Introduction male circumcision is a common procedure, generally
performed during the newborn period. Few reports have described
circumcision in patients with bleeding disorders. Aim to determine
bleeding rate after circumcision in neonatal male subjects who were
diagnosed later in life with delta-storage pool disease (SPD). Methods
we retrospectively reviewed the medical records of male subjects
(<18 years of age) who were diagnosed with SPD later in life
and were circumcised at birth without hemostatic prophylaxis due to lack
of family history at that time from 2000-2020.
Intraoperative/postoperative bleeding and bleeding severity were the
main outcomes evaluated. Results 153 male subjects were included.
Circumcision was performed at a median age of 2 days (range, 1 day-4
months). The main indication for circumcision was parental request.
Median severity of granule deficiency was 2.76 dense granules/platelet
(range, 1.12-3.82 DG/Plt). None of the subjects had intraoperative
bleeding. Three subjects (2%) had postoperative bleeding and only one
(0.65%) required ER intervention to stop bleeding. Conclusion the
overall incidence of bleeding in our subjects with SPD who were
undiagnosed and untreated at circumcision, is comparable to that
reported for patients without a bleeding disorder.
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