Case report: sickle cell microembolism—a purported cause
of extreme DIPJ fixed flexion deformity?
Received: 18 July 2013 /Accepted: 23 September 2013 / Published online: 15 October 2013
Springer-Verlag Berlin Heidelberg 2013
Abstract The authors present a unique case of extreme distal
interphalangeal joint flexion contracture in a 21-year-old gen-
tleman. Although asymptomatic, he was concerned about the
cosmesis, which prompted his attendance. With no history of
trauma or other medical conditions, he reported a family
history of sickle cell trait, although was sickle cell negative
himself. This case illustrates that particularly in patients who
may be affected by blood dyscrasias (southeast Asian, Med-
iterranean and African) a ‘medical’ diagnosis for clinical
presentations which may not quite fit with the day to day
clinical workload for a hand surgeon should be borne in mind.
Some blood dyscrasias affect surgical outcomes as well an-
aesthetic safety for patients and taking a thorough history
where possible is crucial and can also affect the postoperative
Level of Evidence: Level V, diagnostic study.
Keywords Sickle cell
A hand surgeon’s practice comprises dealing with acute and
chronic as well as elective and traumatic upper limb condi-
tions ranging from nerve decompression surgery to excision
of ganglia to more complex tendon transfers and soft tissue
reconstruction. Occasionally, a hand surgeon may be required
to think outside the ‘surgical’ box and deal with uncommonly
seen conditions which may be present to other allied medical
professionals such as rheumatologists or internal medicine
physicians. The authors illustrate this point in the unique and
interesting case described below.
Sickle cell anaemia is a hereditary blood dyscrasia
characterised by abnormal sickle shaped red blood cells. This
sickling increases the cells’ overall malleability and can lead
to a myriad of clinical problems including vaso-occlusive
crises typified vaso-occlusive crises (VOC) including venous
thrombolic events, overwhelming postspenectomy sepsis,
avascular necrosis of joints to name but a few.
The authors present a case of extreme distal interphalangeal
joint (DIPJ) flexion contracture in a young Nigerian gentleman.
The 21 year-old right-handed computer science student had no
recollection of antecedent trauma. Since then, the index finger
gradually began to flex leading to an extreme fixed flexion
deformity. He was unable to recall the exact events from years
ago; however, blood tests confirmed sickle cell trait. He did not
recall any crises on exposure to extreme cold weather, pain or
dehydration. He was not on any medications of note nor used
any recreational drugs. At clinical presentation, he was keen to
explore his options in order to improve its form and function.
He complained of difficulty in performing keystrokes on his
keyboard and adapted by using his middle finger. He also
mentioned that he conceals his index finger wherever possible,
especially in social settings. Concerned about the aesthetic
appearance, he also reported that he finds it difficult being
introduced to new people, particularly handshakes.
We explained that from the history, this was an acquired
deformity, which has worsened over time. Given there was no
history of trauma, we hypothesise he had suffered a distal
microembolism resulting in ischaemia and flexion contracture.
Z. Ahmad (*)
Department of Plastic and Reconstructive Surgery,
UHCW, Coventry, UK
Eur J Plast Surg (2014) 37:171–172