Leontiasis Ossia and the Sphinx of Giza

Introduction

A 31-year-old female with chronic renal failure, on dialysis noted progressive enlargement of the head and facial region over a period of one year. The patient was also severely osteoporotic and had multiple fractures in the past and was now wheelchair bound.

Her facial enlargement progressed to the extent of having breathing and swallowing difficulty. Helical CT scan of the head and neck region was done with multiplanar reconstructions (Figure 1).

The CT scan showed ground glass expansion of the cranial vault and that of the maxillofacial skeleton including the mandible with Interspersed cystic lucencies (Figure 2 and 3). There was significant expansion of the alveolar, palatal and that of the mandibular region, leading to oral and oro-pharyngeal airway compromise. These changes had led to prognathic appearance.

There were similar changes in the cervical spine and rest of the axial skeleton including ribs and the pelvic bones (Figure 4). High resolution ultrasound and nuclear scintigraphy showed presence of parathyroid adenomas (Figure 5). Her lab parameters showed serum calcium of 8.2 mg/dl, PTH of 1349 pg/ml, serum albumin 3g/dl and serum phosphorus 5.3 pg/ml. She was also anaemic with hemoglobin of 9 g/dl. The patient underwent percutaneous parathyroid adenoma ablation procedure and is presently being followed up.

Patient continues to be on dialysis with facial dysmorphology, feeding and breathing issues and is receiving supportive management.

Diagnosis of renal osteodystrophy and secondary leontiasis ossia was made on review of clinical details and typical CT scan observations.

The CT scan showed ground glass expansion of the cranial vault and that of the maxillofacial skeleton, including the mandible with Interspersed cystic lucencies (Figure 2 and 3). There was significant expansion of the alveolar, palatal and that of the mandibular region, leading to oral and oro-pharyngeal airway compromise. These changes had led to prognathic appearance.

There were similar changes seen in the cervical spine and rest of the axial skeleton including ribs and the pelvic bones (Figure 4).

High resolution ultrasound and nuclear scintigraphy showed presence of parathyroid adenomas (Figure 5). Her lab parameters showed serum calcium of 8.2 mg/dl, PTH of 1349 pg/ml, serum albumin 3g/dl and Figure 4: Radiograph of the pelvis with both the hips showing generalized changes of renal osteodystrophy with pathological fractures. Figure 5(a): High resolution ultrasound showing a large oval hypoechoic parathyroid adenoma, posterior to the left lobe of thyroid gland. serum phosphorus 5.3pg/ml. She was also anaemic with hemoglobin of 9g/dl. The patient underwent percutaneous parathyroid adenoma ablation procedure and is presently being followed up.

Patient continues to be on dialysis with facial dysmorphology, feeding and breathing issues and is receiving supportive management. Diagnosis of renal osteodystrophy and secondary leontiasis ossia was made on review of clinical details and typical CT scan observations.

Discussion

Leontiasis ossia is a rare medical condition manifesting with overgrowth of facial and cranial bones.¹ The hypertrophy of the facial skeleton resembles a lion’s face. Virchow had used the term leontiasis ossia to the conditions of hyperostosis affecting the facial bones in one of his classic lectures (1862-1863) at the University of Berlin.² The famous Great Sphinx of Giza is hypothesized to have suffered from leontiasis ossia.³ This is observed in variety of conditions which can cause hypertrophy of the craniofacial region. These include renal osteodystrophy, Paget’s disease and fibrous dysplasia.⁴ Leontiasis ossia is rarest manifestation of renal osteodystrophy. It is manifested by marked expansion of the maxillofacial skeleton and can be associated with obliteration of the maxillary sinuses and dental deformities.⁵ The inner and outer cortical table of the bones is obscured by ground glass matrix. Histopathology, it shows vascularized cellular connective tissue in the expanded bones.

Chronic renal disease leads to derangement of calcium and phosphorus metabolism with overproduction of the parathyroid hormone. There is disruption of the bone formation and resorption process with multiple skeletal changes.⁶

The skeletal manifestations that are typically noted in renal osteodystrophy are osteitis fibrosa and salt and pepper skull. In Osteitis fibrosa, cystic changes, cortical thickening and coarsened trabeculae are seen. In salt and pepper skull, multiple tiny latencies are seen in the skull vault.

Leontiasis ossia is a rare manifestation of renal osteodystrophy. Early recognition of this condition is important to prevent facial disfigurement and associated morbidities. The treatment modalities include renal transplant, parathyroidectomy and plastic reconstructive surgery.

Conclusion

Renal osteodystrophy can have varied skeletal manifestations from the salt and pepper skull morphology to osteitis fibrosa cystica. All these skeletal changes lead to significant clinical morbidity with patient’s symptoms ranging from fatigue, bone pain and frequent pathological fractures. Leontiasis ossia is the rare presentation where predominant facial deformation can lead to swallowing and breathing difficulties. Early recognition of secondary hyperparathyroidism in the renal insufficiency patients should be sought and a multidisciplinary approach for the management is needed. What has four legs than two and then three asked the sphinx again. Man who crawls as a baby, then walks on two Legs and Needs cane in old age said Oedipus. The Sphinx jumped from the Cliff to its death.

Conflict of interest

Nil.

Financial support

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