Human pathology

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alveolar capillary dysplasia

congenital alveolar capillary dysplasia and/or misalignment of lung vessels, congenital alveolar capillary dysplasia with misalignment of pulmonary veins, misalignment of pulmonary vessels (MPV) with alveolar capillary dysplasia (ACD), acinar dysplasia

Definition: complex vascular abnormality in the lungs causing progressive hypoxemia in the newborn.

Congenital alveolar capillary dysplasia is a rare cause of irreversible pulmonary hypertension with 100% mortality.

Synopsis

- possible familial occurence (3721495)

- macroscopicla examination

- persistent pulmonary hypertension of newborn
- dilated veins around pulmonary arteries
- pulmonary arteriole showing marked thickening of the medial musculature
- broad alveolar septa containing centrally located large alveolar capillaries
- artery is surrounded by an anomalous venous ring derived from branches of septal veins
- interstitial thickening
- markedly decreased septal capillaries
- poor development of the capillary-alveolar interface
- muscularized pulonary arteries within aleolar septa away from bronchioles
- centrally placed capillaries beneath the basement membrane of the alveolar lining cells and surrounded by loose mesenchyme
- ectatic veinswithin bronchovascular bundles, or within the adventia of the pulmonary arteries
- intermittent venous ring auround bronchioles
- small muscularized arteries in the acini, extending to the precapillary area
- severe pulmonary capillary hypoplasia
- normal anatomical and biochemical parenchymal maturation
- intractable pulmonary hypertension
- deficient capillaries in airspace walls
- anomalous small pulmonary veins in bronchiolar-arterial rays
- medial thickening in small pulmonary arteries
- peripheral arterial muscularization
- abnormally immature parenchymal development in the lungs
- underdevelopment of alveoli
- pulmonary interstitial fibrosis
- misalignment of lung vessels

  • the pulmonary veins joined the pulmonary arteries, rather than following a course away from them.

Associations

- phocomelia
- trisomy 21 (16944970, 16235130)
- duodenal atresia (17060189)
- cardiac malformations (congenital heart disease)

- asplenia
- fetal ureteric obstruction
- fetal urethral obstruction
- anorectal anomaly (17060189)
- intestinal malrotation (17060189)
- total colonic Hirschsprung disease (17060189)
- familial microphthalmia (17036407)
- anterior segment dysgenesis of the eye (11536028)
- trisomy 21 (16235130)
- hypoplastic left heart syndrome (HLH) (11178633)
- persistent pulmonary hypertension (PPH) (11178633)
- intestinal malrotation (11178633)
- intestinal obstruction (11178633)
- congenital pulmonary alveolar proteinosis (MIM.265120) (10378403)
- bilateral tibial agenesis with ectrodactyly (MIM.119100) (11754046)

Physiopathology

- The primary pulmonary vascular anomaly is likely to be a failure of fetal lung vascularization dating from the second trimester and to be due to action of an unknown teratogen.

- centroacinar veins may represent bronchial veins that do not normally develop beyond the ends of cartilaginous bronchi.

- Pulmonary arterial occlusive changes are interpreted as reactive to obstruction at the level of pulmonary arterioles.

Etiology

- germline mutations in STRA6 gene (17273977)
- trisomy 21 (16235130)

See also

- congenital alveolar capillary dysplasia with misalignment of pulmonary veins
- progressive hypoxemia
- persistent fetal circulation
- pulmonary capillary maturation

References

- Eulmesekian P, Cutz E, Parvez B, Bohn D, Adatia I. Alveolar capillary dysplasia: a six-year single center experience. J Perinat Med. 2005;33(4):347-52. PMID: 16207122

- Shehata BM, Abramowsky CR. Alveolar capillary dysplasia in an infant with trisomy 21. Pediatr Dev Pathol. 2005 Nov-Dec;8(6):696-700. PMID: 16235130

- Eulmesekian P, Cutz E, Parvez B, Bohn D, Adatia I.Alveolar capillary dysplasia: a six-year single center experience.J Perinat Med. 2005;33(4):347-52. PMID: 16207122

- Hugosson CO, Salama HM, Al-Dayel F, Khoumais N, Kattan AH.Primary alveolar capillary dysplasia (acinar dysplasia) and surfactant protein B deficiency: a clinical, radiological and pathological study.Pediatr Radiol. 2005 Mar;35(3):311-6. PMID: 15490144

- Rabah R, Poulik JM. Congenital alveolar capillary dysplasia with misalignment of pulmonary veins associated with hypoplastic left heart syndrome. Pediatr Dev Pathol. 2001 Mar-Apr;4(2):167-74. PMID: 11178633

- Gutierrez C, Rodriguez A, Palenzuela S, Forteza C, Rossello JL. Congenital misalignment of pulmonary veins with alveolar capillary dysplasia causing persistent neonatal pulmonary hypertension: report of two affected siblings. Pediatr Dev Pathol. 2000 May-Jun;3(3):271-6. PMID: 10742415

- Haraida S, Lochbühler H, Heger A, Nerlich A, Diebold J, Wiest I, Müller-Höcker J, Löhrs U.Congenital alveolar capillary dysplasia: rare cause of persistent pulmonary hypertension.Pediatr Pathol Lab Med. 1997 Nov-Dec;17(6):959-75. PMID: 9353836

- Cullinane C, Cox PN, Silver MM. Persistent pulmonary hypertension of the newborn due to alveolar capillary dysplasia. Pediatr Pathol. 1992 Jul-Aug;12(4):499-514. PMID: 1409149

- Langston C. Misalignment of pulmonary veins and alveolar capillary dysplasia. Pediatr Pathol. 1991 Jan-Feb;11(1):163-70. PMID: 2014189

- Khorsand J, Tennant R, Gillies C, Phillipps AF. Congenital alveolar capillary dysplasia: a developmental vascular anomaly causing persistent pulmonary hypertension of the newborn. Pediatr Pathol. 1985;3(2-4):299-306. PMID: 4095026

- Wagenvoort CA. Misalignment of lung vessels: a syndrome causing persistent neonatal pulmonary hypertension. Hum Pathol. 1986 Jul;17(7):727-30. PMID: 3721495

- Janney CG, Askin FB, Kuhn C 3rd. Congenital alveolar capillary dysplasia—an unusual cause of respiratory distress in the newborn. Am J Clin Pathol. 1981 Nov;76(5):722-7. PMID: 7293984

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