대한척추외과학회지제 14 권제 2 호 Journal of Korean Spine Surg. Vol. 14, No. 2, pp 96~100, 2007 경추황색인대에발생한칼슘피로인산염이수산기화합물결정침착병 - 증례보고 - 이재철 황석하 김연일 신병준 순천향대학교의과대학정형외과학교실, 척추센터 Calcium Pyrophosphate Dihydrate Crystal Deposition Disease in the Cervical Ligamentum Flavum - A Case Report - Jae Chul Lee, M.D., Seok-Ha Hwang, M.D., Yon-Il Kim, M.D., Byung-Joon Shin, M.D. Department of Orthopedic Surgery, Spine Center, Soonchunhyang University College of Medicine, Seoul, Korea Abstract Calcium pyrophosphate dihydrate deposition disease (CPPD) is an inflammatory arthropathy that is defined by the deposition of CPPD crystals in articular and periarticular structures. The cervical ligamentum flavum is a rare location of CPPD deposition. A 65-year-old woman was admitted with complaints of neck pain and a tingling sensation and numbness below the xiphoid process for 2 months. Magnetic resonance (MR) imaging and computed tomography (CT) revealed compression of the spinal cord due to a nodular calcified mass in or attached to the ligamentum flavum at the C4-5, C5-6, or C6-7 level. The patient underwent a laminectomy at C4-5, C5-6, and C6-7, and resectioning of calcified extradural nodules that impinged on the cervical cord. The operation resulted in a resolution of neck pain and hypoesthesia, except in the feet. Histopathological examination of the excised specimen revealed rectangular CPPD crystals. Here, we report a case of compressive cervical spine due to CPPD deposition disease of the cervical spine and describe the literature relevant to CPPD deposition disease of the cervical spine. Key-Words: Calcium pyrophosphate dihydrate crystal deposition disease, Ligamentum flavum, Cervical spine 서 론 칼슘피로인산염이수산기화합물결정침착병 (Calcium pyrophosphate dihydrate crystal deposition disease) 은관절과관절주변에칼슘피로인산염이수산기화합물 결정이축적되는염증성관절병증으로경추에있는황색인대에서도드물게발생하며, 1978 년 Ellman et al. 1) 가처음보고하였다. 경추황색인대에발생한칼슘피로인산염이수산기화합물결정침착병은황색인대의비후와석회화를일으켜척수의단순한압박에서부터진행성의신경증상 Address reprint requests to Byung-Joon Shin, M.D. Department of Orthopedic Surgery, College of Medicine, Soonchunhyang University 657 Hannam-dong, Youngsan-gu, Seoul, 140-743, Korea Tel: 82-2-709-9056, Fax: 82-2-796-3682, E-mail: schsbj@hosp.sch.ac.kr 본논문의요지는 2006 년도대한정형외과학회에추계학술대회에서포스터로발표되었음. - 96 -
경추부에발생한칼슘피로인산염이수산기화합물결정침착병 이재철외 까지다양한임상증상을나타낼수있으나, 국내에는아직보고가없었다. 저자들은경추황색인대에칼슘피로인산염이수산기화합물결정침착으로인한척수압박을보이는증례를경험하였기에문헌고찰과함께보고하는바이다. 증례보고 65 세여자로내원 2 개월전부터발생한경부통증과검상돌기이하의저린감과감각저하를주소로내원하였다. 과거력상특이소견은없었으며, 경추부의수술을받은일도없었다. 내원당시신경학적검사상제 7 흉추근지배부위의감각저하가있었으나, 운동신경은정상이었고병적반사나심부건반사상이상소견은관찰되지않았다. 경추단순방사선소견에서제 6-7 경추황색인대에결절모양의석회화음영이관찰되고있었으며 (Fig. 1), 경추전산화단층검사에서제 4-5, 5-6, 6-7 경추간황색인대에후방으로비후된석회화결절이보이면서제 6-7 경추에비후된석회화결절에의해경막이압박되는소견이관찰되었다 (Fig. 2). 경추자기공명영상검사상에서도제 4-5, 5-6, 6-7 경추간황색인대후 방에저신호강도의결절이관찰되었다 (Fig. 3). 신경학적이상부위가제 7 흉추부이하여서흉추자기공명영상검사를시행하였으나특이소견은없었다. 제 4-5, 5-6, 6-7 경추간황색인대비후증으로제 5, 7 경추의추궁판부분절제술및제 6 경추의추궁판전절제술을통한후방감압술을시행하였다. 수술소견에서제 6 경추추궁판을제거하고살펴보니백색의분비물이배액되면서석회화된결절과혼재되어있는소견이관찰되고있었으며, 경막과의유착소견은보이지않았다. 병리학적소견에서육아종을동반한전형적인방사형의 CPPD 결정과섬유연골성의기질 (fibrocartilaginous stroma) 이확인되었다 (Fig. 4). 수술후 1 년추시결과양측족부의감각만다소떨어져있을뿐, 그이외부위의감각저하와통증은소실되었다. 고 찰 칼슘피로인산염이수산기화합물결정침착병은관절이나관절주변부의유리질연골 (hyaline cartilage) 과섬유질연골 (fibrocartilage) 에칼슘피로인산염이수산기화합물이침착되어연골석회증 (chondrocalcinosis) 이발생 Fig. 1. Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease in a 65-year-old woman with a history of neck pain and numbness below xiphoid process. Plain radiographs show dense and homogenous radiopaque deposits (arrows indicated) at C6-7 level in AP view (A) and lateral view (B). - 97 -
대한척추외과학회지 Vol. 14, No. 2, 2007 하는염증성관절병증이다. 칼슘피로인산염이수산기화합물은대개무증상인경우가많으나, 일부에서통풍, 류마토이드관절염과유사한염증반응을나타내어가성통풍 (pseudogout) 이라불리기도한다. 칼슘피로인산염이수산기화합물침착이발생하는정확한생화학적 Fig. 2. Computed tomographic scan showing nodular calcified deposits in the ligamentum flavum at C6-7. 기전은알려져있지않으나, 유발인자로혈색소침착증, 갑상선저하증, 부갑상선항진증, 윌슨씨병, 통풍, 류마토이드관절염과같은내분비혹은대사성질환등이있다 2). 또한외상이나수술, 허혈성심질환, 임신, 유전에의해발생한다는보고가있다 3,4). 본증례의환자는내분비질병이나대사성질병은없었고, 경추부의수술을받은일도없었다. 칼슘피로인산염이수산기화합물결정침착병은 1962 년에처음보고되었다 5). 이질병은임상적으로는가성통풍으로인한관절염을보이며, 방사선소견과함께, 활액이나조직검사에서칼슘피로인산염이수산기화합물결정을발견할수있다. 칼슘피로인산염이수산기화합물결정침착병은흔히슬관절, 완관절, 견관절, 족관절이나그주변조직에호발하며 6,7), 척추에발생은드문경우로, 발생위치에따라임상증상이다양하다 4). 경추에발생하는경우에는척수증이발생할수있고, 요추에발생하는경우에는요추의척추관협착증이나, 급성추간판탈출증증상을보일수도있으며, 추간판염이나가성척추전방전위증과유사한증상을보이기도한다. 칼슘피로인산염이수산기화합물결정침착병이경추에발생하는경우는대부분제 2 경추의치아돌기주변에발생하는것으로보고되고있다 2). 본증례의경우척추의호발부인경추부위에발생했으나제 2 경추가아닌제 5, 6, 7 경추에발생했고, 척수압박이있고통증과 Fig. 3. T2-weighted sagittal MR images showing a hypointense mass compressing the cervical cord at C6-7, C5-6, C4-5 levels. Fig. 4. Photomicrography showing multiple CPPD crystals (arrow indicated) in fibrocollagenous stroma with numerous giant cells. (H & E stain; 100) - 98 -
경추부에발생한칼슘피로인산염이수산기화합물결정침착병 이재철외 저린감이있었으나전형적인척수증의증상은나타나지않았다. 척추에서발생한칼슘피로인산염이수산기화합물결정침착병의방사선학적소견은황색인대에인접한척추관뒷면에타원형의석회화된결절이보이거나, 황색인대자체가비후된소견을보이기도한다 9). 자기공명영상에서는황색인대인접부위에서 T1 과 T2 강조영상에서모두저신호강도를보이는타원형모양의종괴를보인다 10). 본증례의경우경추단순방사선소견에서제 6-7 경추황색인대에결절모양으로석회화음영이관찰되고, 경추자기공명영상검사상에서도 T1 과 T2 강조영상에서모두제 4-5, 5-6, 6-7 경추간황색인대후방에저신호강도의결절이관찰되어, 전형적방사선소견을보였다. 조직병리소견에서섬유연골기질 (fibrocartilaginous stroma) 내의칼슘피로인산염이수산기화합물결정이관찰되며, 편광현미경소견상칼슘피로인산염이수산기화합물결정은복굴절된장사방형 (rhomboid) 모양의결정으로나타나는것으로알려져있다 1,3,6,7). 본증례표본의일반염색에서특징적인장사방형결정의모양을보여서바늘모양의통풍결정과병리학적감별진단에는어려움이없었으나, 편광현미경상에서전형적이라알려진약한복굴절이 (weak birefringence) 관찰되지는않았다. Ivorra 등 11) 도칼슘피로인산염이수산기화합물결정침착병에서일반적으로알려진바와달리복굴절이관찰되지않는결정체가대부분임을보고하였다. 경추황색인대에발생한칼슘피로인산염이수산기화합물결정침착병의치료는감압술및석회화된황색인대를모두제거하는것이다 12). 본증례에서는추궁판절제술로충분한감압및결정종괴제거가가능하였으며, 감압과정에서후관절의손상은없었기에추가적인유합술이나고정술은하지않았다. 비스테로이드성진통소염제계열의약물이급성칼슘피로인산염이수산기화합물침착관절병증에서사용되기는하나, 아직까지결정침착을방지하거나결정을용해시키는데유용한약물은보고되어있지않다. 저자들은척수압박의한원인으로흔하지않은경추황색인대에발생한칼슘피로인산염이수산기화합물결정침착병을경험하였기에문헌고찰과함께보고하자한다. 참고문헌 01) Ellman MH, Vazquez T, Ferguson L: Calcium pyrophosphate deposition in ligamentum flavum. Arthritis Rheum 1978; 21: 611-613. 02) Fye KH, Weinstein PR, Donald F: Compressive cervical myelopathy calcium pyrophosphate dihydrate deposition disease: Report of a case and review of the literature. Arch Intern Med 1999; 159: 189-193. 03) Joseph J, McGrath H: How to differentiate crystalinduced arthropathies. Geriatrics 1995; 50: 33-39. 04) Muthukumar N, Karuppaswamy U, Sankarasubbu B: Calcium pyrophosphate dihydrate deposition disease causing thoracic cord compression. Neurosurg 2000; 46: 222-225. 05) Kohn NN, Hughes RE, McCarty DJ, Faires JS: The significance of calcium pyrophosphate crystals in the synovial fluid of arthritic patients, The pseudogout syndrome. Ann Intern Med 1962; 56: 738-745. 06) Fam AG: Calcium pyrophosphate crystal deposition disease and other crystal deposition disease. Curr Opin Rheumatol 1995; 7: 364-368. 07) Halverson PB: Calcium crystal-associated diseases. Curr Opin Rheumatol 1996; 8: 259-261. 08) Maigne JY, Ayral X, Guerin-Surville H: Frequency and size of ossifications in the caudal attachments of the ligamentum flavum of the thoracic spine. Surg Radiol Anat 1992; 14: 119-124. 09) Sato R, Takahashi M, Yamashita Y, et al.: Calcium crystal deposition disease in cervical ligamentum flavum, CT and MRI findings. J Comput Assist Tomogr 1992; 16: 352-355. 10) Sugimura H, Kakitsubata Y: MRI of ossification of the ligamentum flavum. J Comput Assist Tomogr 1992; 16: 73-76. 11) Ivorra J, Rosas J, Pascual E: Most calcium pyrophosphate crystals appear as non-birefringent. Ann Rheum Dis 1999; 58: 582-584. 12) Pakasa NM, Kalengayi RM: Tumoral calcinosis: A clinicopathological study of 111 cases with emphasis on the earliest changes. Histopathol 1997; 31: 18-24. - 99 -
대한척추외과학회지 Vol. 14, No. 2, 2007 국문초록 칼슘피로인산염이수산기화합물결정침착병 (Calcium pyrophosphate dihydrate crystal deposition disease) 은관절과관절주변에칼슘피로인산염이수산기화합물결정이축적되는염증성관절병증으로경추에있는황색인대에서는드물게발생한다. 65세여자가 2개월전부터시작된경부에통증과검상돌기이하에저린감과감각저하를주소로내원하였다. 자기공명영상과전산화단층촬영에서제 4-5, 5-6, 6-7 경추간황색인대에후방에발생한석회화결절로척수가압박되는소견을보이고있었다. 제 5, 6, 7 경추의추궁판절제술을하여후방감압술을시행하고수술후감각저하와통증은양측족부를제외하고호전되었고병리학적소견상황색인대의 CPPD 결정의침착을보였다. 경추황색인대에발생한칼슘피로인산염이수산기화합물결정침착병은드물지만척수압박의한원인이되는질환으로척수신경증을유발할수있다. 저자들은이런칼슘피로인산염이수산기화합물결정침착병을경험했기에이를문헌고찰과함께보고하고자한다. 색인단어 : 칼슘피로인산염이수산기화합물결정침착병, 황색인대, 경추 통신저자 : 신병준서울특별시용산구한남동 657번지순천향대학교의과대학서울병원정형외과학교실, 척추센터 Tel: 82-2-709-9056 Fax: 82-2-796-3682 E-mail: schsbj@hosp.sch.ac.kr - 100 -