INFORMATION ON INIENCEPHALY

Iniencephaly is a type of Cephalic disorder. It is a rare neural tube defect that combines extreme retroflexion of the head with severe defects of the spine. The affected infant tends to be short, with a disproportionately large head. In Iniencephaly, the defect results in extreme retroflexion of the head combined with severe distortion of the spine. In most infants the neck is absent and the skin of the face is connected directly to the skin of the chest, while the scalp is directly connected to the skin of the back. The prognosis for those with Iniencephaly is extremely poor. Newborns with Iniencephaly seldom live more than a few hours. The distortion of the fetal body may also pose a danger to the mother's life.

SYNONYMS OF INIENCEPHALY

Some of the Synonyms and related keywords are:-

1. Elevated maternal serum alpha-fetoprotein level
2. Absent cerebellum
3. Brain malformation
4. Dysphasia epiphysealis congenita
5. Stippled epiphyses
6. Chondrodysplasia punctata dominant type
7. Chondrodysplasia epiphysealis punctata Chondrodystrophia calcificans congenita, Conradi-Hunermann syndrome

CAUSES OF INIENCEPHALY

The some of the affective causes are described below:-

1. Mostly likely a mix of genetic and environmental factors.
2. Damage to the developing nervous system is a major cause of chronic, disabling disorders and, sometimes, death in infants, children, and even adults
3. Four main processes are responsible for the development of the nervous system: cell proliferation, cell migration, cell differentiation, and cell death, a natural process in which cells die.
4. A developmental failure occurs between the 23rd and 26th days of pregnancy with diet and vitamins may play a role

SYMPTOMS OF INIENCEPHALY

Symptoms may include delayed development, convulsions, seizures, and brain cortex/spinal cord dysfunction. Unilateral megalencephaly usually results in mental retardation and severe seizures the most severe symptoms of holoprosencephaly produce seizures and mental retardation. Another are involve the eyes, nose, and upper lip. The nose may be missing entirely or may develop above the eye in the form of a proboscis, a tubular appendage. Other facial anomalies include:
Ethmocephaly absent nose, a proboscis separates narrow-set,
Sometimes abnormally small, eyes or narrow-set eyes

DIAGNOSIS ON INIENCEPHALY

Diagnosis of Iniencephaly was made and the pregnancy terminated. Back showed black area in mid line with excessive presence of hair.
Differential diagnosis - klippel feil, or anencephaly with spinal retroflexion. Associated anomalies include - Agnathia, cephalocele, microstomia, palatal anteversion, spinabifida, omphalocele, gastroschisis, diaphragmatic hemia, pulmonary agenesis.

TREATMENT OF INIENCEPHALY

No standard treatment for Iniencephaly has been discovered since most infants rarely live longer than a few hours. Medicine is based more on prevention using supplementation with folic acid. Maternal obesity and diabetes are also known to increase the risk for these disorders. Pregnant women should avoid taking antiepileptic drugs, diuretics, antihistamines, and sulfa drugs, which have been shown to be associated with an increased risk of neural tube defects.