Diprosopia

Diprosopia

Article
Ear, Nose, & Throat (ENT)
Brain & Nerve
+8
Contributed byKrish Tangella MD, MBAApr 10, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Craniofacial Duplication
  • Diprosopus

What is Diprosopia? (Definition/Background Information)

  • Diprosopia, also known as Diprosopus, is a rare, life-threatening developmental defect during embryogenesis, and a subtype of conjoined twins, characterized by partial or complete duplication of the facial structures on a single head, neck, trunk and body 
  • The disorder may be associated with congenital anomalies involving the central nervous, cardiovascular, gastrointestinal and respiratory systems 
  • Cleft lip and palate have been reported in rare cases

(Source: Diprosopus; Orphanet, National Institute of Health and Medical Research (INSERM), Paris.)

Who gets Diprosopia? (Age and Sex Distribution)

  • Diprosopia is a rare congenital disorder, with less than 50 cases reported in the medical literature since 1864
  • The symptoms are apparent in-utero and at birth
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Diprosopia? (Predisposing Factors)

  • Currently, no risk factors have been clearly identified for Diprosopia

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Diprosopia? (Etiology)

The exact underlying cause of Diprosopia is currently unknown.

What are the Signs and Symptoms of Diprosopia?

The signs and symptoms of Diprosopia may include:

  • Partial or complete duplication of the facial structures on a single head, neck, trunk and body
  • Congenital anomalies involving the central nervous, cardiovascular, gastrointestinal and respiratory systems
  • Cleft lip
  • Cleft palate

(Source: Diprosopus; Orphanet, National Institute of Health and Medical Research (INSERM), Paris.)

How is Diprosopia Diagnosed?

Diprosopia is diagnosed on the basis of the following information:

  • Complete physical examination
  • Thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests
  • Imaging studies
  • Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Diprosopia?

The complications of Diprosopia may include:

  • Breathing problems
  • Trouble with feeding, digestion
  • Heart malfunction

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Diprosopia Treated?

Diprosopia is a congenital disorder, and treatment is usually given to manage the signs and symptoms and any complication that develops.

How can Diprosopia be Prevented?

  • Diprosopia may not be preventable, since the cause of this congenital disorder is unknown
  • Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Diprosopia? (Outcomes/Resolutions)

  • The prognosis of Diprosopia is poor, with very few affected individuals surviving after birth
  • Typically, the prognosis can only be assessed on a case-by-case basis

Additional and Relevant Useful Information for Diprosopia:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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