): 573-87.
  • [5] Hall BD. Lethality in Desbuquois dysplasia: three new cases. Pediatric radiology. 2001; 31(1): 43-7.
  • [6] Guo L, Elcioglu NH, Iida A et al. Novel and recurrent XYLT1 mutations in two Turkish families with Desbuquois dysplasia, type 2. Journal of human genetics. 2017; 62(3):447-51.
  • [7] Takata Y, Lenke LG, Kelly MP. Posterior vertebral column resection for rigid proximal thoracic kyphoscoliosis with broken growing rods in a patient with Desbuquois dysplasia. Spine deformity. 2020; 8(1): 135-8.
Acta Medica-Cover
  • ISSN: 2147-9488
  • Yayın Aralığı: Yılda 4 Sayı
  • Başlangıç: 2012
  • Yayıncı: HACETTEPE ÜNİVERSİTESİ
Sayıdaki Diğer Makaleler

A Review of Glutamate and Its Receptors: Their Roles in Brain Physiology and Pathology

Sevgi UĞUR MUTLUAY, Hülya KARATAŞ

Evaluation of Prognostic Markers in Cancer-associated Fibroblast Based Sub-groups of Colorectal Cancer

Seçil DEMİRKOL CANLI

Monitoring Serum Lipid Profile and Liver Transaminase Levels During Isotretinoin Therapy

Sibel ERSOY EVANS, Gonca ELÇİN, Duygu GÜLSEREN, Ecem BOSTAN, Neslihan AKDOĞAN, Başak YALICI ARMAĞAN, Sibel DOĞAN, Ayşen KARADUMAN

Persistent Extensive Microcalcifications After Neoadjuvant Chemotherapy: Benign or Malignant?

Gamze DURHAN

Non-Wilms’ Renal Tumors In Childhood

Saniye EKİNCİ, Arbay ÖZDEN ÇİFTÇİ, Feridun Cahit TANYEL, Diclehan ORHAN, Canan AKYÜZ, İbrahim KARNAK, Burak ARDIÇLI

COVID-19 Pandemic Experience in a Tertiary Care Center in Turkey: What have We Learned?

Yasemin ÖZSÜREKÇİ, Özlem TEKŞAM, Ümit Murat ŞAHİNER, Elif Nursel ÖZMERT, Mehmet CEYHAN

A Case of Pneumatosis Intestinalis Associated with Sjogren’s Syndrome

Masashi OHE, Seiichi HORİNAKA, Haruki SHİDA, Ken FURUYA

Can Syndecan-1 Be Used As A Biomarker In Alzheimer’s Disease?

Rana Tuna DOĞRUL, Cagatay ÇAVUŞOĞLU, Gozde Sengul AYÇİÇEK, Cemile ÖZSÜREKÇİ, Hatice ÇALIŞKAN, Hacer DOĞAN VARAN, Zeliha GÜNNÜR DİKMEN, Mustafa CANKURTARAN, Burcu Balam DOĞU

Anesthesia Management in An Adult Patient with Desbuquois Syndrome

Murat TÜMER, Hazar KÖKTEN, Aysun ANKAY YILBAŞ, Fatma SARICAOĞLU

From Thesis to Publication - Analysis of 2580 theses in the field of Anesthesiology and Reanimation

İlke KÜPELİ, Merve YAZICI KARA, Ayşe KAYHAN