Turricephaly

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Obesity, Sleep & Internal medicine
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Turricephaly
Synonyms	Oxycephaly, Acrocephaly
Pronounce	N/A
Specialty	N/A
Symptoms	Abnormally shaped skull, Increased intracranial pressure
Complications	Developmental delay, Cognitive impairment
Onset	Congenital
Duration	Lifelong
Types	N/A
Causes	Genetic mutations, Craniosynostosis
Risks	Family history, Certain genetic syndromes
Diagnosis	Physical examination, Imaging studies
Differential diagnosis	Plagiocephaly, Brachycephaly
Prevention	None
Treatment	Surgery, Supportive care
Medication	N/A
Prognosis	Varies
Frequency	Rare
Deaths	N/A

Turricephaly, also known as oxycephaly, is a type of craniosynostosis characterized by a high, tower-like skull. This condition results from the premature fusion of the coronal suture and sometimes other sutures, leading to a vertical elongation of the skull. Turricephaly is a rare cranial deformity and is often associated with other syndromic conditions.

Pathophysiology

In turricephaly, the premature closure of the coronal suture restricts the normal growth of the skull perpendicular to the suture. As a result, the skull compensates by growing in a vertical direction, leading to a tower-like appearance. This condition can also involve the sagittal suture and lambdoid suture, further contributing to the abnormal skull shape.

Clinical Features

Acrocephaly in an infant

Patients with turricephaly often present with a high, steep forehead and a pointed top of the head. The condition can lead to increased intracranial pressure, which may cause symptoms such as headaches, visual disturbances, and developmental delays. In some cases, turricephaly is associated with syndromic craniosynostosis conditions such as Apert syndrome, Crouzon syndrome, and Pfeiffer syndrome.

Diagnosis

The diagnosis of turricephaly is typically made through clinical examination and imaging studies. Computed tomography (CT) scans are particularly useful in assessing the extent of suture fusion and the degree of skull deformity. Magnetic resonance imaging (MRI) may also be used to evaluate any associated brain abnormalities.

Treatment

Historical depiction of acrocephalus

The primary treatment for turricephaly is surgical intervention. The goal of surgery is to correct the skull shape, relieve intracranial pressure, and prevent further complications. Surgical techniques may include cranial vault remodeling and fronto-orbital advancement. Early intervention is crucial to optimize outcomes and minimize the risk of developmental delays.

Prognosis

The prognosis for individuals with turricephaly varies depending on the severity of the condition and the presence of associated syndromes. With timely surgical intervention, many patients experience significant improvement in skull shape and a reduction in symptoms. However, ongoing monitoring and supportive therapies may be necessary to address any developmental or neurological issues.

Related Conditions

Turricephaly is often discussed in the context of other craniosynostosis conditions, such as:

• Plagiocephaly
• Brachycephaly
• Trigonocephaly
• Scaphocephaly

See also

• Craniosynostosis
• Apert syndrome
• Crouzon syndrome
• Pfeiffer syndrome

Oxyhydrocephalus illustration

See Also

• Skull
• Suture (joint)
• Intracranial pressure