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Ocular Hypertelorism
Hypertelorism is an abnormally increased distance between two organs or bodily parts, usually referring to an increased distance between the orbits (eyes), or orbital hypertelorism. In this condition, the distance between the inner eye corners, as well as the distance between the pupils, is greater than normal. Hypertelorism should not be confused with telecanthus, in which the distance between the inner eye corners is increased, but the distances between the outer eye corners and the pupils remain unchanged.Michael L. Bentz: ''Pediatric Plastic Surgery''; Chapter 9 Hypertelorism by Renato Ocampo, Jr., MD/ John A. Persing, MD Hypertelorism is a symptom in a variety of syndromes, including Edwards syndrome (trisomy 18), 1q21.1 duplication syndrome, basal cell nevus syndrome, DiGeorge syndrome and Loeys–Dietz syndrome. Hypertelorism can also be seen in Apert syndrome, Autism spectrum disorder, craniofrontonasal dysplasia, Noonan syndrome, neurofibromatosis, LEOPARD syndrome, Cr ...
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Craniofrontonasal Dysplasia
Craniofrontonasal dysplasia (craniofrontonasal syndrome, craniofrontonasal dysostosis, CFND) is a very rare X-linked malformation syndrome caused by mutations in the ephrin-B1 gene (''EFNB1''). Phenotypic expression varies greatly amongst affected individuals, where females are more commonly and generally more severely affected than males. Common physical malformations are: craniosynostosis of the coronal suture(s), orbital hypertelorism, bifid nasal tip, dry frizzy curled hair, longitudinal ridging and/or splitting of the nails, and facial asymmetry. The diagnosis CFND is determined by the presence of a mutation in the EFNB1 gene. Physical characteristics may play a supportive role in establishing the diagnosis. The treatment is always surgical and is based on each patients specific phenotypic presentation. Presentation Phenotypic expression varies greatly between individuals with CFND. Some of the more prominent characteristics are: * Craniosynostosis of the coronal suture( ...
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Mucopolysaccharide
Glycosaminoglycans (GAGs) or mucopolysaccharides are long, linear polysaccharide Polysaccharides (), or polycarbohydrates, are the most abundant carbohydrates found in food. They are long-chain polymeric carbohydrates composed of monosaccharide units bound together by glycosidic linkages. This carbohydrate can react with wat ...s consisting of repeating disaccharide units (i.e. two-sugar units). The repeating two-sugar unit consists of a uronic acid, uronic sugar and an amino sugar, except in the case of the sulfated glycosaminoglycan keratan sulfate, keratan, where, in place of the uronic sugar there is a galactose unit. GAGs are found in vertebrates, invertebrates and bacteria. Because GAGs are highly polar molecules and attract water; the body uses them as lubricants or shock absorbers. Mucopolysaccharidosis, Mucopolysaccharidoses are a group of metabolic disorders in which abnormal accumulations of glycosaminoglycans occur due to enzyme deficiencies. Production Glycosamin ...
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Dural Tear
Dural tear is a tear occurring in the dura mater of the brain. It is usually caused as a result of trauma or as a complication following surgery. __TOC__ Diagnosis In case of head injury, a dural tear is likely in case of a depressed skull fracture. A burr hole is made through the normal skull near the fractured portion, and Adson's elevator is introduced. Underlying dura is separated carefully from the overlying depressed bone fragments. The dura that is now visible is carefully examined to exclude any dural tear. Treatment The whole extent of the dural tear is exposed by removing the overlying skull. The ragged edges of the tear are excised. However, care should be taken not to excise too much dura as it may increase the chances for spread of infection into the subarachnoid space. Removing too much dura will also make it difficult to close the tear. If there is not much dural loss, interrupted sutures are made with non-absorbable material. In case of dural loss, the defect is ...
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Cerebrospinal Fluid
Cerebrospinal fluid (CSF) is a clear, colorless Extracellular fluid#Transcellular fluid, transcellular body fluid found within the meninges, meningeal tissue that surrounds the vertebrate brain and spinal cord, and in the ventricular system, ventricles of the brain. CSF is mostly produced by specialized Ependyma, ependymal cells in the choroid plexuses of the ventricles of the brain, and absorbed in the arachnoid granulations. It is also produced by ependymal cells in the lining of the ventricles. In humans, there is about 125 mL of CSF at any one time, and about 500 mL is generated every day. CSF acts as a shock absorber, cushion or buffer, providing basic mechanical and immune system, immunological protection to the brain inside the Human skull, skull. CSF also serves a vital function in the cerebral autoregulation of cerebral blood flow. CSF occupies the subarachnoid space (between the arachnoid mater and the pia mater) and the ventricular system around and inside t ...
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Human Mandible
In jawed vertebrates, the mandible (from the Latin ''mandibula'', 'for chewing'), lower jaw, or jawbone is a bone that makes up the lowerand typically more mobilecomponent of the mouth (the upper jaw being known as the maxilla). The jawbone is the skull's only movable, posable bone, sharing joints with the cranium's temporal bones. The mandible hosts the lower teeth (their depth delineated by the alveolar process). Many muscles attach to the bone, which also hosts nerves (some connecting to the teeth) and blood vessels. Amongst other functions, the jawbone is essential for chewing food. Owing to the Neolithic advent of agriculture (), human jaws evolved to be smaller. Although it is the strongest bone of the facial skeleton, the mandible tends to deform in old age; it is also subject to fracturing. Surgery allows for the removal of jawbone fragments (or its entirety) as well as regenerative methods. Additionally, the bone is of great forensic significance. Struct ...
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Zygoma
The term zygoma generally refers to the zygomatic bone, a bone of the human skull that is commonly referred to as the cheekbone or malar bone, but it may also refer to: * The zygomatic arch, a structure in the human skull formed primarily by parts of the zygomatic bone and the temporal bone * The zygomatic process, a bony protrusion of the human skull, mostly composed of the zygomatic bone but also contributed to by the frontal bone, temporal bone, and maxilla In vertebrates, the maxilla (: maxillae ) is the upper fixed (not fixed in Neopterygii) bone of the jaw formed from the fusion of two maxillary bones. In humans, the upper jaw includes the hard palate in the front of the mouth. The two maxil ... See also * Zygoma implant * Zygoma reduction plasty {{set index article Anatomy ...
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Distraction Osteogenesis
Distraction osteogenesis (DO), also called callus distraction, callotasis and osteodistraction, is a process used in orthopedic surgery, podiatric surgery, and oral and maxillofacial surgery to repair skeletal deformities and in reconstructive surgery. The procedure involves cutting and slowly separating bone, allowing the bone healing process to fill in the gap. Medical uses Distraction osteogenesis (DO) is used in orthopedic surgery, and oral and maxillofacial surgery to repair skeletal deformities and in reconstructive surgery. It was originally used to treat problems like unequal leg length, but since the 1980s is most commonly used to treat issues like hemifacial microsomia, micrognathism (chin so small it causes health problems), craniofrontonasal dysplasias, craniosynostosis, as well as airway obstruction in babies caused by glossoptosis (tongue recessed too far back in the mouth) or micrognathism. In 2016, a systematic review of papers describing bone and soft tissu ...
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Dysplasia
Dysplasia is any of various types of abnormal growth or development of cells (microscopic scale) or organs (macroscopic scale), and the abnormal histology or anatomical structure(s) resulting from such growth. Dysplasias on a mainly microscopic scale include epithelial dysplasia and fibrous dysplasia of bone. Dysplasias on a mainly macroscopic scale include hip dysplasia, myelodysplastic syndrome, and multicystic dysplastic kidney. In one of the modern histopathological senses of the term, dysplasia is sometimes differentiated from other categories of tissue change including hyperplasia, metaplasia, and neoplasia, and dysplasias are thus generally not cancerous. An exception is that the myelodysplasias include a range of benign, precancerous, and cancerous forms. Various other dysplasias tend to be precancerous. The word's meanings thus cover a spectrum of histopathological variations. Microscopic scale Epithelial dysplasia Epithelial dysplasia consists of an expa ...
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Paul Tessier
Paul Tessier (August 1917 – June 6, 2008) was a French maxillofacial surgeon. He was considered the father of modern craniofacial surgery. Biography Born in Héric, Loire-Atlantique, Dr. Tessier first attended the Ecole de Médecine in Nantes, Loire-Atlantique, eventually receiving his Doctor of Medicine degree from the Faculté de Médecine de Paris in 1943. In 1942, during internship he started operating on people with cleft lip and cleft palate and Dupuytren's contracture. He joined the pediatric surgery service at Hospital St. Joseph in Paris in 1944. From late 1944 to 1946, he worked at the Center of Maxillofacial Surgery of the Military Region of Paris in Hospital Puteaux. In 1949, he returned to Nantes to become a surgical consultant in ophthalmology. Dr. Tessier started to improve surgical techniques to correct craniofacial deformations in the mid-1950s. He performed his first craniofacial operation in 1967. Throughout the 1960s and 1970s, he developed the follo ...
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Craniofacial Surgery
Craniofacial surgery is a surgical subspecialty that deals with congenital disorder, congenital and acquired deformity, deformities of the human head, head, human skull, skull, face, neck, jaws and associated structures. Although craniofacial treatment often involves manipulation of bone, craniofacial surgery is not tissue-specific; craniofacial surgery, surgeons deal with bone, skin, nerve, muscle, teeth, and other related anatomy. Defects typically treated by craniofacial surgeons include craniosynostosis (isolated and syndromic), rare facial cleft, craniofacial clefts, acute and chronic sequelae of facial fractures, cleft lip and palate, micrognathia, Treacher Collins Syndrome, Apert's Syndrome, Crouzon's Syndrome, Craniofacial microsomia, microtia and other congenital ear anomalies, and many others. Training in craniofacial surgery requires completion of a Craniofacial surgery fellowship. Such fellowships are available to individuals who have completed residency in oral and maxi ...
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Paranasal Sinuses
Paranasal sinuses are a group of four paired air-filled spaces that surround the nasal cavity. The maxillary sinuses are located under the eyes; the frontal sinuses are above the eyes; the ethmoidal sinuses are between the eyes and the sphenoidal sinuses are behind the eyes. The sinuses are named for the facial bones and sphenoid bone in which they are located. Their role is disputed. Structure Humans possess four pairs of paranasal sinuses, divided into subgroups that are named according to the bones within which the sinuses lie. They are all innervated by branches of the trigeminal nerve (CN V). * The maxillary sinuses, the largest of the paranasal sinuses, are under the eyes, in the maxillary bones (open in the back of the semilunar hiatus of the nose). They are innervated by the maxillary nerve (CN V2). * The frontal sinuses, superior to the eyes, in the frontal bone, which forms the hard part of the forehead. They are innervated by the ophthalmic nerve (CN V1 ...
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Sphenoid Bone
The sphenoid bone is an unpaired bone of the neurocranium. It is situated in the middle of the skull towards the front, in front of the basilar part of occipital bone, basilar part of the occipital bone. The sphenoid bone is one of the seven bones that articulate to form the orbit (anatomy), orbit. Its shape somewhat resembles that of a butterfly, bat or wasp with its wings extended. The name presumably originates from this shape, since () means in Ancient Greek. Structure It is divided into the following parts: * a median portion, known as the body of sphenoid bone, containing the sella turcica, which houses the pituitary gland as well as the paired paranasal sinuses, the sphenoidal sinuses * two Greater wing of sphenoid bone, greater wings on the lateral side of the body and two Lesser wing of sphenoid bone, lesser wings from the anterior side. * Pterygoid processes of the sphenoides, directed downwards from the junction of the body and the greater wings. Two sphenoidal co ...
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