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ICD Hub / Developmental anomalies (Q00–Q99) / LA00.1 - Iniencephaly
ICD-11

ICD-11 Codes
for LA00.1 - Iniencephaly
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Synonyms and Alternative Terms for Iniencephaly

Iniencephaly is also known by the clinical synonym Iniencephalus. It is broadly described as a rare cephalic disorder or a severe form of neural tube defect.

Diagnosis of Iniencephaly

Antenatal diagnosis of Iniencephaly is possible through ultrasonographic monitoring during pregnancy. The characteristic physical malformations, including the occipital bone defect, absence of the neck, and extreme backward bending of the head, can be identified via imaging techniques. This allows for early identification and management planning.

Understanding Iniencephaly: A Rare Neural Tube Defect

Iniencephaly, identified by the ICD-11 code LA00.1, is a severe and rare congenital disorder. It is classified as a neural tube defect and a cephalic disorder, characterised by significant malformations at the cervico-occipital junction and abnormalities within the central nervous system. This condition presents a complex clinical picture, often leading to profound developmental challenges.

Factors Contributing to Iniencephaly

As with other neural tube defects, Iniencephaly is believed to arise from a multifactorial origin. While specific causes are not fully understood, it is recognised as a complex developmental anomaly.

Key Signs and Presentation of Iniencephaly

The hallmark features of Iniencephaly include a defect in the occipital bone, a partial or complete absence of cervicothoracic vertebrae, and a characteristic retroflexion (backward bending) of the head on the cervical spine. This results in a striking absence of the neck, where the skin of the face appears continuous with the chest. Associated malformations frequently involve the central nervous system, such as spina bifida or anencephaly, and can also include gastrointestinal defects like omphalocele and cardiovascular anomalies. The prognosis is typically poor, with stillbirth being the most common outcome. Live births are rare, and survival is usually very short.

Coding and Documentation for Iniencephaly (LA00.1)

Accurate medical record documentation is essential for Iniencephaly. The official ICD-11 code for this condition is LA00.1. This code is used for clinical reporting, billing purposes, and statistical tracking of this rare disorder. When documenting, healthcare professionals should clearly state the diagnosis and use the appropriate ICD-11 code, LA00.1, for precise medical coding. This ensures correct classification for patient care and administrative processes.

Frequently asked questions

What is the ICD-11 classification for Iniencephaly?
Iniencephaly is classified under ICD-11 code LA00.1, within the broader category of 'Anencephaly or similar anomalies' (LA00).
What is the ICD-11 classification meaning of Iniencephaly (LA00.1)?
Within ICD-11, Iniencephaly (LA00.1) falls under the classification for 'Anencephaly or similar anomalies' (LA00), which describes a malformation of the nervous system due to failure of neuropore closure, characterized by the absence of neural structures above the spinal cord, cerebellum, and brainstem, with a partially formed skull.
How is Iniencephaly (LA00.1) distinguished from other codes in the 'Anencephaly or similar anomalies' block in ICD-11?
ICD-11 provides specific codes for related conditions within the LA00 block. Iniencephaly is coded as LA00.1, distinct from Anencephaly (LA00.0), Acephaly (LA00.2), and Amyelencephaly (LA00.3), as well as unspecified or other specified anomalies in this group (LA00.Z, LA00.Y).
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