Agnathia

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Agnathia
Symptoms Absence of a portion or the entirety of one or both jaws. [1]
Diagnostic method X-rays or CT scans of the mandible and temporomandibular joint. [2]
TreatmentSurgery
FrequencyRare. [1]

Agnathia (also termed hypognathous) [1] is the absence of a portion or the entirety of one or both jaws. [1] [3] It is a very rare condition. [1] External, middle, and inner ear abnormalities, as well as temporal bone, parotid gland, masticatory muscles, and facial neural abnormalities, frequently coexist with Agnathia. Agnathia is seen in agnathia-holoprosencephaly, otocephaly, and Ivemark syndrome. [2]

Contents

Diagnosis

X-rays or CT scans of the mandible and temporomandibular joint showcase the extent of underdevelopment and differentiate Agnathia from other disorders that cause similar facial abnormalities but do not involve substantial structural loss. [2]

Treatment

Agnathia treatment consists of rapid reconstruction using autogenous bone grafting to stop the further development of facial deformity. Mentoplasty, bone and cartilage onlay grafts, and soft-tissue grafts are frequently used to restore facial symmetry. Distraction osteogenesis, which involves performing an osteotomy and attaching a distraction device to both portions of the mandible, is becoming more common. Early orthodontic therapy in adolescents aids in the correction of malocclusion. [2]

See also

Related Research Articles

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In anatomy, the temporomandibular joints (TMJ) are the two joints connecting the jawbone to the skull. It is a bilateral synovial articulation between the temporal bone of the skull above and the mandible below; it is from these bones that its name is derived. The joints are unique in their bilateral function, being connected via the mandible.

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<span class="mw-page-title-main">Ameloblastoma</span> Medical condition

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<span class="mw-page-title-main">Orthognathic surgery</span> Surgery of the jaw

Orthognathic surgery, also known as corrective jaw surgery or simply jaw surgery, is surgery designed to correct conditions of the jaw and lower face related to structure, growth, airway issues including sleep apnea, TMJ disorders, malocclusion problems primarily arising from skeletal disharmonies, and other orthodontic dental bite problems that cannot be treated easily with braces, as well as the broad range of facial imbalances, disharmonies, asymmetries, and malproportions where correction may be considered to improve facial aesthetics and self-esteem.

<span class="mw-page-title-main">Hemifacial microsomia</span> Birth defect which affects the lower half of the face

Hemifacial microsomia (HFM) is a congenital disorder that affects the development of the lower half of the face, most commonly the ears, the mouth and the mandible. It usually occurs on one side of the face, but both sides are sometimes affected. If severe, it may result in difficulties in breathing due to obstruction of the trachea—sometimes even requiring a tracheotomy. With an incidence in the range of 1:3500 to 1:4500, it is the second most common birth defect of the face, after cleft lip and cleft palate. HFM shares many similarities with Treacher Collins syndrome.

<span class="mw-page-title-main">Cherubism</span> Medical condition

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The Stafne defect is a depression of the mandible, most commonly located on the lingual surface. The Stafne defect is thought to be a normal anatomical variant, as the depression is created by ectopic salivary gland tissue associated with the submandibular gland and does not represent a pathologic lesion as such. This cavity is commonly observed on panoramic radiograph.

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Parry–Romberg syndrome (PRS) is a rare disease presenting in early childhood characterized by progressive shrinkage and degeneration of the tissues beneath the skin, usually on only one side of the face but occasionally extending to other parts of the body. An autoimmune mechanism is suspected, and the syndrome may be a variant of localized scleroderma, but the precise cause and pathogenesis of this acquired disorder remains unknown. It has been reported in the literature as a possible consequence of sympathectomy. The syndrome has a higher prevalence in females and typically appears between 5 and 15 years of age. There has been only one case report of the syndrome appearing in older adults: a 43-year-old woman with symptoms appearing at the age of 33.

<span class="mw-page-title-main">Mandibular fracture</span> Medical condition

Mandibular fracture, also known as fracture of the jaw, is a break through the mandibular bone. In about 60% of cases the break occurs in two places. It may result in a decreased ability to fully open the mouth. Often the teeth will not feel properly aligned or there may be bleeding of the gums. Mandibular fractures occur most commonly among males in their 30s.

<span class="mw-page-title-main">Hemifacial hypertrophy</span> Medical condition

Hemifacial hypertrophy abbreviated as (HFH) is rare congenital disease characterized by unilateral enlargement of the head and teeth. It is classified as true HFH (THFH) with unilateral enlargement of the viscerocranium, and partial HFH (PHFH) in which not all structures are enlarged. Hemifacial hypertrophy can cause a wide spectrum of defects or may involve only muscle or bone. it is usually treated surgically. It is believed to be a minor form of hemihypertrophy.

<span class="mw-page-title-main">Jaw abnormality</span> Medical condition

A jaw abnormality is a disorder in the formation, shape and/or size of the jaw. In general abnormalities arise within the jaw when there is a disturbance or fault in the fusion of the mandibular processes. The mandible in particular has the most differential typical growth anomalies than any other bone in the human skeleton. This is due to variants in the complex symmetrical growth pattern which formulates the mandible.

<span class="mw-page-title-main">Mandible</span> Lower jaw bone

In jawed vertebrates, the mandible, lower jaw, or jawbone is a bone that makes up the lower – and typically more mobile – component of the mouth.

<span class="mw-page-title-main">Salivary gland disease</span> Medical condition

Salivary gland diseases (SGDs) are multiple and varied in cause. There are three paired major salivary glands in humans: the parotid glands, the submandibular glands, and the sublingual glands. There are also about 800–1,000 minor salivary glands in the mucosa of the mouth. The parotid glands are in front of the ears, one on side, and secrete mostly serous saliva, via the parotid ducts, into the mouth, usually opening roughly opposite the second upper molars. The submandibular gland is medial to the angle of the mandible, and it drains its mixture of serous and mucous saliva via the submandibular duct into the mouth, usually opening in a punctum in the floor of mouth. The sublingual gland is below the tongue, on the floor of the mouth; it drains its mostly mucous saliva into the mouth via about 8–20 ducts, which open along the plica sublingualis, a fold of tissue under the tongue.

Condylar hypoplasia is known as underdevelopment of the mandibular condyle. Congenitally (primary) caused condylar hypoplasia leads to underdeveloped condyle at birth. Hypoplasia of mandible can be diagnosed during birth, in comparison to the hyperplasia which is only diagnosed later in growth of an individual.

Alveoloplasty is a surgical pre-prosthetic procedure performed to facilitate removal of teeth, and smoothen or reshape the jawbone for prosthetic and cosmetic purposes. In this procedure, the bony edges of the alveolar ridge and its surrounding structures is made smooth, redesigned or recontoured so that a well-fitting, comfortable, and esthetic prosthesis may be fabricated or implants may be surgically inserted. This pre-prosthetic surgery which may include bone grafting prepares the mouth to receive a prosthesis or implants by improving the condition and quality of the supporting structures so they can provide support, better retention and stability to the prosthesis.

References

  1. 1 2 3 4 5 Ghom AG; Ghom SA (30 September 2014). Textbook of Oral Medicine. JP Medical Ltd. p. 125. ISBN   978-93-5152-303-1.
  2. 1 2 3 4 "Congenital Jaw Abnormalities - Pediatrics". Merck Manuals Professional Edition. Retrieved 2023-09-29.
  3. Rajendran A; Sundaram S (10 February 2014). Shafer's Textbook of Oral Pathology (7th ed.). Elsevier Health Sciences APAC. p. 12. ISBN   978-81-312-3800-4.