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Illustrated operative management of spontaneous bleeding and compartment syndrome of the lower extremity in a patient with acquired hemophilia A: a case report.

Jentzsch T, Brand-Staufer B, Schäfer FP, Wanner GA, Simmen HP - J Med Case Rep (2014)

Bottom Line: There are no reports on operative management of this entity.He was treated surgically with a long and complicated postoperative course after presenting to a community hospital with a 2-day history of increasing pain and swelling in his left lower leg without a previous history of trauma.However, an interdisciplinary approach with meticulous surgical treatment and bleeding management with recombinant factor VIIa as well as inhibitor eradication by immunosuppressive treatment can be successful and expensive.

View Article: PubMed Central - HTML - PubMed

Affiliation: Division of Trauma Surgery, Department of Surgery, University Hospital Zürich, Rämistrasse 100, 8091 Zürich, Switzerland. thorsten.jentzsch@usz.ch.

ABSTRACT

Introduction: Spontaneous bleeding resulting in compartment syndrome at the lower adult leg due to acquired hemophilia A is rare. There are no reports on operative management of this entity.

Case presentation: We present a case of atraumatic compartment syndrome of the lower leg due to acquired factor VIII deficiency, in an 83-year-old Caucasian man of European descent. He was treated surgically with a long and complicated postoperative course after presenting to a community hospital with a 2-day history of increasing pain and swelling in his left lower leg without a previous history of trauma.

Conclusions: Awareness, prompt diagnosis and effective treatment of compartment syndrome caused by a rare bleeding disorder, which is usually acquired by the elderly, is essential and may spare a patient from surgery or even limb loss, if early administration of recombinant factor VIIa is effective. The course of disease in a patient with operative management of spontaneous bleeding, compartment syndrome and acquired hemophilia A may be prolonged. However, an interdisciplinary approach with meticulous surgical treatment and bleeding management with recombinant factor VIIa as well as inhibitor eradication by immunosuppressive treatment can be successful and expensive.

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Special wound dressings. a. Wound dressing on lower leg with PVA foamand jetting hose. b. Bulky absorbable wound dressing on lowerleg.
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Figure 1: Special wound dressings. a. Wound dressing on lower leg with PVA foamand jetting hose. b. Bulky absorbable wound dressing on lowerleg.

Mentions: In order to halt the uncontrollable bleeding, the wound was covered with a specialdressing with polyvinyl alcohol (PVA) foam, a jetting hose through which the woundwas kept moist and a big bulky absorbable dressing (Figures 1a, 1b). On day 15, implementation of VAC therapyfailed again and uncontrollable bleeding led to further therapy with the specialdressing mentioned above (Figures 2a and 3). By this time, the patient had received at least seven units of redblood cells and almost 20 administrations of NovoSeven® (rFVIIa), usually inthe dose of 7mg each. No other blood products were used except for 1g of tranexamicacid intraoperatively on his arrival at the university hospital. At that point hisfactor VIII was still 3%. For the next 2 weeks, dressing changes wereundertaken at regular intervals of approximately 3 days. Administration ofrFVIIa at regular intervals, at least once a day just shortly before dressingchanges was continued. His factor VIII started to rise slowly, beginning on day 17with 9%. After 4 weeks, his factor VIII had risen to 41% and VAC therapy couldbe applied again in order to condition the wound for definite closure(Figure 2b). After another VAC change, and rise offactor VIII to 88%, a split-thickness skin graft was carried out 1.5 monthsafter the first fasciotomy. At this time, rFVIIa support was no longer necessary.After leaving a VAC on top of the split-thickness skin graft for 5 days, milddelayed wound healing was observed in the anterior middle and posterior distal partsof the wound (Figure 4a). This was treated withAQUACEL® (primary wound dressing) and Mepitel® (wound contactlayer), (wound contact layer), while the rest of the wound was dressed with drypadding. In the meantime, factor VIII had increased >100% and corticosteroidtherapy could be reduced in a stepwise fashion approximately twice a month. Afterdischarging the patient into a rehabilitation clinic 2 months postoperatively,he was frequently seen in our out-patient clinic (Figure 4b). After 3.5 months, small parts of the wound had still nothealed (Figure 4c), and a split-thickness skin graft wascarried out for a second time (Figure 5a to 5c) Afterward, wound healing went slowly, but finally succeededapproximately 5 months after his initial presentation while his factor VIIIremained normal and prednisone was stopped 5 months after diagnosis(Figure 6). He had 5 out of 5 muscle strengthglobally, but needed to ambulate with crutches after being confined to bed rest fora long time.


Illustrated operative management of spontaneous bleeding and compartment syndrome of the lower extremity in a patient with acquired hemophilia A: a case report.

Jentzsch T, Brand-Staufer B, Schäfer FP, Wanner GA, Simmen HP - J Med Case Rep (2014)

Special wound dressings. a. Wound dressing on lower leg with PVA foamand jetting hose. b. Bulky absorbable wound dressing on lowerleg.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4109126&req=5

Figure 1: Special wound dressings. a. Wound dressing on lower leg with PVA foamand jetting hose. b. Bulky absorbable wound dressing on lowerleg.
Mentions: In order to halt the uncontrollable bleeding, the wound was covered with a specialdressing with polyvinyl alcohol (PVA) foam, a jetting hose through which the woundwas kept moist and a big bulky absorbable dressing (Figures 1a, 1b). On day 15, implementation of VAC therapyfailed again and uncontrollable bleeding led to further therapy with the specialdressing mentioned above (Figures 2a and 3). By this time, the patient had received at least seven units of redblood cells and almost 20 administrations of NovoSeven® (rFVIIa), usually inthe dose of 7mg each. No other blood products were used except for 1g of tranexamicacid intraoperatively on his arrival at the university hospital. At that point hisfactor VIII was still 3%. For the next 2 weeks, dressing changes wereundertaken at regular intervals of approximately 3 days. Administration ofrFVIIa at regular intervals, at least once a day just shortly before dressingchanges was continued. His factor VIII started to rise slowly, beginning on day 17with 9%. After 4 weeks, his factor VIII had risen to 41% and VAC therapy couldbe applied again in order to condition the wound for definite closure(Figure 2b). After another VAC change, and rise offactor VIII to 88%, a split-thickness skin graft was carried out 1.5 monthsafter the first fasciotomy. At this time, rFVIIa support was no longer necessary.After leaving a VAC on top of the split-thickness skin graft for 5 days, milddelayed wound healing was observed in the anterior middle and posterior distal partsof the wound (Figure 4a). This was treated withAQUACEL® (primary wound dressing) and Mepitel® (wound contactlayer), (wound contact layer), while the rest of the wound was dressed with drypadding. In the meantime, factor VIII had increased >100% and corticosteroidtherapy could be reduced in a stepwise fashion approximately twice a month. Afterdischarging the patient into a rehabilitation clinic 2 months postoperatively,he was frequently seen in our out-patient clinic (Figure 4b). After 3.5 months, small parts of the wound had still nothealed (Figure 4c), and a split-thickness skin graft wascarried out for a second time (Figure 5a to 5c) Afterward, wound healing went slowly, but finally succeededapproximately 5 months after his initial presentation while his factor VIIIremained normal and prednisone was stopped 5 months after diagnosis(Figure 6). He had 5 out of 5 muscle strengthglobally, but needed to ambulate with crutches after being confined to bed rest fora long time.

Bottom Line: There are no reports on operative management of this entity.He was treated surgically with a long and complicated postoperative course after presenting to a community hospital with a 2-day history of increasing pain and swelling in his left lower leg without a previous history of trauma.However, an interdisciplinary approach with meticulous surgical treatment and bleeding management with recombinant factor VIIa as well as inhibitor eradication by immunosuppressive treatment can be successful and expensive.

View Article: PubMed Central - HTML - PubMed

Affiliation: Division of Trauma Surgery, Department of Surgery, University Hospital Zürich, Rämistrasse 100, 8091 Zürich, Switzerland. thorsten.jentzsch@usz.ch.

ABSTRACT

Introduction: Spontaneous bleeding resulting in compartment syndrome at the lower adult leg due to acquired hemophilia A is rare. There are no reports on operative management of this entity.

Case presentation: We present a case of atraumatic compartment syndrome of the lower leg due to acquired factor VIII deficiency, in an 83-year-old Caucasian man of European descent. He was treated surgically with a long and complicated postoperative course after presenting to a community hospital with a 2-day history of increasing pain and swelling in his left lower leg without a previous history of trauma.

Conclusions: Awareness, prompt diagnosis and effective treatment of compartment syndrome caused by a rare bleeding disorder, which is usually acquired by the elderly, is essential and may spare a patient from surgery or even limb loss, if early administration of recombinant factor VIIa is effective. The course of disease in a patient with operative management of spontaneous bleeding, compartment syndrome and acquired hemophilia A may be prolonged. However, an interdisciplinary approach with meticulous surgical treatment and bleeding management with recombinant factor VIIa as well as inhibitor eradication by immunosuppressive treatment can be successful and expensive.

Show MeSH
Related in: MedlinePlus