Türk Nöroşirürji Dergisi 2015 , Vol 25 , Num 3
Intraoperative Black Disc: A Case of Ochronotic Spondylopathy
Ali GENÇ1, Arzu DİNÇ2, Gökalp SİLAV3, İlhan ELMACI3
1Medipol Üniversitesi, Esenler SUM Hastanesi, Nöroşirürji Kliniği, İstanbul, Türkiye
2Medipol Üniversitesi, Esenler SUM Hastanesi, Fizik Tedavi ve Rehabilitasyon Kliniği, İstanbul, Türkiye
3Medipol Üniversitesi, Tıp Fakültesi, Nöroşirürji Anabilim Dalı, İstanbul, Türkiye
Ochronosis, a.k.a. alkaptonuria, is a rare congenital metabolic disorder with autosomal recessive inheritance. The disease is characterized by a deficiency in the enzyme homogentisic acid (HGA) oxidase, a tyrosine catabolism enzyme, which leads to homogentisic acid accumulation in the connective tissues. Build-up of homogentisic acid polymers leads to dark coloration of urine (alkaptonuria), dark pigmentation in connective tissues (ochronosis), joint cartilage degeneration, osteoporosis as well as visceral organ deposition with pathomorphological consequences. These degenerative processes may also lead to intervertebral disc herniation. Ochronosis is rarely encountered in neurosurgical practice. Therefore, without a known previous diagnosis, the typical black pigmentation of intervertebral discs encountered during spinal surgery may be confusing to the surgeon and the disease may be missed. Here, we report on a patient with ochronotic spondylosis who complained of radicular pain in the right arm as well as neck and back pain. The patient was operated for a cervical intervertebral disc herniation. The radiological evaluation of the ochronotic spine and the intraoperative findings of the ochronotic intervertebral disc are also described. Anahtar Kelimeler : Ochronosis, Spondylopathy, Cervical, Discectomy, Homogentisic acid