Dr Theresa Raju, Dr. Suma Balan(pediatric rheumatology), Dr C Jayakumar. Amrita Institute of Medical Sciences
Twelve year old female child presented with recurrent episodes of swelling and pain in the left submandibular region since the last 2 years.
The symptoms usually last for 1 week and was on and off and was associated with pain. The pain was more during night and also had pain in the left ear. The symptoms usually improves with oral antibiotics . The child used to get doxycycline for 7 days. But last time when she got the episode , she was treated with Amoxyclav.. The child did not respond to the medication and diclofenac sodium was also added.
No history of fever, any other joint pain, abdominal pain, vomiting, diarrhea
Her birth ,development immunisation histiry were normal
The child initially presented 2 years back with pain and swelling in the left mandible of 6 months duration for which she had consulted a local dental specialist and had undergone pulpectomy .
As symptoms recurred she was referred to AIMS .
At PET CT was done which
showed diffuse abnormal FDG uptake with sclerosis and widened cortex of left coronoid process, ramus and angle of mandible- known site of osteomyelitis
There is no family history of any rheumatological conditions.
On examination :vitals were stable.
No pallor, icterus, cyanosis, clubbing, lymphadenopathy or edema
Swelling was present in the submandibular area extending to tip of pinna.
Systemic examination was normal
Initial labs did showed normal counts with ESR of 41mm/hr and negative CRP. RFT/LFT/Serum electrolytes did were normal
MRI Whole body with head and neck additional sequence was done which showed hyperintensities in the rami and condyles of left mandible along with soft tissue swelling and marrow edema suggestive of chronic osteomyelitis of the mandible.
She underwent excision of the osseous mass under general anesthesia
Oral histopathology report biopsy showed features consistent with clinical features of Garres osteomyelitis
Garres osteomyelitis myelitis
It is a type of chronic osteomyelitis mainly affecting children and young adults. It is associated with low grade infection which may be due to dental caries.
The body of the mandible may show irregular lucent or opaque changes with subperiosteal opaque layering along inferior border. It is a chronic osteomyelitis with subperiosteal bone and collagen deposition. There is no suppuration or sinus formation.
Considering the recurrent nature of the disease, the child was advised for
Inj Pamidronate and pre medicated with Injection Avil and paractemol. She tolerated the drug very well and was hemodynamically stable . The child is currently on monthly pamidronate injection
DISCUSSION: Mandibular CRMO( Chronic Recurrent Multifocal Osteomyelitis):
It is a relapsing inflammatory disease and is considered a type of seronegative spondyloarthropathy characterized periods of exacebrations and remissions over many years.
It is characterized inflammatory process presenting with findings similar to infective osteomyelitis , however no infectious source is identifiable. It si most often characterized insidious onset of low grade fever , local swelling and bone pain
Epidemology: Rare estimated frequency of 1:1,000,000
Primarly affects children. Median age of 10 years with female/male ratio of 5:1
Sites: Mainly affects the metaphyses of long bones. When involving the jaw, it predominantly involves the mandible and the prevelance of mandibular involvement is 2-8%.
Clinical features: Characterized periods of intermittent exacebrations and improvement over a period of years. Some patients have associated recurrent skin lesions that closely parallel the exacerbations of the bone lesion.
Diagnosis: Collectively based on clinical, radiological and longitudinal findings. CRMO is a diagnosis of exclusion since the clinical presentation and laboratory findings are not disease specific and vary between affected individuals.
Radiology findings: Diffuse bone radioopacity is the prominent finding in patients with mandibular involvement. While radiology governs the quite phase of CRMO, areas of mottled cortical and cancellous bone osteolysis prevail during recurrence. Small osteolytic lacunae that contain lymphocytes and plasma cells are potential sites of exacerbation leading to cortical erosions and periosteal reaction.
Whole body imaging techniques including MRI and Tc 99m labelled methylene bone scintigraphy represent novel and valuable diagnostic tools. MRI is especially sensitive during early stages since it can detect bone edema even before erosion or sclerosis become apparent.
Treatment: NSAIDs are commonly used as first line treatment of CRMO in less to moderately severe cases. NSAIDs provide pain control in most cases and most likely alter the disease course since prostaglandins are centrally involved in osteoclast activation and bone remodelling.
Other options include corticosteroids, sulfasalazine, methotrexate, anti TNF agents or bisphosphonates(usually pamidronate)
Surgical decortication has been recorded in refractory cases but in young, growing patients the associated risk must be considered since the results from surgical intervention have been inconsistent.