The Bonnet Syndrome - A Background | Lecture notes Nursing

Bohring–Opitz syndrome

A uncommon illness called Bohring-Opitz syndrome impairs the growth of several

body components.

Bohring-Opitz syndrome is characterised by significant to severe intellectual

impairment, developmental delay, and seizures in the majority of cases. Most

afflicted people have normal-sized heads and no brain abnormalities; however,

some people have aberrant head growth. Microcephaly, a condition in which the

head is abnormally tiny, can result from aberrant development, as can

trigonocephaly, a condition in which the forehead appears pointy. Head

abnormalities are not a need for structural brain abnormalities to exist. For

instance, the tissue that links the left and right sides of the brain (the corpus

callosum) might be unusually thin, or the fluid-filled cavities towards the centre of

the brain (ventricles) may be typically big (ventriculomegaly).

Bohring-Opitz syndrome sufferers also experience eye issues that might impair

eyesight. Exophthalmos (protruding eyes), strabismus (eyes not pointing in the

same direction), hypertelorism (eyes spaced widely apart), and upward-pointing

outer corners of the eyes are all signs of this disorder (upslanting palpebral

fissures). Those who are affected may have problems in the nerves that transmit

information from the eyes to the brain, the retina, or their light-sensitive tissue at

the back of the eye (severe nearsightedness, high myopia) (optic nerves).

A flat nasal bridge, nostrils that open to the front rather than downward, a high

arch or opening in the roof of the mouth, a split in the upper lip (cleft lip), a small

lower jaw (micrognathia), low-set ears that are rotated backwards, a red

birthmark (nevus simplex) on the face (usually the forehead), and a low frontal

hairline often with eyebrows are additional facial differences connected to

Bohring-Opitz syndrome.

Bohring-Opitz syndrome causes some people to develop poorly before birth

(intrauterine growth retardation). They develop slowly and put on weight

throughout infancy and frequently have terrible eating problems accompanied by

frequent vomiting.

Bohring-Opitz syndrome posture, characterised by distinctive body stance, is

frequently seen in individuals with this disorder. The hands are held in this

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Bohring–Opitz syndrome A uncommon illness called Bohring-Opitz syndrome impairs the growth of several body components. Bohring-Opitz syndrome is characterised by significant to severe intellectual impairment, developmental delay, and seizures in the majority of cases. Most afflicted people have normal-sized heads and no brain abnormalities; however, some people have aberrant head growth. Microcephaly, a condition in which the head is abnormally tiny, can result from aberrant development, as can trigonocephaly, a condition in which the forehead appears pointy. Head abnormalities are not a need for structural brain abnormalities to exist. For instance, the tissue that links the left and right sides of the brain (the corpus callosum) might be unusually thin, or the fluid-filled cavities towards the centre of the brain (ventricles) may be typically big (ventriculomegaly). Bohring-Opitz syndrome sufferers also experience eye issues that might impair eyesight. Exophthalmos (protruding eyes), strabismus (eyes not pointing in the same direction), hypertelorism (eyes spaced widely apart), and upward-pointing outer corners of the eyes are all signs of this disorder (upslanting palpebral fissures). Those who are affected may have problems in the nerves that transmit information from the eyes to the brain, the retina, or their light-sensitive tissue at the back of the eye (severe nearsightedness, high myopia) (optic nerves). A flat nasal bridge, nostrils that open to the front rather than downward, a high arch or opening in the roof of the mouth, a split in the upper lip (cleft lip), a small lower jaw (micrognathia), low-set ears that are rotated backwards, a red birthmark (nevus simplex) on the face (usually the forehead), and a low frontal hairline often with eyebrows are additional facial differences connected to Bohring-Opitz syndrome. Bohring-Opitz syndrome causes some people to develop poorly before birth (intrauterine growth retardation). They develop slowly and put on weight throughout infancy and frequently have terrible eating problems accompanied by frequent vomiting. Bohring-Opitz syndrome posture, characterised by distinctive body stance, is frequently seen in individuals with this disorder. The hands are held in this

position with the wrists or all of the fingers inclined outward towards the fifth finger (ulnar deviation), the shoulders slumped, the elbows and wrists bent, and the legs often stretched straight. When people with the condition age, they often stop displaying the Bohring-Opitz syndrome posture. Additional anomalies include altered muscle tone and joint deformities (also known as contractures) that are present at birth in the knees, hips, or other joints. Recurrent infections, heart, kidney, or genital problems might occur in those who are affected. Wilms tumour, a kind of paediatric kidney cancer, can occasionally manifest.

The Bonnet Syndrome - A Background, Lecture notes of Nursing

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