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Leontiasis

Leonine Facies Due to Leprosy


Presentation

  • […] characteristic radiologic features of uremic leontiasis ossea. 6 Case Report We present case report of two patients (12-year-old girl and 29- year-old male) presenting with jaw masses.[slideplayer.com]
  • In the present article, we describe the case of an uncooperative female patient with a brown tumor, involving the left maxillary sinus and massive thickening of the cranial vault and facial bones, secondary to severe secondary hyperparathyroidism during[ncbi.nlm.nih.gov]
  • In conclusion, we present an unusual presentation of PFD with diffuse involvement of the cranio-facio-vertebral skeleton with leontiasis ossea and a post traumatic cervical cord contusion secondary to hyperextension injury of the cord in a compromised[doi.org]
  • Entities such as cherubism usually present at 2-3 years of age and spontaneously resolve after puberty. 8 Paget’s disease typically presents later than the current patient’s age and has a male predilection.[appliedradiology.com]
Turkish
  • Look up "leontiasis" at Merriam-Webster Look up "leontiasis" at dictionary.com In other languages: Spanish French Italian Portuguese Romanian German Dutch Swedish Russian Polish Czech Greek Turkish Chinese Japanese Korean Arabic Advertisements Word of[wordreference.com]
Snoring
  • Our patient had a relatively asymptomatic leontiasis ossea of long duration with only mild obstructive nasal symptoms, snoring and cosmetic disfigurement.[doi.org]
Mouth Breathing
  • Physical examinations revealed: toggled speech (nasal speech), mouth breathing and facial change, saddle nose, nares widening, increased interdental space and mandibular enlargement.[journalrip.com]
  • Although he had facial deformities since early childhood, he professed only to cosmetic embarassment, nasal blockage, mouth breathing, mild snoring and progressing bowing of his shins. There were no other affected family members.[doi.org]
Widely Spaced Teeth
  • Physical exam revealed marked non-tender maxillary and mandibular hypertrophy with nasal flattening, wide-spaced teeth and severe palatal changes.[file.scirp.org]
Visual Impairment
  • Leontiasis ossea can be associated with progressive proptosis, visual impairment or nasal obstruction.[doi.org]
Osteopenia
  • Radiologically, the maxillofacial changes of renal osteodystrophy have previously been broken down into three separate patterns. 3 Osteitis fibrosa is a constellation of osteolytic lesions, cortical thinning, and coarsened trabeculation. 4,5 Microlunar osteopenia[appliedradiology.com]
  • Findings in secondary (and tertiary) hyperparathyroidism are numerous: sub periosteal bone resorption, osteopenia, osteosclerosis e.g. rugger-jersey spine, soft tissue calcification, generalized increased uptake on Tc-99m pertechnetate bone scan, superior[file.scirp.org]
Joint Subluxation
  • subluxations or dislocations and compressive symptoms [ 1 ].[doi.org]
Hand Muscle Weakness
  • He was discharged home on the 10 th day with residual distal hand muscle weakness and grade 4 muscle power in his legs.[doi.org]
Neck Pain
  • He was able to turn over, but immediately noticed some neck pain and weakness of all four limbs. He was just able to lift his arms and move his legs from side to side.[doi.org]
Saber Shin
  • He had broad forearms, sausage like fingers and bowing of both legs with saber shins (Figure 2 ). The nasal bridge was flat and elevated due to a soft tissue thickening (Figure 1 & 2 ). No café au lait spots were observed.[doi.org]
Facial Swelling
  • The second case represents a patient with hyperparathyroïdism, showing an unusual facial swelling as only physical symptom of the disease.[ncbi.nlm.nih.gov]

Workup

Rugger-Jersey Spine
  • Findings in secondary (and tertiary) hyperparathyroidism are numerous: sub periosteal bone resorption, osteopenia, osteosclerosis e.g. rugger-jersey spine, soft tissue calcification, generalized increased uptake on Tc-99m pertechnetate bone scan, superior[file.scirp.org]
Soft Tissue Calcification
  • Findings in secondary (and tertiary) hyperparathyroidism are numerous: sub periosteal bone resorption, osteopenia, osteosclerosis e.g. rugger-jersey spine, soft tissue calcification, generalized increased uptake on Tc-99m pertechnetate bone scan, superior[file.scirp.org]
Ventricular Hypertrophy
  • Electro-cardiogram : The conclusion from the examination was one of left ventricular hypertrophy. X-ray of skull: A.P. and Lateral views showed diffuse hyperostosis of bone with loss of differentiation between outer and inner tables [Figure - 2].[ijo.in]
  • Electro-cardiogram : The conclusion from the examination was one of left ventricular hypertrophy. X-ray of skull: A.P. and Lateral views showed diffuse hyperostosis of bone with loss of differentiation between outer and inner tables [Figure - 2] .[ijo.in]

Treatment

  • […] case of significant amelioration of massive modification of the facial appearance of a 30-year-old uremic Chinese woman with severe skeletal deformities who underwent total parathyroidectomy with a forearm autograft concurrently with effective drug treatment[ncbi.nlm.nih.gov]
  • […] the case of an uncooperative female patient with a brown tumor, involving the left maxillary sinus and massive thickening of the cranial vault and facial bones, secondary to severe secondary hyperparathyroidism during 8 years of regular hemodialysis treatment[ncbi.nlm.nih.gov]
  • At the time, she refused surgical treatment. In the meantime, she lost around 16 cm of height and developed pronounced kyphoscoliosis.[ncbi.nlm.nih.gov]
  • A medical practitioner should always be consulted for diagnosis and treatment of all medical conditions.[hospitalhelp.co.uk]
  • There is no treatment other than exposing the overgrown bone, and chipping away pieces, or excising entirely where possible.[en.wikipedia.org]

Prognosis

  • Treatment usually entails parathyroidectomy with monitoring of serum calcium, phosphate, and vitamin D levels while on appropriate replacement (calcium/vitamin D) or binding (phosphate) pharmacotherapy. 4 The prognosis is usually favorable.[appliedradiology.com]
  • More often, most lesions can be managed through staged procedures with overall very favorable outcomes and good long term prognosis.[skullbaseinstitute.com]

Etiology

  • Clinical-Medical Image: Paget disease of the bone is a chronic disorder of unknown etiology that typically results in enlarged, deformed bones due to abnormal and excessive bone remodeling.[imagejournals.org]
  • Indian J Ophthalmol 1969;17:166-70 Introduction The term leontiasis ossea was first coined by Virchow [6] in 1896 to describe the leonine appearance of the facial deformity in this rare condition of unknown etiology.[ijo.in]
  • However, nutritional and secondary hyperparathyroidism due to uremia has been suggested as major etiologies for the disease [5] . After parathyroidectomy, facial changes can be stabilized or improve mildly.[file.scirp.org]

Pathophysiology

  • Pathophysiological origin of the disease is not clearly understood. However, nutritional and secondary hyperparathyroidism due to uremia has been suggested as major etiologies for the disease [5] .[file.scirp.org]

Prevention

  • Early recognition andconfident diagnosisof ULO is essential to prevent progression to facial disfigurement andavoid invasive procedures.[posterng.netkey.at]
  • During hemodialysis patients often develop numerous comorbid conditions that may interfere with the existing disease and prevent further treatment, as was the reported case.[ncbi.nlm.nih.gov]
  • Ultimately, early recognition of incipient uremic leontiasis ossea clinically and on imaging is essential to prevent progression to severe disfigurement that can result from prolonged untreated secondary hyperparathyroidism [12] .[file.scirp.org]

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