Anesthetic Approach in a Patient With Ehler-Danlos Syndrome

Türkel S. İ., Sağıroğlu G., Sarı M.

Asian Society of Paediatric Anesthesiologists Conference, İstanbul, Türkiye, 14 - 16 Ekim 2022, ss.1, (Özet Bildiri)

  • Yayın Türü: Bildiri / Özet Bildiri
  • Basıldığı Şehir: İstanbul
  • Basıldığı Ülke: Türkiye
  • Sayfa Sayıları: ss.1
  • Dokuz Eylül Üniversitesi Adresli: Evet

Özet

ANESTHETIC APPROACH IN A PATIENT WITH EHLERS-DANLOS SYNDROME

 

PURPOSE/AIM

Ehlers-Danlos syndrome (EDS) is a group of genetically transmitted connective tissue disorders which are characterized by the synthesis and structure of the procollagen and collagen. The features of this syndrome has very wide spectrum such as temporomandibular joint (TMJ) dysfunction, atlantoaxial instability,joint dyslocation due to hypermobility,hyperelastic skin,aneursym and dissection of big vessels.

CASE

Our patient was a 13-year-old girl,interned for pectus excavatum-Nuss procedure. We did our preanesthesic evaluation and physical examination for EDS symptoms. She has hyperelasticity and hypermobility in her fingers,skin elasticity,has pectus carinatum and other examination findings were normal. Although her cardiovascular examination were normal we asked for preoperative pediatric cardiology consultation for EDS’s cardiac involvement. Echocardiogram were performed and showed us she has minimal mitral valve prolapse. We used 0.1 mg/kg midazolam intravenosus for premedication. Anesthesia was induced with 1 mg/kg lidocaine,1 mcg/kg fentanyl,2 mg/kg propofol and 0.6 mg/kg rocuronium,followed by intubation. We gave patient’s head in neutral position and used videolaryngoscope for atlantoaxial instability. During the operation patient’s vital signs were within normal limits. Surgery was performed without complication. Extubation was successful and uneventful.

DISCUSSION

Ehler-Danlos syndrome is classified into 6 subtypes and each subtype has its own characteristic features. There are also rare types which has undefinied yet. In our anesthesic management we considered that potential problems such as positional injuries,accidental dyslocation of TMJ and during intubation neck flexibility or excessive extansion may cause atlantoaxial joint injury,due to high inspiration positive pressures during ventilation may cause pneumothorax. In conclusion,complications are different for each patient and we should be managed this patient based problems with a balanced anesthesia and special care for joint mobility and pay attention for every subtype’s own characteristic.