Lionitis

What is Lionitis?

Lionitis, also known as craniodiaphysealdysplsia (CDD) is a bone disorder that comes about as a result of excess calcium deposits in the skull. It is a very rare autosomal recessive disorder, which can reduce the life expectancy of a patient.

The calcium build up in the skull reduces the size of the cranial foramina, holes at the base of the skull that make possible the passage of nerves, arteries, veins and other structures. It also reduces the size of the hole in the cervical spinal canal.

CDD reduces the life expectancy of its sufferers drastically and most die while still infants. Lionitis disfigures the facial features of those affected causing their appearance to resemble that of a lion hence its name.

 

 

 

 

 

 

Causes

Lionitis is caused by an autosomal recessive gene. This means that the parents, although unaffected by the disorder, are carriers of the gene that causes it. When they mate, one in four of their children is likely to get both genes and end up with lionitis.

Calcium deposits build up in their skull and cause compression of the foramen, cranial nerves and other contents in the cranium. The exact genes responsible for the disorder are however unknown.

Signs and Symptoms

It is vital to observe and report any symptoms of craniodiaphyseal dysplasia as early as possible. The disorder manifests itself in various signs and symptoms as discussed below:

Dacryocystitis

This is an acute or chronic infection of the lacrimal sac. The lacrimal sac is responsible for reserving overflow tears, draining microbes and draining eye debris. The lacrimal excretory system works by draining tears from the eye all the way into the nasal cavity. Infection or inflammation of the lacrimal sac causes stagnation of tears hence dacryocystitis.

It mostly occurs on the left side. The affected individual experiences pain in the area near the eye. The region becomes red and swollen. In certain cases an abscess forms oozing pus. Fever is also common.

Other symptoms are such as:

• Seizures or epilepsy
• The affected individuals have mental retardation
• The eyes of people with lionitis are usually located far from each other
• Paralysis
• They have different eye sizes
• In severe cases, loss of hearing is common
• Blindness
• Disfigured facial features.

Diagnosis

Diagnosis of this particular disorder is done by a physician’s observation of the symptoms of the patient. These clinical symptoms are caused by a diminutive foramina and hyperostosis.

An X-Ray is then done to determine the region of the skull with abnormal bone density. Excess density is as a result of elevated number of osteoblasts and excess calcium deposits.

The symptoms of lionitis are however similar to those of camurati-engleman disease, craniotubular dysplasias and van buchem’s dysplasia. To avoid a mix up, an analysis of the mutation of gene TGFB1 is done. Early diagnosis of craniodiaphyseal dysplasia is key if the life of the affected individual is to be prolonged.

Treatment

Treatment of lionitis is mostly based on controlling the malformation of bones. Here are some of the treatment options that may be applied in treating lionitis:

• Changing one’s diet to include low calcium foods
• Tracheal intubation by an anesthetist can be done for patients with breathing problems due to blockage of nasal cavity. A flexible tube is placed in the trachea to keep open the airway.
• Patients with papilledema undergo decompression procedures of the optic and orbital nerves
• Administration of calcitriol. Calcitriol also known as Rocaltrol is a Vitamin D analog that regulates absorption of Calcium and its usage in the body.
• Calcitonin therapy – This therapy employs the use of peptide Calcitonin to inhibit the function of osteoblasts responsible for bone formation.
• Administration of Prednisone. This is a drug that acts as an anti-inflammatory agent by preventing the release of substances that cause swelling.
• Decompressing craniectomy procedure is put in place for patients with elevated intracranial pressure. The result is an enlarged anterior and middle fossae.
• Craniofacial remodeling
• Choanal stenosis surgery- This is done to correct choanal atresia, which causes abnormal narrowing of body canals. It is however a short term treatment as recurrence is common. Recurrence is as a result of the rapid reconstitution of osseous optic canals.
• Dacryocystorhinostomy- This is a surgery done for patients with chronic dacryocystitis. It bypasses blockage of the lacrimal excretory system. It is the most common treatment for the condition.
• Compressing the area affected by dacryocystitis with warm cloth several times a day.
• Administering of antibiotics to cure acute dacryocystitis.

Repeated magnetic resonance imaging should be done to evaluate the state of the individual with lionitis. It is also important to keenly watch the health of affected individuals. Early diagnosis and treatment yield better results.

Complications

Lionitis may bring other complications. It is important that a patient sees a doctor for further examination to ensure treatment and management begins early. The below are some of the complications likely to occur if an individual have lionitis:

• Compression of the brain.
• Reduced vascular supply – Hyperostosis (bone overgrowth) reduces the size of the cranial foramina. This means blood supply to and from the brain is limited due to compression of the blood vessels.
• Choanal atresia – This is the blockage of nasal canals by hard tissue or bone from the back of the nose to the throat causing the affected individual to have breathing problems.
• Dysfunction of the nerves of the face.
• Ossicles are deformed because of bone overgrowth. Ossicles in this case are three tiny bones located in the middle of the ear otherwise known as the Malleus, Incus and Stapes. Their purpose is to transmit sound from the air to the cochlea. Deformation of ossicles ultimately leads to loss of hearing.
• Deformity of the face leading to a lion like appearance.
• Highly elevated number of osteoblasts. Osteoblasts are mesenchymal cells that produce matrix responsible for bone formation. A larger than normal number of osteoblasts result in bone overgrowth in the skull.
• Damage of nerve fibers.
• Reduced air spaces of the mastoid process of the temporal bone which allow passage of blood vessels.
• Defects in the posture of the individual with the disorder.
• Reduced air spaces of external auditory canal because of bone overgrowth.
• Papilledema – This is a condition where pressure around the brain causes swelling of the optic nerve.

Reference List

1. Lionitis. www.primehealthchannel.com/craniodiaphyseal-dysplsia.html
2. Signs of Lionitis. www.emedicine.medspace.com/article/1210688-overview
3. Cranial Foramina. www.anatomyzone.com/tutoraials/musculoskeletal/foramina-of-the-skull/
4. Treatment of Lionitis. www.healthh.com/craniodiaphyseal-dysplasia/