Osteopetrosis of the mandible masquerading as tubercular osteomyelitis

Abstract

Osteopetrosis is a rare congenital (autosomal type) disorder of the skeletal system. Several variants have been described in the literature with grossly variant prognosis and clinical behaviour. Several reports of intractable osteomyelitis of the jaw bones secondary to osteopetrosis, particularly the mandible, have been published widely. However, there is no published report of the complete mandible sequestrating de novo, in the literature. An overview of this spectrum of sclerotic bone disease, its presentation in the oro-facial region, the diagnostic challenge it poses and the management dilemma it offers to the maxillofacial surgeon is discussed and a protocol for managing this disease effectively is presented. A clinical illustration of the complexities of management of osteopetrosis-induced osteomyelitis of jaw bones is demonstrated with a very rare case in which the entire mandible had sequestrated.

Osteomyelitis Possibly Caused by Exfoliation of Primary Teeth in a Patient with Osteopetrosis

“…However, osteomyelitis subsequently appeared in other sites in a sequential manner. There are few published reports concerning the successful treatment of osteomyelitis in children [ 20 , 21 , 22 , 23 , 24 ]. Surgical intervention with drug therapy (i.e., systemic administration of antibacterial agents) is often used to manage osteomyelitis [ 6 , 22 , 23 , 24 ].…”

“…There are few published reports concerning the successful treatment of osteomyelitis in children [ 20 , 21 , 22 , 23 , 24 ]. Surgical intervention with drug therapy (i.e., systemic administration of antibacterial agents) is often used to manage osteomyelitis [ 6 , 22 , 23 , 24 ]. Local sequestrectomy of the affected region is sometimes performed [ 12 , 16 , 20 ].…”

“…Local sequestrectomy of the affected region is sometimes performed [ 12 , 16 , 20 ]. However, osteomyelitis recurs because poor bone blood flow hinders antibiotic penetration of affected tissue, and it is difficult to distinguish sclerotic from necrotic bone during sequestrum removal [ 22 ]. In patients with multiple instances of osteomyelitis, corticotomy is sometimes performed [ 17 , 22 ].…”

Osteopetrosis in a six-month-old infant

“…Due to the rare nature of the disorder, there have been no large studies evaluating the morphology of bone marrow core biopsies in osteopetrosis patients; however, several smaller case series and case reports of osteopetrosis describe bone marrow biopsies that typically demonstrate increased bone density with new woven bone formation at varying stages of ossification, as well as significant narrowing of bone marrow spaces with both marked hypocellularity and suppressed trilineage hematopoiesis [5][6][7][8][9][10][11][12]. In addition, there is abnormal bone formation with thickened, disorganized bony trabeculae [13][14][15]. Areas of patchy reticulin fibrosis can also be seen [16].…”

Oral and Radiographic Findings of a Patient with Osteopetrosis: A Rare Case Report

“…Osteomyelitis may occur as a complication to odontogenic infections caused by tooth extraction or pulpal necrosis in almost 10% of the cases (Ambika et al, 2010). Osteomyelitis secondary to osteopetrosis is more common in the mandible than in the maxilla due to thin cortical bone and a rich collateral blood supply to the maxilla (Sharma et al, 2013;Garcı´a et al, 2013). The leading cause of the increased rate of infection is thought to be a lack of adequate bone vasculature (Ambika et al, 2010).…”