[13] Postmortem Assessment of Polysplenia and Asplenia Syndromes: What Do Complex Congenital Anomalies Tell Us about Autopsy Quality?

Lisa Rooper, Michelle Olson, Katie Flickinger, Elizabeth C Burton. Johns Hopkins Hospital, Baltimore, MD

Background: While autopsy is often cited as a quality assurance tool for hospitals, opportunities are limited for examining the quality of autopsies themselves. Polysplenia and asplenia syndromes, related congenital disorders that include splenic abnormalities and various cardiac and visceral malformations, provide a unique setting in which to assess the completeness of autopsy practice. This study analyzed autopsied cases of polysplenia and asplenia syndromes at a large tertiary care center to determine how effectively a general autopsy service evaluates these conditions.
Design: A database of autopsies performed from May 28, 1889 to January 30, 2012 was searched for all cases of asplenia or polysplenia. Cases were included if there was absence of spleen or presence of multiple spleens as well as one or more other anomalies frequently associated with polysplenia or asplenia syndrome. Autopsy records were reviewed for each case, and splenic, cardiac, and visceral morphology was noted.
Results: 37 cases met criteria for asplenia and 58 cases for polysplenia. Reports mentioned a specific diagnosis of asplenia syndrome in 17 (46%) of cases with no spleen and polysplenia syndrome in 33 (56%) of cases with multiple spleens. A total of 28 (76%) of asplenia cases and 56 (97%) of polysplenia cases did not list any findings, normal or abnormal, for one or more commonly involved organs. Overall frequencies of missing observations ranged from 18% to 81% (see table). Additionally, frequencies of observed anomalies varied widely from previously published values.

Missing observations in asplenia and polysplenia cases
Absent observationAsplenia n(%)Polysplenia n(%)Total n(%)
Spleen position-21(36)21(36)
Spleen number-46(79)46(79)
Lung lobation9(24)13(22)22(23)
Liver morphology13(35)28(48)41(43)
Stomach position20(54)38(66)58(61)
Intestinal rotation9(24)8(14)17(18)
Cardiac position24(65)46(79)70(74)
Atrial isomerism26(70)46(79)72(76)
Systemic venous return21(57)21(36)42(44)
Pulmonary venous return26(70)51(88)77(81)

Conclusions: Polysplenia and asplenia syndromes are rare disorders that are unfamiliar to many autopsy pathologists. However, this study illustrates widespread gaps in autopsy findings that may reflect a more basic lack of completeness in postmortem examination or reporting. It also raises the question of whether standardization of autopsy practice could improve the quality of results, regardless of diagnosis. A checklist is proposed to guide pathologists in better evaluation of polysplenia and asplenia cases.
Category: Autopsy

Wednesday, March 6, 2013 9:30 AM

Poster Session V # 4, Wednesday Morning


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