Cephalic disorder
Encyclopedia
Cephalic disorders are congenital
conditions that stem from damage to, or abnormal development of, the budding nervous system
. Cephalic is a term that means "head" or "head end of the body."
Cephalic disorders are not necessarily caused by a single factor, but may be influenced by hereditary
or genetic
conditions, nutrition
al deficiencies, or by environmental exposures during pregnancy
, such as medication
taken by the mother, maternal infection, or exposure to radiation
(such disorders are more common in areas of the former Soviet Union
affected by nuclear waste disposal problems, such as the area around the Mayak
plant in Chelyabinsk
, Russia
.) Some cephalic disorders occur when the cranial sutures (the fibrous joints that connect the bone
s of the skull
) join prematurely. Most cephalic disorders are caused by a disturbance that occurs very early in the development of the fetal
nervous system.
The human nervous system develops from a small, specialized plate of cells
on the surface of the embryo
. Early in development, this plate of cells forms the neural tube, a narrow sheath that closes between the third and fourth weeks of pregnancy
to form the brain and spinal cord
of the embryo. Four main processes are responsible for the development of the nervous system: cell proliferation, the process in which nerve
cells divide to form new generations of cells; cell migration, the process in which nerve cells move from their place of origin to the place where they will remain for life; cell differentiation, the process during which cells acquire individual characteristics; and cell death, a natural process in which cells die.
Damage to the developing nervous system is a major cause of chronic, disabling disorders and, sometimes, death in infants, children, and even adults. The degree to which damage to the developing nervous system harms the mind and body varies enormously. Many disabilities
are mild enough to allow those afflicted to eventually function independently in society. Others are not. Some infants, children, and adults die, others remain totally disabled, and an even larger population is partially disabled, functioning well below normal capacity throughout life.
code is listed below.
Congenital disorder
A congenital disorder, or congenital disease, is a condition existing at birth and often before birth, or that develops during the first month of life , regardless of causation...
conditions that stem from damage to, or abnormal development of, the budding nervous system
Nervous system
The nervous system is an organ system containing a network of specialized cells called neurons that coordinate the actions of an animal and transmit signals between different parts of its body. In most animals the nervous system consists of two parts, central and peripheral. The central nervous...
. Cephalic is a term that means "head" or "head end of the body."
Cephalic disorders are not necessarily caused by a single factor, but may be influenced by hereditary
Heredity
Heredity is the passing of traits to offspring . This is the process by which an offspring cell or organism acquires or becomes predisposed to the characteristics of its parent cell or organism. Through heredity, variations exhibited by individuals can accumulate and cause some species to evolve...
or genetic
Genetics
Genetics , a discipline of biology, is the science of genes, heredity, and variation in living organisms....
conditions, nutrition
Nutrition
Nutrition is the provision, to cells and organisms, of the materials necessary to support life. Many common health problems can be prevented or alleviated with a healthy diet....
al deficiencies, or by environmental exposures during pregnancy
Pregnancy
Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...
, such as medication
Medication
A pharmaceutical drug, also referred to as medicine, medication or medicament, can be loosely defined as any chemical substance intended for use in the medical diagnosis, cure, treatment, or prevention of disease.- Classification :...
taken by the mother, maternal infection, or exposure to radiation
Radiation
In physics, radiation is a process in which energetic particles or energetic waves travel through a medium or space. There are two distinct types of radiation; ionizing and non-ionizing...
(such disorders are more common in areas of the former Soviet Union
Soviet Union
The Soviet Union , officially the Union of Soviet Socialist Republics , was a constitutionally socialist state that existed in Eurasia between 1922 and 1991....
affected by nuclear waste disposal problems, such as the area around the Mayak
Mayak
Mayak Production Association refers to an industrial complex that is one of the biggest nuclear facilities in the Russian Federation. It housed plutonium production reactors and a reprocessing plant...
plant in Chelyabinsk
Chelyabinsk
Chelyabinsk is a city and the administrative center of Chelyabinsk Oblast, Russia, located in the northwestern side of the oblast, south of Yekaterinburg, just to the east of the Ural Mountains, on the Miass River. Population: -History:...
, Russia
Russia
Russia or , officially known as both Russia and the Russian Federation , is a country in northern Eurasia. It is a federal semi-presidential republic, comprising 83 federal subjects...
.) Some cephalic disorders occur when the cranial sutures (the fibrous joints that connect the bone
Bone
Bones are rigid organs that constitute part of the endoskeleton of vertebrates. They support, and protect the various organs of the body, produce red and white blood cells and store minerals. Bone tissue is a type of dense connective tissue...
s of the skull
Human skull
The human skull is a bony structure, skeleton, that is in the human head and which supports the structures of the face and forms a cavity for the brain.In humans, the adult skull is normally made up of 22 bones...
) join prematurely. Most cephalic disorders are caused by a disturbance that occurs very early in the development of the fetal
Fetus
A fetus is a developing mammal or other viviparous vertebrate after the embryonic stage and before birth.In humans, the fetal stage of prenatal development starts at the beginning of the 11th week in gestational age, which is the 9th week after fertilization.-Etymology and spelling variations:The...
nervous system.
The human nervous system develops from a small, specialized plate of cells
Cell (biology)
The cell is the basic structural and functional unit of all known living organisms. It is the smallest unit of life that is classified as a living thing, and is often called the building block of life. The Alberts text discusses how the "cellular building blocks" move to shape developing embryos....
on the surface of the embryo
Embryo
An embryo is a multicellular diploid eukaryote in its earliest stage of development, from the time of first cell division until birth, hatching, or germination...
. Early in development, this plate of cells forms the neural tube, a narrow sheath that closes between the third and fourth weeks of pregnancy
Pregnancy
Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...
to form the brain and spinal cord
Spinal cord
The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
of the embryo. Four main processes are responsible for the development of the nervous system: cell proliferation, the process in which nerve
Nerve
A peripheral nerve, or simply nerve, is an enclosed, cable-like bundle of peripheral axons . A nerve provides a common pathway for the electrochemical nerve impulses that are transmitted along each of the axons. Nerves are found only in the peripheral nervous system...
cells divide to form new generations of cells; cell migration, the process in which nerve cells move from their place of origin to the place where they will remain for life; cell differentiation, the process during which cells acquire individual characteristics; and cell death, a natural process in which cells die.
Damage to the developing nervous system is a major cause of chronic, disabling disorders and, sometimes, death in infants, children, and even adults. The degree to which damage to the developing nervous system harms the mind and body varies enormously. Many disabilities
Disability
A disability may be physical, cognitive, mental, sensory, emotional, developmental or some combination of these.Many people would rather be referred to as a person with a disability instead of handicapped...
are mild enough to allow those afflicted to eventually function independently in society. Others are not. Some infants, children, and adults die, others remain totally disabled, and an even larger population is partially disabled, functioning well below normal capacity throughout life.
More common cephalic disorders
Where known, the ICD-10ICD
The International Statistical Classification of Diseases and Related Health Problems is a medical classification that provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances, and external causes of injury or disease...
code is listed below.
- AnencephalyAnencephalyAnencephaly is a cephalic disorder that results from a neural tube defect that occurs when the cephalic end of the neural tube fails to close, usually between the 23rd and 26th day of pregnancy, resulting in the absence of a major portion of the brain, skull, and scalp...
(Q00.0) - ColpocephalyColpocephalyColpocephaly refers to an abnormal appearance of the brain in which there is asymmetric dilatation of its lateral ventricle occipital horns, but with normal caliber frontal horns. It is typically associated with microcephaly and developmental delay...
(ICD10 unknown) - HoloprosencephalyHoloprosencephalyHoloprosencephaly is a cephalic disorder in which the prosencephalon fails to develop into two hemispheres. Normally, the forebrain is formed and the face begins to develop in the fifth and sixth weeks of human pregnancy...
(Q04.2) - EthmocephalyEthmocephalyEthmocephaly is a type of cephalic disorder caused by holoprosencephaly. Ethmocephaly is the least common facial anomaly. It consists of a proboscis separating narrow-set eyes with an absent nose and microphthalmia...
(ICD10 unknown) - HydranencephalyHydranencephalyHydranencephaly, synonym hydroanencephaly, is a type of cephalic disorder.These disorders are congenital conditions that derive from either damage to, or abnormal development of, the fetal nervous system in the earliest stages of development in utero...
(Q04.3) - IniencephalyIniencephalyIniencephaly 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.-Presentation:...
(Q00.2) - LissencephalyLissencephalyLissencephaly, which literally means smooth brain, is a rare brain formation disorder caused by defective neuronal migration during the 12th to 24th weeks of gestation, resulting in a lack of development of brain folds and grooves . It is a form of cephalic disorder...
(Q04.3) - MegalencephalyMegalencephalyMegalencephaly is a condition in which there is an abnormally large brain. It is usually functioning abnormally. Megalencephaly is, by definition, when the brain weight is greater than average for the age and sex of the infant or child...
(Q04.5) - MicrocephalyMicrocephalyMicrocephaly is a neurodevelopmental disorder in which the circumference of the head is more than two standard deviations smaller than average for the person's age and sex. Microcephaly may be congenital or it may develop in the first few years of life...
(Q02) - PorencephalyPorencephalyPorencephaly is a type of cephalic disorder involving encephalomalacia. This is an extremely rare disorder of the central nervous system involving a cyst or cavity in a cerebral hemisphere. The cysts or cavities are usually the remnants of destructive lesions, but are sometimes the result of...
(Q04.6) - SchizencephalySchizencephalySchizencephaly is a rare grey matter malformation of the brain. It belongs to the group of malformations of the central nervous system.-Presentation:...
(Q04.6)
Less common cephalies
- AcephalyAcephaly (medicine)Acephaly is a type of cephalic disorder. It literally means absence of the head. It is a much rarer condition than anencephaly. The acephalic fetus is a parasitic twin attached to an otherwise intact fetus. The acephalic fetus has a body but lacks a head and heart; the fetus's neck is attached to...
(Q00.0) - ExencephalyExencephalyExencephaly is a type of cephalic disorder wherein the brain is located outside of the skull. This condition is usually found in embryos as an early stage of anencephaly. As an exencephalic pregnancy progresses, the neural tissue gradually degenerates....
(ICD10 unknown) - MacrocephalyMacrocephalyMacrocephaly , occurs when the head is abnormally large; this includes the scalp, the cranial bone, and the contents of the cranium.-Causes:...
(Q75.3) - Micrencephaly (Q02)
- OtocephalyOtocephalyOtocephaly is a type of cephalic disorder.This is a lethal condition in which the primary feature is agnathia — a developmental anomaly characterized by total or virtual absence of the lower jaw...
(Q18.2) - Craniostenosis (ICD10 unknown)
- BrachycephalyBrachycephalyBrachycephaly, also known as flat head syndrome, is a type of cephalic disorder. This can result from premature fusion of the coronal sutures or from external deformation . The coronal suture is the fibrous joint that unites the frontal bone with the two parietal bones of the skull. The parietal...
(ICD10 unknown) - OxycephalyOxycephalyOxycephaly, also known as turricephaly and high-head syndrome, is a type of cephalic disorder. This is a term sometimes used to describe the premature closure of the coronal suture plus any other suture, like the lambdoid, or it may be used to describe the premature fusing of all sutures. It should...
(Q75.0) - PlagiocephalyPlagiocephalyPlagiocephaly is a condition characterized by an asymmetrical distortion of the skull.-Causes:It is a common finding at birth and may be the result of a restrictive intrauterine environment. If there is premature union of skull bones, this is more properly called craniosynostosis...
(Q67.3 - ScaphocephalyScaphocephalyScaphocephaly , derived from the Greek skaphe , describes a specific variety of a long narrow head that resembles an inverted boat....
(ICD10 unknown) - TrigonocephalyTrigonocephalyTrigonocephaly is a congenital condition of premature fusion of the metopic suture leading to a triangular shaped forehead. The merging of the two frontal bones leads to transverse growth restriction and parallel growth expansion...
(Q75.0)
- Brachycephaly
- PolycephalyPolycephalyPolycephaly is a condition of having more than one head. The term is derived from the Greek stems poly- meaning 'much' and kephali- meaning "head", and encompasses bicephaly and dicephaly . A variation is an animal born with two faces on a single head, a condition known as diprosopus...
External links
- Positional Plagiocephaly information from Seattle Children's Hospital Craniofacial Center
- Holoprosencephaly Article from Journal of Postgraduate Medicine