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1999-07-24-03 AIDS embryopathy © Silva  www.thefetus.net/
Acquired Immunodeficiency syndrome embryopathy (AIDS embryopathy)

Sandra R Silva, MD, & Philippe Jeanty, MD, PhD

Definition: AIDS embryopathy is characterized by a group of dysmorphic features, which manifests either before or after birth in offsprings of women who are infected by HIV virus. Transplacental infection occurs generally in early gestation[1].

Synonym: Congenital AIDS, fetal AIDS, and fetal HIV infection.

Incidence: The transmission of HIV infection from mothers to newborns varies throughout the world from 14.1% in Europe, to 28% in New York, to 20% in San Francisco, and 45% in Africa[2]. Of all children infected with HIV virus, approximately 99% where infected from their mothers. Vertical transmission includes intrauterine, intrapartum and postpartum (breastfeeding) infections. Intrauterine or transplacental infection accounts for 30% to 50% of the total. The incidence of AIDS embryopathy is uncertain, and depends mainly on the severity of the maternal disease, and early intrauterine transmission (the earlier the transmission, the higher risk of fetal compromise).

Etiology: The causative agent is the HIV virus.

Recurrence risk: The risk of vertical transmission in another pregnancy will be the same if the fetus be exposed to the same risk factors (severity of maternal disease, high maternal viral load, low maternal CD4, maternal fever and anemia, prolonged ruptured of the membranes, membrane inflammation).

Diagnosis: Intrauterine diagnosis applying invasive techniques is precluded to avoid fetal exposure to maternal blood, and false positive results due to maternal cross-reaction. Ultrasonographic signs of fetal infection may be present, including: fetal demise, growth retardation, microcephaly, craniofacial abnormalities (present in 50%-75% of the affected, which confers a characteristic facial appearance to them after birth) including prominent, square or boxlike forehead, lateral bossing, hypertelorism, flat nasal bridge, and short nose. Other findings that may be present at birth include long palpebral fissures, blue scleras, prominent upper vermilion border, short stature, large wide eyes, and well-formed philtrum[3].

Associated anomalies: Maternal opportunistic diseases, in particular infections that may also affect the fetus such as toxoplasmosis, cytomegalovirus, and parvovirus.

Differential diagnosis: Other maternal viral infections, which can be excluded by maternal serology.

Prognosis: Up to now, AIDS is an uniformly lethal condition, with variable length of survival, which depends on multiple factors (maternal, infant, and viral). Mean survival ranges from 6.2 to 7.5 years, and only 70% reach 6 years of age[4]. Intrauterine death may occur in cases of severe maternal and/or fetal disease, in particular on those cases infected early in pregnancy[5]. The severity of congenital abnormalities seems to be associated with early fetal viremia (the earlier the infection, the more severe the compromise). Introduction of early antiviral therapy may reduce vertical transmission from 25% to 8%[6], and prolong the length of survival for those who are infected.

Management: Termination of pregnancy can be offered before viability for every patient infected with HIV virus. Monthly ultrasound evaluation throughout the pregnancy is recommended to search for growth restriction and structural anomalies. Maternal treatment with 100mg of zidovudine 5 times daily, throughout the pregnancy associated with postnatal treatment of the newborn with the same drug is mandatory. Prenatal maternal treatment with antiretrovirals is strongly reccomended. The most common regimen consists of 600mg daily of zidovudine throughout the pregnancy starting at 14 weeks (if the mother did not develop the disease yet). Special short-course zidovudine or nevirapine regimens for intrapartum prophylaxis are indicated. Cesarean section delivery before rupture of membranes and onset of labor approximately halves the risk of vertical transmission, and thus vaginal delivery should be avoided[7],[8],[9]. 

Acknowledgements: The authors would like to express their gratitude to Dr. Gisele Duboc for her assistance in the review and references of this manuscript.

Reference:


 

[1] Nyhan WL. Structural Abnormalities – A systematic approach to diagnosis in Clinical Symposia – Ciba-Geigy, 1990, 42(2): 31-32.

[2] Wara DW, Dorenbaum A. Pediatric AIDS: Perinatal transmission and early diagnosis in The medical management of AIDS, by Sande MA and Volberding PA. W B Saunders Company - Philadelphia, 1997, 469-473.

[3] Iosub S, Bamji M, Stone RK, Gromisch DS, Wasserman E. More on human immunodeficiency virus embryopathy. Pediatrics 1987, 80(4):  512-516.

[4] Ruiz Contreras J. Natural history of HIV infection in the child. Allergol. Immunopathol, 1998, 26(3): 135-139.

[5] Landers DV and Shannon MT. Management of pregnant women with HIV infection in The medical management of AIDS, by Sande MA and Volberding PA. W B Saunders Company - Philadelphia, 1997, 459-468.

[6] Connor E, Sperling R, Gelber R, et al. Reduction of maternal-infant transmission of human immunodeficiency virus type 1 with zidovudine treatment. N England J Med, 1994,  331: 1173-1180.

[7] Musoke P, Guay LA, Bagenda D, Mirochnick M, Nakabiito C, Fleming T, Elliott T, Horton S, Dransfield K, Pav JW, Murarka A, Allen M, Fowler MG, Mofenson L, Hom D, Mmiro F, Jackson JB. A phase I/II study of the safety and pharmacokinetics of nevirapine in HIV-1-infected pregnant Ugandan women and their neonates (HIVNET 006).  AIDS 1999 Mar 11;13(4):479-86

[8] Thorne C, Newell ML. Epidemiology of HIV infection in the newborn. Early Hum Dev 2000 Apr;58(1):1-16

[9] Fowler MG, Simonds RJ, Roongpisuthipong A. Update on perinatal HIV transmission. Pediatr Clin North Am 2000 Feb;47(1):21-38

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