CASE REPORT

Therapeutic embolization and surgical excisionof haemophilic pseudotumour

J. SEVILLA, M. T. ALVAREZ, D. HERNAÂ NDEZ, M. CANALES, J. G. DE BUSTOS, M. MAGALLOÂ N,

G. GARZOÂ N* and F. HERNAÂ NDEZ-NAVARRO

Hemophilia Unit, Hematology Service, Hospital General La Paz, Madrid, Spain; *Interventional Radiology Service,

Hospital General La Paz, Madrid, Spain

The iliac haemophilic pseudotumour occurs in only1%±2% of patients with a severe to moderatede®ciency in factor VIII or IX [1, 2]. It consists ofencapsulated blood collection, which appears in softtissue, periosteum and bone tissue, and grows due torecurrent haemorrages and the consequent increaseof pressure in its interior [2, 4, 5]. In this way itslowly destroys adjacent structures [2, 4, 5]. For thisreason, resection should be performed as early aspossible [6]. Very few cases of haemophilic pseudo-tumour of the iliac have been reported [1, 2]. Theusual radiological manifestations consist of liticosseous areas and areas of osseous neoformation,with calci®cation in adjacent soft tissue [4]. Inpseudotumours of the iliac, these later manifestationsmay be absent, with only litic osseous areas beingapparent [4]. These tumours may cause cutaneous®stulas [3] and may act as a focus for infection. Ifthey present perforations and infections of endoge-nous origin their course is usually fatal [1].

Treatments have been investigated on variousoccasions. Most of the literature agrees that theonly curative treatment is surgical resection [2, 4].Conservative treatments have also been tried withsubstitution therapy and immobilization, or radio-therapy [2, 7]. Another form of treatment used isthe puncture and aspiration of the pseudotumour,although currently it is widely agreed that thistreatment frequently causes ®stulas, posterior in-fections, and septic process [2, 4, 5]. Recently anew form of treatment has been reported, consist-ing of the aspiration of the content of the tumour.The resulting cavity is subsequently re®lled withhuman ®brin and Rifampicina, inducing local®brosis [8, 9], or with an osteal graft withoutremoving the pseudocapsule [10]. The case herepresented is a haemophilic pseudotumour of theiliac and caecum with cutaneous ®stulas, withseptic process of endogenous origin. Treatmentconsisted of surgical resection after arterial thera-peutic embolization.

Case report

The patient had moderate haemophilia B (FIX:C,0.25 UI mL±1), was male, 56 years old, without

Summary. Haemophilic pseudotumour is a rarecomplication of haemophilia. Few cases of iliachaemophilic pseudotumour have been reported inthe literature. These tumours can act as a focus forinfection and cause cutaneous ®stulas. When theypresent perforations and infections of endogenousorigin their course is usually fatal.

Suitable treatment has been investigated on nu-merous occasions, most of the literature agreeingthat the only curative treatment is surgical resection.

We present a case of haemophilic pseudotumourof the iliac and caecum with cutaneous ®stulas, witha septic process of endogenous origin. It was treatedwith surgical resection after performing arterialembolization to reduce the vascularization of thepseudotumour, thereby reducing its size and the riskof bleeding complications during surgery.

Keywords: arterial therapeutic embolization, haemo-philia, pseudotumour.

Correspondence: JuliaÂn Sevilla Navarro, Hemophilia Unit, Hema-tology Service, Planta semisotano, Hospital General La Paz, Paseode la Castellana 261, Madrid 28046, Spain.

Received 23 March 1998; accepted 22 March 1999

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circulant inhibitor, and with bleeding complicationssince his ®rst year. He was seropositive to HBV andHCV, and seronegative to HIV. He had cholelithia-sis, renal bilateral lithiasis and functional breakdownof the left kidney. Diagnosed with right iliac pseu-dotumour in 1977, he subsequently presented glutealand right upper thigh ®stulas and sepsis related to thedrainage of the ®stulas, one of which requiredattention in an intensive care unit.

In November 1996 he was admitted for drainageof purulent content and fecal waste from one of thecutaneous ®stulas. A CT scan revealed seriousdestructive changes in the right ilium in the formof round foci with unclear contours and accompa-nied by osteoesclerosis. A soft tissue mass wasobserved in the psoas area, consisting of ¯uidcollection with gas in it, communicating with thecaecum and skin. These pictures were suggestive ofan abscess in this area. An intestinal X-ray showed®stulas to the bowel. Shortly afterwards, arterialembolization of the pseudotumours' main nutrientvessels was carried out, with polivinyl particles andGianturco coils (complete distal embolization of thelumbar artery conserving the proximal vasculature,and also of the two appropriate nutrient vessels ofthe right femoral artery) (Fig. 1). Subsequently, therewas no apparent vascularization of the existinglesion in the right iliac (Fig. 2). Ninety days later,and after a new angiographic study showing thelesion little vascularized, surgical resection of thepseudotumour was undertaken. The substitutiontherapy was modi®ed before surgery and concen-

trates of ultrapure factor IX were administered incontinuous infusion.

During surgery it was observed that the lesion wassmaller in size than expected.

Resection was performed of two ®stulae fromcaecum to the pseudotumour cavity, and curettage ofits walls was also performed. The appendix was®brosed and its resection was done. Finally, a Dexonmesh was inserted into the cavity and secured with®ve stitches. After surgery the patient suffered acuteincisional hernia with compromise of a small bowelloop, which was operated upon. In this secondintervention the Dexon mesh was checked and wasfound to be ®xed in place. A local infection processwas also noticed around the surgical woundby Hafnia alvei, which was successfully treatedwith intraveneous Amikacina. Throughout thepostoperative period, the patient continued to receivesubstitution therapy with ultrapure factor IX, main-taining plasmatic levels of around 30%±80%.

One month after the intervention, a curettage wascarried out on the edges of the surgical bound. Ondischarge from hospital the wound was found to bein resolution, and healed at the third attempt.

Three months after the intervention, the patientpresented with cutaneous ®stulas and a closedsurgical wound. A radiological study using axialcomputerized tomography showed that the ®stulas tothe bowel were closed (con®rmed by opaque anema).In the previous location of the pseudotumour therewas a mass of liquid appearance with perilesional®brotic reaction. Eight months after the intervention,

7Fig. 1. Angiographic study showing main nutrient vessels of the

pseudotumour.Fig. 2. Angiographic study after the embolization.

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an increase in ®brotic tissue and a decrease in theliquid mass was observed (Figs 3 & 4).

Discussion

Treatment of the haemophilic pseudotumour iscurrently based on conservative measures or surgicalresections. The conservative measures consist of theadministration of substitutive therapy for long peri-ods of time, or local radiotherapy. On occasions,both procedures have been used together [7]. Themain obstacle to surgery is the dif®culty of thetechnique due to the size of the pseudotumour andthe heightened likelihood of substantial bleedingcomplications. Arterial embolization has been usedin our patient to diminish the vascularization of thepseudotumour and in this way reduce its size andbleeding during surgery. This technique is not dif®-cult and the arterial puncture performed did notshow bleeding using adecuated substitution therapyfor 4 days. The doses of factor IX administered werethose indicated to reach clearly haemostatic plas-matic levels. We have not found any similar therapydescribed in previous literature.

Another problem that affects the surgical treat-ment of iliac pseudotumours is the resulting deadspaces after its extirpation [1, 3]. In our patient this

space was closed with a Dexon mesh. This structurewas checked in the second intervention performed bypostsurgical eventration, and it was found to becorrectly situated closing the previous cavity of thepseudotumour.

The complications are those of any surgicalprocedure, and cannot be attributed in this case tothe de®cit of factor IX since in the controls perform-ed, this patient always had levels above 50%. Therewas a delay in the healing of the surgical woundrelated to the second intervention and the localizedinfection.

Arterial therapeutic embolization of haemophilicpseudotumours should be considered in tumours oflarge size, since it may effectively reduce its size anddecrease the risk of bleeding complications duringsurgery.

References

1 Iwata H, Oishi Y, Itoh A et al. Surgical excision ofHemophilic Pseudotumor of the ilium. Clin Orthop1992; 284: 234±8.

2 Ahlberg AKM. On the natural history of hemophilicpseudotumor. J Bone Joint Surg 1975; 57A: 1133±5.

3 Tezanos Pinto M, Nieto R, PeÂrez Bianco R. Hemophilicpseudotumor. In: Lusher JM and Kessner CM, ed. A

Fig. 3. CT scan showing the pseudotumour

cavity and contrast from bowel to pseudo-

tumour.

Fig. 4. CT scan showing an area of liquid

appearance with perilesional ®brotic reac-

tion in the pseudotumour area, with no

contrast from the bowel.

362 J. SEVILLA et al.

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Report of 25 Cases of Hemophilia and Von Wille-brand's Disease in the 1990s. Amsterdam: ElsevierScience Publishers, 1991: 165±78.

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