Publication:


abstract

Abstract Adrenocortical cytomegaly (AC) is a relatively uncommon phenomenon, but tends to occur in certain situations including specific congenital anomalies and hydrops due to maternal-fetal Rhesus incompatibility. Since the pathology in the latter condition does not differ greatly from hemoglobin Bart hydrops fetalis, the authors performed a retrospective review of fetal and perinatal autopsy cases with hemoglobin Bart to determine the prevalence of AC in that condition. Over a 10-year period (2001-2010) at King Chulalongkorn Memorial Hospital, there were 16 hydropic cases confirmed to have hemoglobin Bart. AC was found in 13 cases (81%). For comparison, the authors determined the occurrence of AC in cases of hydrops fetalis not due to hemoglobin Bart (n=33) and a heterogeneous group of congenital anomalies (n=34). AC was identified only in one case of Beckwith-Wiedemann syndrome and two cases of anencephaly. Thus, AC is a common finding in cases of hemoglobin Bart, a finding not previously documented. Moreover, our study suggests that hemoglobin Bart is one of the conditions most commonly associated with AC. The reasons for this are not known. The mean hemoglobin levels for the hydrops cases with hemoglobin Bart and those with other forms of anemia showed no significant difference (p=0.63) nor was there any significant difference in hemoglobin levels between cases of hemoglobin Bart with and without AC. Nonetheless, the consistency of AC in cases of Hb Bart suggests that further study of this particular group of patients might shed light on the pathogenesis of this poorly understood pathologic finding.

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