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2.1
Congenital Mandibular Hypoplasia

Presentation

INTRODUCTION: Young children with severe mandibular hypoplasia usually present with varying degrees of peripheral airway obstruction and difficulty with feeding. Early treatment is important for such children. [ncbi.nlm.nih.gov]

[…] genioplasty using the resected mandibular angle J J Kim H S Song and C G Park 258 Radiation injury to the face I T Jackson J S Topf M Gowda S Lash and B Audet 264 A new surgical treatment for the obstructive sleep apnea syndrome O A Uysal 270 Video presentations [books.google.com]

The presented case began when a female 15-year-old patient searched a dentist, a specialist in functional orthopedics of the jaws, complaining of large skeletal asymmetry. [scielo.br]

Entire Body System

  • Congestive Heart Failure

    heart failure 438 Pulmonary edema or congestion with normal or slightly enlarged heart 439 Cor pulmonale General overview 440 Azygos or hemiazygos vein enlargement 441 Asplenia syndrome 442 Systemic hypertension in childhood General overwview 443 Surgical [books.google.com]

  • Pseudotumor

    […] of the gastrointestinal tract in infancy and childhood 508 Benign pneumatosis 509 Reduced intestinal gas 510 Increased distance between the intestinal loops 511 Intraabdominal cysts in children 512 Migratory peritoneal calcifications 514 Peritoneal pseudotumors [books.google.com]

Respiratoric

  • Pleural Effusion

    effusion 350 Pleural effusion with or without pulmonary pathology 351 Parietal pleural thickening without pleural fluid 352 Localized pleural thickening in children 353 The Mediastinum and the Diaphragm Mediastinum 1 Pneumomediastinum in infancy and [books.google.com]

Cardiovascular

  • Heart Disease

    It is an autosomal dominant disorder and the second most common genetic syndrome of congenital heart disease. Its incidence is 1 in 1000–2500 births. Congenital heart diseases occur in 50%–80% of individuals. [annals.in]

    disease, esp AVSD (less commonly ASD/VSD) - hypothyroidism - megaloblastic luekaemia + lymphoproliferative disorders (solid tumoure rare in Tri21) - immune dysfunction - diabetes - hearing and vision problems - Alzheimer's disease-like dementia (4th [quizlet.com]

    […] repair procedures for congenital heart disease 444 Indentation on the esophageal wall 466 Inadequate peristalsis of the esophagus or neuromuscular deficit 467 Gastroesophageal reflux in the infantile period 468 Esophagitis 469 Dilated or wide esophagus [books.google.com]

    The common anomalies seen are congenital heart disease (44%), head and neck (48%), skeletal (22%) and genitourinary tract anomalies (24%) [ 1 ]. [ncbi.nlm.nih.gov]

  • Hypertension

    […] pattern 434 Pulmonary veins engorgement 435 Congestive heart failure 438 Pulmonary edema or congestion with normal or slightly enlarged heart 439 Cor pulmonale General overview 440 Azygos or hemiazygos vein enlargement 441 Asplenia syndrome 442 Systemic hypertension [books.google.com]

    He claimed to have hypertension and to have undergone cranioplasty for the treatment of craniosynostosis when he was seven and nine months old. Ten years ago, he underwent bilateral corneal transplantation. [medical-case-reports.imedpub.com]

    The findings are dwarfism, webbed neck, low hairline, epicanthal folds, mandibular deformity, webbed elbows and knees, coarctation of the aorta, hypertension, lymphedema of the hands and feet, and mental retardation. [emedicine.medscape.com]

Liver, Gall & Pancreas

  • Jaundice

    Intraabdominal cysts in children 512 Migratory peritoneal calcifications 514 Peritoneal pseudotumors 515 The Liver 1 Hepatomegaly in the newborn period 516 Hepatomegaly in children General overview 517 Hepatomegaly with portal hypertention and obstructive jaundice [books.google.com]

  • Hepatomegaly

    […] in the newborn period 516 Hepatomegaly in children General overview 517 Hepatomegaly with portal hypertention and obstructive jaundice 519 Liver calcifications in the pediatric age 520 حقوق النشر [books.google.com]

Jaw & Teeth

  • Receding Chin

    Background : Micrognathia is usually associated with genetic syndromes, characterized by mandibular hypoplasia causing a receding chin. The overall incidence of micrognathia was 1 per 1600 births, makes it a rare case. [jprjournal.com]

Skin

  • Ulcer

    Chapter on the diagnosis of oral lesions and developmental anomalies uses tables and extensive illustrations to depict developmental anomalies, white soft tissue lesions and enlargements, dark soft tissue lesions, ulcerative lesions, radiolucent lesions [books.google.com]

Musculoskeletal

  • Fracture

    PEDIATRIC FRACTURE Common - 39% Young bone possesses unique physical properties that coupled with space occupying developing dentition give rise to patterns of fracture not seen in adults. [slideshare.net]

    […] breast M Lejour 208 Facelift in a bloodless plane by the use of microelectric dissection techniques M B Habal 223 Contents 239 The combined graft of bone and fascia for augmentation rhinoplasty M Shirado 245 Direct transcutaneous reduction of zygoma fractures [books.google.com]

    The essential features of CCMS are mandibular hypoplasia and also gap defects of many ribs that possibly could simulate rib fractures. [mandibulartori.com]

    The bony fragments are held in place during the first week following surgical fracture to allow callus to form between the fragments. [aetna.com]

    Treatment of condylar fracture using a functional appliance in a 6-year-old child. Facial Orthop Temporomandibular Arthrol 1986;3:3-5. 10. Chate RA. The propellant unilateral magnetic appliance (PUMA): A new technique for hemifacial microsomia. [mjdrdypu.org]

  • Ankylosis

    Associated anomalies included temporomandibular joint ankylosis in five patients, aglossia/microglossia in three patients, and rare craniofacial clefts in three patients. [ncbi.nlm.nih.gov]

    Summary By using distraction osteogenesis technique, 3 cases of mandibular hypoplasia were treated by home-made and German-made jaw distractors: including one patient suffered from bilateral ankylosis of temporo-mandibular joint and 2 patients from deficiency [link.springer.com]

    Acquired condylar hypoplasia may develop after the loss of one or both condylar growth centers in very early stages of life and is sometimes accompanied by ankylosis. [omicsonline.org]

    As the clinical findings were consistent with those of TMJ ankylosis a working diagnosis of early ankylosis of the right TMJ was made. [mjdrdypu.org]

    Absence of any history of premature birth, prenatal or postnatal trauma rules out the possibility of TMJ ankylosis congenitally or due to trauma. [jofs.in]

Workup

X-Ray

  • Atelectasis

    […] chest in the newborn 283 Unilateral hyperlucent thorax in the newborn 284 Coarse parenchymal markings with irregular bilateral changes and hyperinflation in the newborn 285 Cystlike structures in the lung parenchyma of the newborn 286 Neonatal platlet atelectasis [books.google.com]

  • Colonic Stricture

    Single or multiple 494 Colitis in children 495 Colon stricture or narrowing 496 Segmental colonic narrowing in children 497 Toxic megacolon 498 Distention of the small andor large bowelnonobstructive paralytic ileus 499 Distended large bowel 500 Large [books.google.com]

  • Hyperlucent Lungs

    […] parenchyma 57 Hyperinflationhyperaeration in infants 312 Unilateral hyperinflation or unilateral hyperlucent lung 313 Bilateral underaeration of the lungs in the pediatric age 315 Pneumonia pattern in children 316 Acute alveolar disease 317 Lobar pneumonia [books.google.com]

Pleura

  • Pleural Effusion

    effusion 350 Pleural effusion with or without pulmonary pathology 351 Parietal pleural thickening without pleural fluid 352 Localized pleural thickening in children 353 The Mediastinum and the Diaphragm Mediastinum 1 Pneumomediastinum in infancy and [books.google.com]

Treatment

AIM OF THE WORK: To evaluate the long-term efficacy of intra-oral bilateral DO in the treatment of severe congenital mandibular hypoplasia in early childhood. [ncbi.nlm.nih.gov]

Ohura T Sugihara H Minakawa 66 Utilization of premolded free flaps J MoraisBesteiro F E Aki and M Castro Ferreira 85 Selective skeletonization in the functional and aesthetic correction of the nose A Mastroianni 118 Split calvarial bone grafting in the treatment [books.google.com]

The surgical treatment depends on the degree of deformity. [westvillageplasticsurgery.com]

Treatment is aimed at improving the facial aesthetics as well as the functional bite (occlusion). Dr. [craniony.com]

Deferring treatment until the patient is closer to completion of facial growth (girls, 15 years old; boys, 17–18 years old) helps minimize subsequent contralateral normal growth effect on the treatment outcome. [drlarrywolford.com]

Prognosis

Prognosis Prognosis is variable and depends on the presence and severity of associated cardiovascular, neurological and other complications. [patient.info]

The overall prognosis is highly variable dependent on the presence of other associated anomalies. Distraction osteogenesis is a surgical procedure that may be helpful. [texaschildrens.org]

[…] operations for improving velopharyngeal speech problems H Salo M L Haapanen J Hukki J Rautio 511 Flap skin graft 563 Basic research 618 Surgical anatomy of the orbit of Korean adults K Hwang and S H Baik 631 cmyc Oncoprotein acts as a marker of poor prognosis [books.google.com]

The prognosis is generally favourable. We present a very rare case report of bony fusion between the maxillary and mandibular alveoli without any other associated anomaly. [ispub.com]

Thus, corroborating above statements that hypoglossia with limb anomalies has a poor prognosis. Tongue is the most agile, adept and versatile appendage in the human body. [ijdr.in]

Etiology

• Cause is unknown, but local circulating problems, endocrine disturbances, and trauma have been suggested as possible etiologic factors. 38. [slideshare.net]

It often appears during puberty affecting predominantly males. [6] The exact etiology of coronoid hyperplasia is not yet elucidated, but several theories have been postulated, including hyperactivity of the temporal muscle that causes reactive elongation [mjdrdypu.org]

[…] interstitial and granulonodulo alveolar pattern 324 Interstitialreticular pulmonary pattern 326 Disseminated interstitial pneumonitis 327 The miliary pattern of pneumonia 328 Short linear andor wedgeshaped densities 329 Pulmonary edema in infancy and childhood Etiology [books.google.com]

Treacher Collins syndrome: etiology, pathogenesis and prevention. Eur J Hum Genet. 2009;17:275-283. http://www.ncbi.nlm.nih.gov/pubmed/19107148 Jones NC, Lynn ML, Gaudenz K, et al. [rarediseases.org]

Several etiologies are associated with growth disorders and abnormalities in the formation of the mandibular condyle and related structures. [medical-case-reports.imedpub.com]

Epidemiology

Each age-specific section covers the physical, cognitive, emotional, and social changes that children experience, as well as the epidemiology of dental disease at that age. [books.google.com]

The epidemiology of anotia and microtia. J Med Genet. 1996;33(10):809-813. Mastroiacovo P, Corchia C, Botto LD, Lanni R, Zampino G, Fusco D. Epidemiology and genetics of microtia-anotia: a registry-based study on over one million births. [journalofethics.ama-assn.org]

Epidemiology Incidence is approximately 1 in 35,000 to 1 in 56,000 births. However, the incidence is higher (around 1 in 1,000) in those children with congenital deafness. [ 1 ] The male:female ratio is 3:2. Most cases are sporadic. [patient.info]

Tempromandibular joint disorders Causes & epidemiology In 1930’s, JB Costen, an otolaryngologist, stated that TMD is a different disease process from otalgia, and is a condition derived from structural malalignment between mandible and cranium which requires [jkamprs.springeropen.com]

Pathophysiology

Pierre Robin syndrome pathophysiology of obstructive episodes. Laryngoscope 1969; 79:547-60. 13. Moyers RE, Enlow DH. Growth of the craniofacial skeleton. In: Moyers RE, editor. Handbook of Orthodontics. 4 th ed. [jofs.in]

Familiarity with craniofacial embryology and its associated effects on resultant anatomy also leads to a better understanding of the pathophysiologic basis of craniofacial syndromes. [ajnr.org]

Int J Oral Maxillofac Surg 28:411–8 PubMed View Article Google Scholar Gunson MJ, Arnett GW, Milam SB (2012) Pathophysiology and pharmacologic control of osseous mandibular condylar resorption. [jkamprs.springeropen.com]

Prevention

Other chapters explore the examination, treatment planning, radiographic concerns, prevention, trauma, restorative dentistry, pulp therapy, orthodontics, and behavior management of each age range. [books.google.com]

Prevention of Treacher Collins syndrome craniofacial anomalies in mouse models via maternal antioxidant supplementation. [rarediseases.org]

Fortunately, you can prevent many tooth disorders by taking care of your teeth and keeping them clean. [icdlist.com]

In children, the bone dense barriers may not yet have developed to the point where this prevention occurs. [scielo.br]

Therefore there may be no prevention techniques available. Treatment: Treatment for mandibular hypoplasia requires reconstructive surgery. [medigoo.com]

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2.1
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