Dijagnostikovanje i lecenje Pedzetove bolesti lokalizovane na kicmenom stubu

• Published in: Acta chirurgica Iugoslavica Vol. 57; no. 1; pp. 49 - 55
• Main Authors: Slavkovic, S.M., Vukasinovic, Z., Slavkovic, N.S., Apostolovic, M.D.
• Format: Journal Article
• Language: English
• Published: 2010
• ISSN: 0354-950X; 2406-0887
• DOI: 10.2298/ACI1001049S

Paget disease, localized only on thoracic spine is extremly rare. It is a huge diagnostic problem and an equally big therapy challenge. Course of this disease is slow, it chronically worsens, thus demanding differentiation of various conditions, along with orthopaedic, neurological, radiographic and endocrine evaluation. Paget disease is followed by back pain, progressive weakening of legs and sphincter functions, due to pathological spine fractures and spinal stenosis. The treatment is surgical, in form of decompression and applying biphosphonates. Three patients are presented, all with worsening back pain which lasted several months. Hyperthonia and hyperreflexion of lower extremities were developed significantly. Over the course of time, neurological deficit got worse, almost to the point of spastic parapalegia. All of the patients were male, 50 to 68 years old. They had higher values of alcaline phosphatase and serum calcium than usual. In all cases, bone scintigraphy was positive where the lession of thoracic spine existed. Biopsy suggested hyperparathyroidism, but, as it turned out, wrongly. After surgical treatment, neurological improvement was noticed on all three patients. In case of two, recidives occurred after three and five years respectively, so surgical reinterventions were made. These recidives are consequences of Paget disease progression. Aside from surgical treatment, they were treated by encrinologist. Remission of the disease lasted for seven and twelve years, respectively. Third patient had his condition recognized almost two years after first reception, and, due to big changes on vertebral body and paraplegia, posterior and anterior decompression of spinal canal and stabilization were done simultaneously. Biopsy confirmed Paget disease. During the postoperative course, nearly complete neurological improvement occured almost instantly. Only moments after hospital release, twenty four days after the operation, patient got sick in the hall. Unfortunately, he passed away due to massive lung embolism, even though he received adequate thromboembolic prophylaxion. Monoostotic form of Paget disease localized on vertebral body must be taken and considered seriously, especially when the patients suffer from progressive evolution of neurological deficit, followed by adequate biochemical blood and urine analysis, as well as radiographic signs of pathological fracture or invasive vertebral process. Surgical treatment can lead to significant improvement of clinical findings, but due to recidive, biphosphonate treatment is recommended. Pedzetova bolest lokalizovana samo na grudnim kicmenim prsljenovima retka je pojava. Predstavlja veliki dijagnosticki problem i terapijski izazov. Tok bolesti je usporen, hronicno se pogorsava, tokom vise meseci, pa i godina, a to zahteva detaljnu diferencijaciju raznih stanja, uz klinicku, neurolosku, radiolosku i endokrinolosku procenu. Bolest prate bolovi u ledjnoj kicmi, progresivno slabljenje funkcije nogu i sfinktera, i to usled patoloskih preloma prsljenova i pratece spinalne stenoze na mestu obolelih prsljenova. Lecenje je hirursko, u vidu dekompresije nervnih struktura i primene bifosfonatne medikamentozne terapije. Prikazana su tri bolesnika, sa visemesecnim bolovima u ledjima, koji su s vremenom postajali sve jaci, s razvojem hipertonije i hiperrefleksije nogu uz 'neme' tabane, da bi se s vremenom neuroloski deficit produbljivao skoro do spasticne oduzetosti nogu. Svi su bili muskarci zivotne dobi izmedju 50 i 68 godina. Imali su povisene vrednosti alkalne fosfataze i kalcijuma u serumu. Kod svih je scintigrafija skeleta bila pozitivna, i to na mestu obolelih grudnih prsljenova. Biopsija je budila sumnju na hiperparatireoidizam, ali, ispostavilo se, neosnovano. Posle hirurskih lecenja, doslo je do neuroloskog oporavka kod sva tri bolesnika. Kod dvojice je, posle tri, odnosno pet godina, doslo do recidiva tegoba, to je zahtevalo hirurske reintervencije. Sve su to posledice progresije Pedzetove bolesti. Pored hirurskog lecenja, tretirani su i od strane endokrinologa, uz protokolarne doze bisfosfonata. Remisija bolesti traje sedam, odnosno dvanaest godina. Kod treceg je bolesnika bolest prepoznata skoro dve godine nakon prvog prijema, a zbog opseznosti promena na tri prsljena, kao i zbog oduzetosti nogu, istovremeno je uradjena zadnja i prednja dekompresija kicmenog kanala i stabilizacija tog segmenta. Biopsija je potvrdila Pedzetovu bolest, a u postoperativnom toku doslo je do veoma brzog, skoro potpunog neuroloskog oporavka. Prilikom otpusta iz bolnice, dvadeset cetvrtog dana od operacije, bolesniku je u hodniku naglo pozlilo, te je nazalost preminuo od masivne plucne embolije, i pored adekvatne tromboembolijske zastite. Monoostotski oblik bolesti lokalizovan na prsljenovima mora ozbiljno da se razmatra, posebno kod bolesnika s progresivnim razvojem nervnog deficita, pracenih odgovarajucim biohemijskim analizama krvi, urina, kao i radioloskim znacima patoloskog preloma ili invazivnog procesa na prsljenovima. Hirursko lecenje omogucava znacajno poboljsanje klinickog nalaza, ali zbog recidiva bolesti preporucuje se i lecenje bisfosfonatskim preparatima.