A term male fetus suddenly and unexpectedly died in utero at the 40th week of gestation.
The mother had a regular and unremarkable pregnancy except for an anticardiolipin
antibodies positivity. The histological examination of the cardiac conduction system
showed islands of conduction tissue in the central fibrous body, known as persistent
fetal dispersion. The brainstem examination revealed a severe bilateral hypoplasia
of the arcuate nucleus. This morphological finding has been described in more than
35% of our stillborn as well as sudden infant death syndrome (SIDS) cases, independently
from the presence of anticardiolipin antibodies. Some authors have given emphasis
to the possible lethal association of maternal autoantibodies and QT prolongation.
Our findings emphasize the need of an accurate postmortem examination including the
study of brainstem and cardiac conduction system in every case of unexpected late
intrauterine death, following the same standardized autopsy protocol adopted in SIDS
cases.
KEYWORDS
Stillbirth - sudden unexplained intrauterine death - anticardiolipin antibodies
REFERENCES
- 1 Kalousek D K, Gilbert-Barness E. Causes of stillbirth and neonatal death. In: Gilbert-Barness
E Potter's Pathology of the Fetus and Infant. St. Louis, MO; Mosby 1997: 128-162
- 2
Matturri L, Minoli I, Lavezzi A M, Cappellini A, Ramos S, Rossi L.
Hypoplasia of medullary arcuate nucleus in unexpected late fetal death (stillborn
infants): a pathologic study.
Pediatrics.
2002;
109
E43
- 3
Matturri L, Biondo B, Suàrez-Mier M P, Rossi L.
Brain stem lesions in the sudden infant death syndrome: variability in the hypoplasia
of the arcuate nucleus.
Acta Neuropathol (Berl).
2002;
104
12-20
- 4 Rossi L, Matturri L, Ottaviani G. Sudden unexpected death of a term fetus with maternal
anti-cardiolipin antibodies. In: Proceedings of the 7th SIDS International Conference
Florence, Italy; August 31-September 4 2002: 137
- 5
Matturri L, Ottaviani G, Ramos S G, Rossi L.
Sudden infant death syndrome (SIDS): a study of cardiac conduction system.
Cardiovasc Pathol.
2000;
9
137-145
- 6
Rossi L.
Salvage the pacemaker at autopsy.
Am Heart J.
1978;
95
540-541
- 7 Olsezewski J, Baxter D. Cytoarchitecture of the Human Brain Stem. Basel, Switzerland;
Karger 1982
- 8
Askenazi S S, Perlman M.
Pulmonary hypoplasia: lung weight and radial alveolar count as criteria of diagnosis.
Arch Dis Child.
1979;
54
614-618
- 9
James T N, Marshall T K.
De subitaneis mortibus XVIII. Persistent fetal dispersion of the atrio-ventricular
node and His bundle within the central fibrous body.
Circulation.
1976;
53
1026-1034
- 10
Vinatier D, Dufour P, Cosson M, Houpeau J L.
Antiphospholipid syndrome and recurrent miscarriages.
Eur J Obstet Gynecol Reprod Biol.
2001;
96
37-50
- 11
Cimaz R, Stramba-Badiale M, Brucato A, Catelli L, Panzeri P, Meroni P L.
QT interval prolongation in asymptomatic anti-SSA/Ro-positive infants without congenital
heart block.
Arthritis Rheum.
2000;
43
1049-1053
- 12
Brucato A, Frassi M, Franceschini F et al..
Risk of congenital heart block in newborns of mothers anti-Ro/SSA antibodies detected
by counterimmunoelectrophoresis: a prospective study of 100 women.
Arthritis Rheum.
2001;
44
1832-1835
- 13
Matturri L, Lavezzi A M, Minoli I et al..
Association between pulmonary hypoplasia and hypoplasia of arcuate nucleus in stillbirth.
J Perinatol.
2003;
23
328-332
Luigi MatturriM.D. Ph.D.
Institute of Pathology, University of Milan
Via della Commenda, 19, 20122 Milan, Italy