The absence of a major portion of the brain, skull and scalp is known as:

Appeared in: Bihar NHM CHO-2025

Explanation

Anencephaly is a severe neural tube defect resulting from the failure of the anterior neural tube to close during early embryonic development.
It is characterized by the absence of the forebrain (cerebral hemispheres) and the overlying skull (calvarium) and scalp.
The remaining brain tissue, known as the area cerebrovasculosa, is exposed, disorganized, and non-functional.
This condition is uniformly lethal, with most affected infants being stillborn or dying shortly after birth.

Option A: Brachycephaly refers to a specific skull shape where the head is disproportionately short and broad. It does not involve the absence of the brain or skull.
Option C: Lissencephaly, meaning "smooth brain," is a malformation where the brain lacks the normal folds and grooves (gyri and sulci). The skull and scalp are present.
Option D: Plagiocephaly describes an asymmetrical or flattened shape of the head. It is a deformity of the skull, not an absence of its major parts.

Visual 1: Medical Illustration - A clear illustration showing a fetus/newborn with anencephaly, highlighting the absent cranial vault and exposed brain tissue.
Visual 2: Diagram - A comparative diagram showing the skull shapes in anencephaly, brachycephaly, lissencephaly (via brain scan), and plagiocephaly to help differentiate the conditions.

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Congenital anomalies of the central nervous system as background academic context rather than a clinical decision trigger.
Anencephaly is detectable via prenatal ultrasound and elevated maternal serum alpha-fetoprotein (MSAFP) levels.
Nursing care for a family receiving a diagnosis of anencephaly focuses on providing emotional support, clear information, and counseling on options, which may include termination of pregnancy or perinatal palliative care.
Folic acid supplementation before and during early pregnancy is a critical preventive measure that significantly reduces the risk of neural tube defects, including anencephaly.

How to Approach the Question

This is a factual recall question that tests your knowledge of medical terminology for congenital anomalies.
Read the question carefully to identify the key descriptive features: "absence of a major portion of the brain, skull and scalp."
Analyze each option by defining it based on your medical vocabulary.
Anencephaly: 'An-' means without, and 'encephalon' refers to the brain. This aligns with the description.
Brachycephaly, lissencephaly, and plagiocephaly describe abnormalities in shape or structure, not the complete absence of these parts.
Select the term that most accurately matches the pathological description in the question stem.

Concept Tested & Keywords

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Practise the full Bihar NHM CHO-2025

Attempt every question from this paper in a timed mock, then review the full solution for each one.