Pathology of the umbilical cord in adrenal fusion syndrome.
We report the placental findings of a newborn with multiple anomalies that include small and fused adrenal glands, meningomyelocele, lobar holoprosencephaly, cerebellar hypoplasia, short pancreas, and small spleen, fitting with the features of the so-called adrenal fusion syndrome. The placenta was small and presented a short, undercoiled and segmentally uncoiled umbilical cord with a short constriction in the middle of its length. We speculate that adrenal fusion syndrome and abnormal spleen lobulation and short pancreas may represent part of the same complex and probably related to the one including midline anomalies and defects of laterality formation in which horseshoe (fused) adrenal gland also may be found. Undercoiled umbilical cord might represent the placental component of this malformation complex. We also underline the value in identifying abnormalities of the umbilical cord to establish the cause of death.
Drut
Ricardo
R
Servicio de Patología, Hospital de Niños, La Plata, Argentina. patologi@netverk.com.ar
Mónica Drut
Rosa
R
D000015:Abnormalities, Multiple / Q000473:pathology*; D000311:Adrenal Glands / Q000002:abnormalities*; D005260:Female; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000401:mortality / Q000473:pathology*; D007234:Infant, Premature; D013577:Syndrome; D014470:Umbilical Cord / Q000473:pathology*