6 https://www.phoenixpub.org/journals/index.php/jmhsr Journal of Medicine and Health Sciences Research 2020, 3: 6-8 http://dx.doi.org/10.21839/jmhsr.2020.v3.341 Case Report Giant juvenile ossifying fibroma from diagnosis to management: A rare case Ejaz Ahmad Mokhtar 1 *, Sumit Verma 2 , Shamshad Alam 3 , Qalbi Fatima 4 1 Oral and Maxillofacial Surgery, Post Graduate Institute of Medical Sciences and Research, Chandigarh, India, 2 Oral and Maxillofacial Surgery, Dr. B.R. Ambedkar Institute of Dental Sciences and Hospital, Patna, India, 3 Oral and Maxillofacial Surgery, Teerthankar Mahaveer Dental College and Research Centre, Moradabad, India, 4 Department of Periodontics, Buddha Institute of Dental Sciences And Hospital, Kankarbagh, Patna, India (Received: 10-05-2020; Accepted: 14-08-2020; Published: 01-09-2020) *Corresponding author: Ejaz Ahmad Mokhtar (dr.ejazahmadmokhtar@gmail.com) INTRODUCTION Ossifying fibroma is a benign fibro-osseous lesion that is divided into subtypes depending on the age of onset as conventional ossifying fibroma and juvenile ossifying fibroma (JOF). Juvenile ossifying fibroma is further subdivided into Juvenile psammomatoid ossifying fibroma (JPOF) and juvenile trabecular ossifying fibroma (JTOF) according to histological characteristics (El-mofty, 2002). Age of onset of juvenile ossifying fibroma is generally younger than 15 years. Unlike conventional ossifying fibroma JOF is characterized by aggressive growth and causing damage to cortical bone, eye nose, and even involving cerebrum. The imaging characteristics of JOF show expansile, well defined, spherical outline with radiolucent to radio-opaque image separated from surrounding normal bone. The management of JOF remains controversial. According to literature recurrence rate after surgery is around 30-58% (Olentino et al., 2013). Treatment can be controversial or radical (Abuzinada and Alyamani, 2010). Radical surgery is advocated for aggressive lesion having high recurrence rate. (El-mofty, 2002; Smith et al., 2009; Han et al., 2016) while others advocate conservative treatment (Leimola- Virtanen et al., 2001; Abuzinada and Alyamani, 2010). Case presentation: A 23-year-old female patient reported to our department with a chief complaint of facial deformity secondary to painless swelling on the right side of face since last 15 years and epiphora and stuffy nose. No diplopia was present. She was previously operated multiple times in the years 2005, 2006, and 2008 but met with recurrence every time. The swelling was around 10x15 cm in size occupying right side maxilla, zygoma, and even caused orbital dystopia. Intra orally the swelling resulted in obliteration of buccal vestibule and expansion of buccal and palatal vestibule. (Figures 1 and 2) Computed tomography was performed which shows spherical shaped lesion occupying right side maxilla, zygoma, and invading right side of lateral orbital wall. The lesion was crossing the midline and encroaching the nasal region. The lesion was mixed radiolucent and radio-opaque. (Figure 3) Incisional biopsy of the lesion was performed which was consistent with previous biopsy report of ossifying fibroma. No malignant transformation was present. Due to the huge size of the lesion and multiple recurrences resection of lesion and reconstruction with patient-specific implant was planned. 3 D model of the patient along with customized patient-specific implant was made (Figure 4). The Patient was taken under general anesthesia and intraoral vestibular incision was. The entire mass was meticulously enucleated in multiple fragments. The mass was present in multiple small compartments in the zygoma and orbit region (Figures 5 and 6). Removing lesion in the palatal region created palatal fistula is accidentally due to loss of palatal bone. No reconstruction was required. After 2 years of follow up, no recurrence was noted. Facial deformity, ocular dystopia, and epiphora to a great extent is resolved. DISCUSSION JAOF is benign odontogenic pediatric tumor which is characterized by aggressive behavior and high recurrence rate. It has two histologic variants JTOF and JPOF. One is characterized by small uniform spherical ossicles resembling psammoma bodies (psammomatoid juvenile ossifying fibroma). The other is distinguished by trabeculae of fibrillary ABSTRACT Juvenile aggressive ossifying fibroma is a rare, benign tumor of the maxillofacial region that is aggressive and recurrent leading to multiple surgeries causing maxillofacial defect and deformities. The management of patients with ossifying fibroma is controversial. The aim was to report a case of huge ossifying fibroma involving maxilla, zygoma, and orbit in a young female with a duration of 15 years. The patient was operated multiple times but the lesion recurred. Patient-specific implant was fabricated to replace the defect area which would be created after surgery but with meticulous intraoperative decision the high morbid surgery was changed to conservative surgery. No recurrence was seen after two year follow up. The patient aesthetic has also improved. In conclusion it can be said in huge benign ossifying fibroma morbid surgery should be avoided. Key words: Juvenile aggressive ossifying fibroma, maxillofacial, conservative surgery