Newborn with pathologic fracture of the tibia: osteofibrous dysplasia

Öz

Dear Editor Osteofibrous dysplasia is a very rare tumor like developmental fibroosseous condition of the long bones 1 As far as we know a total of eigth newborn cases with osteofibrous dysplasia have been reported in the literature three of which had pathologic fracture 2 3 4 5 6 In this article a patient with osteofibrous dysplasia who was found to have pathologic fracture at the second hour after birth and who was diagnosed at the earliest period according to the literature There was no pathology in the medical history of the male infant who was born by cesarean section at the 37 5 7th gestational week because of footling breech presentation The birth weight was 3305 g the height was 52 cm and the head circumference was 35 cm When swelling echymosis pathologic movement crepitation and tenderness in the right leg were found two hours after birth in the second examination two way tibia fibula graphy was taken Picture 1 When pathologic fracture was found on direct graphy bone biopsy was obtained at the 24th hour after birth and sent to pathology A fibro osseous lesion containing ovoid fusiform cells and new bone formation with a trabecular structure were observed in the biopsy specimen Picture 2a 2b A marked osteoblastic array rimming was observed in the midst of the newly forming bone trabecules Picture 2b The laboratory results were found to be within normal limits Fibrous dysplasia congenital syphilis histiocytosis X congenital pseudoarthrosis neurofibromatosis were considered in the differential diagnosis With the existing clinical radiologic and histopathological findings a diagnosis of osteofibrous dysplasia was made The disease has been mostly found in children below the age of 10 years 1 7 It is a condition of growing bones It has been found that it occurs a little more frequently in males It has been reported in advanced ages and rarely in the newborn period The fact that the disease occurs rarely in advanced ages indicates that the disease regresses spontaneously or is maintained regular with the actualization of bone maturation 1 8 9 It has been reported that genetic effect may be involved in the etiology 10 Generally the disease is manifested with tibia extension and curling of the tibia anteriorly or anterolaterally with a mild or moderate degree 8 The lesion is generally painless A part of the cases are determined with pathologic fracture as a result of minor trauma 1 7 Our patient is the earliest diagnosed case in the newborn period and the fourth case with pathologic fracture in the newborn period in the literature The reason of minor trauma leading to pathologic fracture was probably pulling the foot during cesarean section performed because of footling breech presentation Recognition of swelling and ecchymosis for the first time at the second hour supports this Eccentric intracortical osteolysis is the rule in radiologic examination The outer surface of the cortex can be enlarged moderately or severely or thinned to a high degree because of the tumor which shows extension The tumor has a bullous appearance which consists of a single cyst composed of confluent lytic areas or multiple cysts It is typically located in the diaphysis and may extend to the metaphysis The tumor matrix usually has a ground glass appearance If pathologic fracture is present fracture findings may be observed and if pathologic fracture is not present flexion defect may be observed frequently 4 7 8 Although radiological findings are not patognomonic osteofibrosis substantially suggests dysplasia The periostium is always protected well histopathologically and the cortex may be as thin as parshment Microscopic examination reveals immature bone tissue surrounded by ostaoblasts in the fibroblastic stroma This finding is named as ldquo;zonal architectural pattern rdquo; The fibrous part contains small and scattered fusiform cells 2 7 8 11 There is no generally accepted treatment method Generally it has been reported that the lesion may regress spontaneously and may be followed up with ldquo;conservative rdquo; treatment especially in children below the age of 15 years 4 7 8 12 Surgical treatment is usually recommended in cases of excessive growth and pathologic fracture Surgical treatment is not recommended in children below the age of one 4 7 8 12 Regional recurrence has been found in a portion of the patients in whom subperiostal resection was performed 1 8 In one study extraperiostal excision was recommended in all subjects with a diagnosis of osteofibrous dysplasia since adamantinoma was found in recurrent biopsy in the follow up 11 The pathologic fracture of our patient was stabilized by pediatric orthopaedics with plaster splint Surgical excision or curettage was not performed in accordance with the literature information The patient is still being followed up by pediatric orthopaedics Conclusively osteofibrous dysplasia should be considered in presence of lesions located in the anterior side of the tibial diaphysis and in presence of congenital intracortical osteolytic tibia lesions though it is a very rare condition Ad shy;dress for Cor shy;res shy;pon shy;den shy;ce: Serdar Al MD Medipol University Medical Faculty Department of Pediatrics Istanbul Turkey E mail: drserdaral@gmail com Re shy;cei shy;ved: 06 21 2012 Ac shy;cep shy;ted: 08 07 2012 References 1 Campanacci M Osteofibrous dysplasia of long bones a new clinical entity Ital J Orthop Traumatol 1976; 2 2 : 221 237 2 Anderson MJ Townsend DR Johnston JO Bohay DR Osteofibrous dysplasia in the newborn J Bone Surg Am 1993; 75 2 : 265 267 3 Zamzam MM Congenital osteofibrous dysplasia of the tibia associated with pseudoarthrosis of the ipsilateral fibula Saudi Med J 2008; 29 10 : 1507 1509 4 Çetinkaya M Özkan H Köksal N Sarısözen B Yazıcı Z Neonatal osteofibrous dysplasia associated with pathological tibia fracture: a case report and review of the literature J Pediatr Orthop B 2012; 21 2 : 183 186 5 Castellote A Garc iacute;a Pe ntilde;a P Lucaya J Lorenzo J Osteofibrous dysplasia A report of two cases Skeletal Radiol 1988; 17 7 : 483 488 6 Smith NM Byard RW Foster B Morris L Clark B Bourne AJ Congenital ossifying fibroma osteofibrous dysplasia of the tibia a case report Pediatr Radiol 1991; 21 6 : 449 451 7 Park YK Unni KK McLeod RA Pritchard DJ Osteofibrous dysplasia: clinicopathologic study of 80 cases Hum Pathol 1993; 24 12 : 1339 1347 8 Campanacci M Laus M Osteofibrous dysplasia of the tibia and fibula J Bone Joint Surg Am 1981; 63 3 : 367 375 9 Goto T Kojima T Iijima T Yokokura S Kawano H Yamamoto A Matsuda K Osteofibrous dysplasia of the ulna J Orthop Sci 2001; 6 6 : 608 611 10 Hunter AG Jarvis J Osteofibrous dysplasia: two affected male sibs and an unrelated girl with bilateral involvement Am J Med Genet 2002; 112 1 : 79 85 11 Lee RS Weitzel S Eastwood DM et al Osteofibrous dysplasia of the tibia Is there a need for a radical surgical approach? J Bone Joint Surg Br 2006; 88 5 : 658 664 12 Resnick DS Diagnosis of bone and joint disorders 3rd ed Philadelphia: WB Saunders 1995; 1001

Anahtar Kelimeler

• -

Patolojik tibiya kırığı olan yenidoğan: osteofibroz displazi

Öz

Osteofibroz displazi, uzun kemiklerin çok nadir görülen tümör benzeri gelişimsel fibroosöz durumudur (1). Bildiğimiz kadarıyla literatürde yenidoğan döneminde toplam sekiz, patolojik kırığı olan üç osteofibroz displazi olgusu vardır (2-6). Bu yazıda doğumdan sonra ikinci saatinde patolojik kırığı saptanmış ve literatüre göre en erken tanı alan, osteofibroz displazili olgumuz sunulmaktadır. Ayak gelişi olması nedeniyle 37 5/7 gestasyon haftasında sezaryen ile doğan erkek bebeğin öyküsünde özellik yoktu. Doğum ağırlığı 3305 g, boyu 52 cm, baş çevresi 35 cm idi. Doğumundan iki saat sonraki ikinci kontrolünde sağ bacakta şişlik, ekimoz, patolojik hareket, krepitasyon ve hassasiyet saptanması üzerine iki yönlü tibiya-fibula grafisi çekildi (Resim 1). Direkt grafide patolojik kırık saptanması üzerine doğumun 24. saatinde kemik biyopsisi alınıp patolojiye gönderildi. Biyopsi parçasında ovoid-iğsi hücreler içeren fibro-osöz lezyon, trabeküler yapıya sahip yeni kemik oluşumu görüldü (Resim 2a2b). Yeni oluşan kemik trabeküllerinin arasında belirgin osteoblastik dizilim vardı (Resim 2b). Laboratuvar sonuçları normal sınırlarda bulundu. Ayırıcı tanıda adamantinoma, fibröz displazi, doğuştan sifiliz, histiyositoz X, doğuştan psödoartroz (nörofibromatoz) düşünüldü. Mevcut klinik, radyolojik ve histopatolojik bulgularla olguya osteofibröz displazi tanısı kondu.

Anahtar Kelimeler

• Patolojik
• tibiya kırığı
• yenidoğan

Kaynakça

1. Campanacci M. Osteofibrous dysplasia of long bones a new clinical entity. Ital J Orthop Traumatol 1976; 2(2): 221-37.
2. Anderson MJ, Townsend DR, Johnston JO, Bohay DR. Osteofibrous dysplasia in the newborn. J Bone Surg Am 1993; 75(2): 265-7.
3. Zamzam MM. Congenital osteofibrous dysplasia of the tibia, associated with pseudoarthrosis of the ipsilateral fibula. Saudi Med J 2008; 29(10): 1507-9.
4. Çetinkaya M, Özkan H, Köksal N, Sarısözen B, Yazıcı Z Neonatal osteofibrous dysplasia associated with pathological tibia fracture: a case report and review of the literature. J Pediatr Orthop B 2012; 21(2): 183Castellote A, García-Peña P, Lucaya J, Lorenzo J. Osteofibrous dysplasia. A report of two cases. Skeletal Radiol 1988; 17(7): 483-8.
5. Smith NM, Byard RW, Foster B, Morris L, Clark B, Bourne AJ. Congenital ossifying fibroma (osteofibrous dysplasia) of the tibia-a case report. Pediatr Radiol 1991; 21(6): 449-51.
6. Park YK, Unni KK, McLeod RA, Pritchard DJ. Osteofibrous dysplasia: clinicopathologic study of 80 cases. Hum Pathol 1993; 24(12): 1339-47. Campanacci M, Laus M. Osteofibrous dysplasia of the tibia and fibula. J Bone Joint Surg Am 1981; 63(3): 367-75.
7. Goto T, Kojima T, Iijima T, et al. Osteofibrous dysplasia of the ulna. J Orthop Sci 2001; 6(6): 608-11.
8. Hunter AG, Jarvis J. Osteofibrous dysplasia: two affected male sibs and an unrelated girl with bilateral involvement. Am J Med Genet 2002; 112(1): 79-85.

9. Lee RS, Weitzel S, Eastwood DM, et al. Osteofibrous dysplasia of the tibia. Is there a need for a radical surgical approach? J Bone Joint Surg Br 2006; 88(5): 658-64.
10. Resnick DS. Diagnosis of bone and joint disorders. 3rd ed. Philadelphia: WB Saunders, 1995; 1001. 77 Türk Ped Arfl 2013; 48: 76-7 Turk Arch Ped 2013; 48: 76-7 Al ve ark.
11. Patolojik tibiya kırığı olan yenidoğan: osteofibroz displazi / Newborn with pathologic fracture of the tibia: osteofibrous dysplasia