developmental dysplasia of the hip

dysplasia /dys·pla·sia/ (dis-pla´zhah)

1. abnormality of development.

2. in pathology, alteration in size, shape, and organization of adult cells.dysplas´tic

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anhidrotic ectodermal dysplasia  an inherited disorder characterized by ectodermal dysplasia associated with aplasia or hypoplasia of the sweat glands, hypothermia, alopecia, anodontia, conical teeth, and facial abnormalities.

anteroposterior facial dysplasia  defective development resulting in abnormal anteroposterior relations of the maxilla and mandible to each other or to the cranial base.

arrhythmogenic right ventricular dysplasia  a congenital cardiomyopathy in which transmural infiltration of adipose tissue results in weakness and bulging of regions of the right ventricle and leads to ventricular tachycardia arising in the right ventricle.

bronchopulmonary dysplasia  a chronic lung disease of infants, possibly related to oxygen toxicity or barotrauma, characterized by bronchiolar metaplasia and interstitial fibrosis.

chondroectodermal dysplasia  achondroplasia with defective development of skin, hair, and teeth, polydactyly, and defect of cardiac septum.

cretinoid dysplasia  a developmental abnormality characteristic of cretinism, consisting of retarded ossification and smallness of the internal and sexual organs.

developmental dysplasia of the hip  (DDH) instability of the hip joint leading to dislocation in the neonatal period; it may be associated with various neuromuscular disorders or occur in utero but occurs most commonly in neurologically normal infants and is multifactorial in origin. Formerly called congenital dislocation of the hip.

diaphyseal dysplasia  thickening of the cortex of the midshaft area of the long bones, progressing toward the epiphyses, and sometimes also in the flat bones.

ectodermal dysplasia  any of a group of hereditary disorders involving tissues and structures derived from embryonic ectoderm, including anhidrotic ectodermal dysplasia, hidrotic ectodermal dysplasia, and EEC syndrome.

epiphyseal dysplasia  faulty growth and ossification of the epiphyses with radiographically apparent stippling and decreased stature, not associated with thyroid disease.

fibromuscular dysplasia  dysplasia with fibrosis of the muscular layer of an artery wall, with collagen deposition and hyperplasia of smooth muscle, causing stenosis and hypertension; seen most often in renal arteries, it is a major cause of renovascular hypertension.

fibrous dysplasia of bone  thinning of the cortex of bone and replacement of bone marrow by gritty fibrous tissue containing bony spicules, causing pain, disability, and gradually increasing deformity; only one bone may be involved (monostotic fibrous d.), with the process later affecting several or many bones (polyostotic fibrous d.) .

florid osseous dysplasia  an exuberant form of periapical cemental dysplasia resembling diffuse sclerosing osteomyelitis but not inflammatory.

hidrotic ectodermal dysplasia  an inherited disorder of ectodermal dysplasia with tooth abnormalities, hypotrichosis, cutaneous hyperpigmentation over joints, and hyperkeratosis of the palms and soles.

metaphyseal dysplasia  a disturbance in enchondral bone growth, failure of modeling causing the ends of the shafts to remain larger than normal in circumference.

oculodentodigital dysplasia  a rare autosomal dominant condition, characterized by bilateral microphthalmos, abnormally small nose with anteverted nostrils, hypotrichosis, dental anomalies, camptodactyly, syndactyly, and missing phalanges of the toes.

periapical cemental dysplasia  a nonneoplastic condition characterized by formation of areas of fibrous connective tissue, bone, and cementum around the apex of a tooth.

septo-optic dysplasia  a syndrome of hypoplasia of the optic disk with other ocular abnormalities, absence of the septum pellucidum, and hypopituitarism leading to growth deficiency.

spondyloepiphyseal dysplasia  hereditary dysplasia of the vertebrae and extremities resulting in dwarfism of the short-trunk type, often with shortened limbs due to epiphyseal abnormalities.

thanatophoric dysplasia  a uniformly fatal type of skeletal dysplasia presenting as extreme shortness of the limbs, thoracic cage deformity, and relative enlargement of the head.

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developmental dysplasia of the hip (DDH),

instability of the hip joint leading to dislocation in the neonatal period. Although it may be associated with various neuromuscular disorders, such as myelodysplasia, or occur in utero, it most commonly occurs in neurologically normal infants and is multifactorial in origin. Usually there is laxity of the hip ligaments. Most affected infants are firstborn children, and 30% to 50% present in the breech position. About 90% of those affected are girls. The condition was formerly called congenital dislocation of the hip, but because the dislocation is not normally present at birth but develops later, the term developmental dysplasia of the hip is preferred.

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dysplasia [dis-pla´zhah]

an abnormality of development; in pathology, alteration in size, shape, and organization of adult cells. See also dysgenesis. adj., adj dysplas´tic.

bronchopulmonary dysplasia chronic lung disease of premature infants with hyaline membrane disease who have needed high concentrations of oxygen and assisted ventilation. Factors related to its development include alveolar damage due to hyaline membrane disease, oxygen toxicity, positive pressure ventilation, and endotracheal intubation. Treatment includes supportive measures and oxygen therapy. Recovery and normal pulmonary function usually occur by the age of 6 months to 1 year; however, some infants may exhibit limited tolerance to exercise.

craniometaphyseal dysplasia metaphyseal dysplasia associated with overgrowth of the head bones, leonine facies, and increased distance between the eyes.

Craniometaphyseal dysplasia. From Dorland's, 2000.

cretinoid dysplasia a developmental abnormality characteristic of cretinism, consisting of retarded ossification and smallness of the internal and reproductive organs.

cystic renal dysplasia renal dysplasia in which there are cysts.

developmental dysplasia of the hip (DDH) instability of the hip joint leading to dislocation in the neonatal period. Although it may be associated with various neuromuscular disorders, such as myelodysplasia, or occur in utero, it most commonly occurs in neurologically normal infants and is multifactorial in origin. Usually there is laxity of the hip ligaments. Most affected infants are first-born children and 30 to 50 per cent present in the breech position. About 90 per cent of those affected are girls. The condition was formerly called congenital dislocation of the hip, but because the dislocation is not normally present at birth but develops later, the term developmental dysplasia of the hip is preferred.

ectodermal dysplasia any of a group of hereditary disorders involving absence or deficiency of tissues and structures derived from the embryonic ectoderm, such as teeth, hair, nails, and certain glands.

fibromuscular dysplasia dysplasia with fibrosis of the muscular layer of an artery wall, with collagen deposition and hyperplasia of smooth muscle, causing stenosis and hypertension. It most commonly occurs in the renal arteries and is a major cause of renovascular hypertension.

fibrous dysplasia of bone thinning of the cortex of bone and replacement of bone marrow by gritty fibrous tissue containing bony spicules, causing pain, disability, and gradually increasing deformity; it may affect a single bone (monostotic fibrous dysplasia) or several or many bones (polyostotic fibrous dysplasia). When associated with melanotic pigmentation of the skin and endocrine disorders, it is known as albright's syndrome.

metaphyseal dysplasia a disturbance in enchondral bone growth, failure of modeling causing the ends of the shafts to remain larger than normal in circumference.

oculodentodigital dysplasia a rare hereditary condition, characterized by bilateral microphthalmos, abnormally small nose with anteverted nostrils, hypotrichosis, dental anomalies, camptodactyly, syndactyly, and missing phalanges of the toes.

renal dysplasia a congenital disorder of the kidney, with persistence of cartilage, undifferentiated mensenchyme, and immature collecting tubules, as well as with abnormal lobar organization and nearly always cysts; it may be unilateral or bilateral and total or subtotal. Total bilateral dysplasia is rapidly fatal in the neonatal period, while milder disease may be asymptomatic.

retinal dysplasia a general term for a congenital defect resulting from the abnormal growth and differentiation of a retina that fails to develop into functioning tissue.