SEVERE RENAL OSTEODYSTROPHY AND HUNGRY BONE SYNDROME

 

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SEVERE RENAL OSTEODYSTROPHY AND HUNGRY BONE SYNDROME

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Aarthi
Muthukumaran
Aarthi Muthukumaran aarthi.muthukumaran@nhs.net Imperial College Healthcare NHS Renal Medicine London United Kingdom *
Aimee Dimarco aimee.dimarco@nhs.net Imperial College Healthcare NHS Endocrine Surgery London United Kingdom -
Susan Kirby susan.kirby1@nhs.net Imperial College Healthcare NHS Anaesthesia London United Kingdom -
Oliviero Crescenzi oliviero.crescenzi@nhs.net Imperial College Healthcare NHS Anaesthesia London United Kingdom -
Adish Talwadker adish.talwadker@nhs.net Imperial College Healthcare NHS Radiology London United Kingdom -
Hatty Douthwaite hatty.douthwaite@nhs.net Imperial College Healthcare NHS Renal Medicine London United Kingdom -
 
 
 
 
 
 
 
 
 

Tertiary hyperparathyroidism is due to excessive secretion of parathyroid hormones by hypertrophied parathyroid glands, usually after long-standing secondary hyperparathyroidism. When severe, it can rarely cause Brown tumours or Osteitis fibrosa cystica, which are non-malignant erosive bony lesions characterised by excessive bone resorption due to osteoclasts and remodelling by fibrous tissue. This is a rare case report of a patient with extensive Brown tumours requiring a challenging parathyroidectomy and the subsequent management of hungry bone syndrome.

A 23 year old woman on haemodialysis for 6 years had presented with progressively increasing swelling in her palate since 2 years, associated with difficulty in swallowing and speech. On examination, she was cachexic and had skeletal deformities in the form of kyphoscoliosis, pectus carinatum, splaying of teeth and short stature. 

Brown tumour of palate

Blood investigations revealed hyperphosphatemia, high PTH and ALP levels indicating rapid bone turnover disease causing severe renal osteodystrophy. Whilst she was being worked up for parathyroidectomy, she had sustained a pathological fracture of her left humerus after a trivial injury and was hospitalised. She underwent skeletal screening in the form of CT imaging of skull, facial bones, neck, chest, abdomen & pelvis and whole spine, which showed classical findings of osteolytic bony lesions amd periosteal fibrosis. 

Salt and pepper skull

Brown tumour of maxilla and mandible

Narrowing of airway due to brown tumours of palate

Dorsal scoliosis

Dorsal kyphosis

Rugger jersey spine

Pathological humerus fracture

Brown tumour of pubic bone

In preparation for parathyroidectomy, the patient was loaded with alfacalcidol. She had bleeding from the oral mucosal tumours, putting her at high risk of aspiration and compromise of her precarious airways. Due to severe narrowing at the level of oro-nasopharynx with extensive Brown tumours in maxilla and mandible, both oral and nasal intubations were not feasible and a diligent anaesthetic airway management was drawn. She underwent subtotal parathyroidectomy (3/4 parathyroids removed) with awake mini-tracheostomy. As anticipated, she developed hungry bone syndrome in the post-operative period, necessitating intravenous calcium infusion. Hungry bone syndrome occurs due to an acute shift in bone metabolism after the removal of excess parathyroid hormone, causing increased osteoblastic activity and rapid consumption of calcium by the bones, resulting in severe hypocalcemia. 

Serum parathyroid hormone level

Serum alkaline phosphatase level

Serum calcium level

Serum phosphate level

Successful and timely management of severe life-threatening tertiary hyperparathyroidism in this patient was made possible due to prompt diagnosis, seamless interprofessional collaboration between multi-disciplinary teams and meticulous pre and post-operative assessments & monitoring.

Kewords