Neonatal osteofibrous dysplasia associated with pathological tibia fracture: a case report and review of the literature Merih C ¸ etinkaya a,d , Hilal O ¨ zkan a,d , Nilgu ¨n Ko ¨ ksal a,d , Bartu Sarıso ¨ zen b,e and Zeynep Yazıcı c Osteofibrous dysplasia is a rare and benign disease that originates from the tibia or fibula. The symptoms of osteofibrous dysplasia include painless enlargement and bowing of the tibia and pain occurring in the presence of pathological fracture. Herein a male infant who was admitted with redness and swelling on the right leg and diagnosed as pathological tibia fracture due to left tibia osteofibrous dysplasia on the third day of life was presented. To our knowledge, this is the earliest presentation of osteofibrous dysplasia with a pathological fracture in a neonate. Therefore, it must be suspected in neonatal bone fractures. J Pediatr Orthop B 21:183–186  c 2012 Wolters Kluwer Health | Lippincott Williams & Wilkins. Journal of Pediatric Orthopaedics B 2012, 21:183–186 Keywords: newborn, osteofibrous dysplasia, pathological fracture, tibia Departments of a Neonatology, b Orthopaedic Surgery, c Radiology, d Department of Pediatrics, Division of Neonatology and e Department of Orthopaedic Surgery, Uludag University, Faculty of Medicine, Bursa, Turkey Correspondence to Merih C ¸ etinkaya, MD, PhD, Uludag ˘U ¨ niversitesi Tıp Faku ¨ ltesi, C ¸ ocuk Sag ˘ lıg ˘ ı ve Hastalıkları ABD, Go ¨ ru ¨kle, Bursa 16059, Turkey Tel: + 00 90 224 2950447; fax: + 00 90 224 4428143; e-mail: drmerih@yahoo.com Introduction Osteofibrous dysplasia, which is also known as congenital osteitis fibrosa and ossifying fibroma of long bones, is a rare disease that usually originates from the cortex of either tibia or fibula in children. Although it is a benign condition, it can be mistaken as a malignant lesion. The lesion is usually seen only in one tibia and occasionally in the first decade of life; however, it is rarely seen in neonates [1,2]. The symptoms usually consist of painless enlargement and bowing of the tibia but pain can be observed in the presence of fracture [3]. The diagnosis is primarily based on radiographical features, which includes eccentric intracortical osteolysis and anterior bowing of the tibia [3]. To our knowledge, there are only a few neonates with osteofibrous dysplasia in the literature and in only one of them had fracture been diagnosed at 20 days of life [1,2,4–6]. Here, we report a male infant who was admitted with redness and swelling on the right leg and diagnosed as pathological tibia fracture due to left tibia osteofibrous dysplasia on the third day of life. Case report A full-term male infant was born by spontaneous vaginal delivery to a 24-year-old primigravida mother in a hospital. His birth weight was 3210 g. The mother did not have any nutritional or health problems and she did not have any toxic exposure during pregnancy. The infant’s parents were nonconsanguineous. The parents noticed restlessness and crying on moving the infant’s right leg. Noticing redness and swelling on the infant’s right leg on the second day of life, the parents took the infant to a medical center, where a cystic lesion and fracture on his right tibia were detected on antero- posterior radiography; the infant was then referred to our hospital. The infant was hospitalized in our Neonatal Care Unit. On admission, he weighed 3275 g, and his body tempera- ture was 38.21C. Physical examination showed poor activity and dehydration. There was a 4  3 cm fusiform, painful mass, which showed swelling and redness over the proximal one-third of the anterior aspect of the right tibia. The foot and ankle were normal on inspection and palpation. The ipsilateral knee and hip had a full range of movement. There were no abnormal physical findings in other systems. Laboratory investigations showed a slight leukocytosis with a white cell count of 14800/mm 3 , and 80% of them were polymorphonuclear leukocytes. Serum biochemistry, urine, and cerebrospinal fluid analyses were normal. C-reactive protein was negative. The anteroposterior and lateral radiographies of his leg showed an extensive lytic lesion leading to thinning of the cortex in the diaphyseal region of the proximal one- third of tibia with a pathological fracture (Figs. 1 and 2). The computed tomography scan showed ground-glass appearance of the lytic lesion (Fig. 3). He was given cefotaxim, amikacin, and clindamycin intravenously for neonatal sepsis. After consultation with pediatric ortho- pedic surgeons, no surgery was performed. A plaster was applied to his leg. The patient was discharged with plaster-cast immobilization. The patient is now able to walk without limping. He has been followed-up by the Departments of Orthopedics and Pediatrics. His last follow-up radiograph is shown in Fig. 4. Case report 183 1060-152X  c 2012 Wolters Kluwer Health | Lippincott Williams & Wilkins DOI: 10.1097/BPB.0b013e328344fd41 Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.