ADDUCTED THUMB SYNDROME RECESSIVE FORM & TREATMENT
Definition: What is "Adducted thumb syndrome recessive form"?
Adducted thumb syndrome recessive form, also known as Christian syndrome or Craniostenosis arthrogryposis cleft palate, is a rare disease that affects multiple systems which causes malformations of the palate, thumbs & upper limbs.
Treatment: How to Treat "Adducted thumb syndrome recessive form"?
Should surgery prove to be necessary, it is typically performed between the 4th & the 7th month after birth. The cranial bone is reshaped to allow uninhibited further growth of the head & a normal development of the brain & sensory organs.
This syndrome is related to microcephaly, arthrogryposis & cleft palate & various craniofacial, neurological, respiratory & limb abnormalities, including bone & joint defects of the upper limbs, adducted thumbs, camptodactyly & talipes equinovarus or calcaneovalgus. Patients affected with the disease are considered mentally retarded & most die in childhood. Patients often undergo respiratory difficulties, such as pneumonia & from seizures due to dysmyelination in the white matter. It has been suggested that the Moro reflex (startle reflex in infants) may be a tool in detecting the congenital clapsed thumb early in infancy. The thumb will normally extend because of this reflex.
The skull consists of five thin, curved, bony plates that intersect along lines called sutures. At birth, the bony plates of the skull are not fully joined along the sutures. This allows the baby's head & brain to increase in size & develop after birth. Typically a baby's brain & skull double in size in the first 6 months of life & again by age 2.1 After age 2, the sutures begin to close so that the bones can fuse together. The skull & brain continue to grow throughout early life but at a slower rate. Craniosynostosis is a rare condition in which the skull sutures close & the bony plates of the skull become fused too early in life. This leads in the baby having an abnormally shaped head. Craniosynostosis typically develops while the fetus is still in the mother's womb & often is evident at birth or soon after. It develops in about 1 in every 2,000 births.2 Having less room for the baby's brain to grow & develop can also lead to increased pressure on the brain. If untreated, the increased pressure can result to brain injury. This may cause developmental delays, blindness, seizures & other problems. Fortunately, the majority of children with craniosynostosis do experience these problems. The effects of craniosynostosis depend on how many sutures are affected & whether the problem arises as part of a genetic disorder or other condition.