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2025 ICD-10-CM code Q00.0

Anencephaly.

Code Q00.0 is used to report anencephaly. It is not to be used on the maternal record. This code should be used for both live births and fetal deaths.

Medical necessity for services related to anencephaly centers around providing supportive and palliative care to the infant and support and counseling to the family. Prenatal diagnosis allows parents to make informed decisions about the pregnancy. Postnatal care focuses on comfort and dignity for the infant.

Diagnosing and managing anencephaly falls under the purview of various medical specialists, including obstetricians, neonatologists, geneticists, and palliative care specialists. Obstetricians play a key role in prenatal diagnosis and counseling parents about the condition. Neonatologists provide care for infants born with anencephaly, focusing on comfort measures and supportive care. Geneticists help families understand the genetic basis of the condition and recurrence risks. Palliative care specialists offer support and guidance in managing pain and other symptoms to ensure the infant's comfort.

In simple words: Anencephaly is a birth defect where a baby is born without parts of their brain and skull. Sadly, babies with this condition usually only live for a short time after birth.

Anencephaly is a severe congenital malformation characterized by the absence of a major portion of the brain, skull, and scalp. This neural tube defect occurs during embryonic development when the rostral (head) end of the neural tube fails to close, typically between the 23rd and 26th day following conception.Infants with anencephaly are usually blind, deaf, unaware of their surroundings, and unable to feel pain. While some may have a brainstem, the absence of a functioning cerebrum prevents awareness. Reflex actions like breathing and responses to stimuli may occur, but higher brain functions are absent.The most common type is holoanencephaly, where the brain fails to form except for the brainstem.

Example 1: A pregnant woman undergoes a routine ultrasound at 20 weeks gestation, revealing findings consistent with anencephaly. Subsequent amniocentesis confirms the diagnosis., A baby is born with anencephaly, exhibiting absent cranial vault and exposed brain tissue. The infant is provided with comfort care and support, but sadly passes away within a few hours., A couple with a family history of neural tube defects seeks genetic counseling before planning a pregnancy. The counselor assesses their risk factors and educates them on preventative measures like folic acid supplementation.

Documentation for anencephaly should include detailed prenatal ultrasound findings, results of any confirmatory testing like amniocentesis, and a description of the infant's physical examination at birth, noting the extent of the malformation. Any supportive care provided and the outcome should also be documented.

** While there is no cure or treatment for anencephaly, prevention through adequate folic acid intake before and during pregnancy is crucial. Women with a family history of neural tube defects should consult with their physician or a genetic counselor regarding appropriate folic acid supplementation.

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