Ileopsoas Hemophilic Pseudotumour with Externalized Bowel Fistulation

A case of ileopsoas hemophilic pseudotumour is presented. Its particularity is a fistula, developed between the pseudotumour and the large bowel, followed later by a second externalized fistula on the abdominal wall. A large abscess involving the pseudotumour and chronic sepsis developed as life-threatening complications.

Diversion colostomy with anus praeter and drainage under FVIII substitution resolved the unusual, severe clinical condition after almost 6 years of evolution.

Introduction

Hemophilic pseudotumour (HPT) is a well organized, encapsulated, progressive blood mass, expanding, compressing and frequently eroding local tissues.

Usually rare, but severe complication of hemophilia and related disorders, HPT is unfortunately quite frequent in developing countries.

Out of a total of 24 patients with HPT (17 proximal and 7 distal) we present a most impressive case. It illustrates the natural history of ileopsoas hematoma with all its burden of complications, focusing the attention on the unusual aspect of HPT.

Case Presentation

M.A. is a 30-year-old man, with severe form of type A hemophilia (Factor VIII con- centration <0.5%; inhibitor status- 0 BU). He presents chronic hemophilic arthro- pathy in the following target joints: knees, right shoulder and left elbow and a total clinical joint score of 35.

He experienced a left ileopsoas hematoma (IPH) in July 1997 (Fig. 1): he de- veloped pain and swelling in the left flank, loss of sensation in the femoral nerve distribution of the left leg, weakness of quadriceps and anemia. He was treated with regular cryoprecipitate and supplementation of FVIII concentrate for 14 days. Over the next 9 months he recovered partially, but in April 1998 his clinical status wor- sened suddenly: a huge abdominal mass appeared in the left flank and fossa (Fig. 2), followed by a posthemorrhagic shock. Surgical removal and drainage was attemp- ted under a substitutive treatment with FVIII concentrate and cryoprecipitate.

I. Scharrer/W. Schramm (Ed.)

34

Hemophilia Symposium Hamburg 2003

” Springer Medizin Verlag Heidelberg 2005

Repositioning of drains was intermittently required. After 2 months he was dischar- ged in stable condition.

March 2002 marked the start of serious worsening in the patient’s evolution. He presented septic fever, with pain in the left iliac fossa, diarrhea and a nasal and malar cutaneous redish erruption. He lost 13 kg in weight. He was serologically negative for HIV, but positive for HCV; his urine and stool cultures remained nega- tive. FVIII inhibitors and autoantibodies (anti-DNA, lupus cells, and rheumatoid factor) were negative. In blood cultures grew Klebsiella. The CT scan revealed that the HPT had doubled in size. Under continuos intermittently adapted antibiotic therapy (ampicillin/sulbactam, ceftriaxone, ceftazidime, gentamicin, amikacin sul- phate, metronidazole, chloramphenicol, ciprofloxacin, meropenem, imipenem-cila- statin, vancomycin) he became afebrile with sterile blood culture; but the disconti- nuance of antibiotics was immediately followed by the reappearance of septic fever.

In the period of April-August 2002 the patient experienced 5 episodes of mae- lena, with negative gastroscopy. A repeat CT showed gas filled cavities (Fig. 3), sug- gesting a communication between the HPT and the bowel. The colonscopy and Fig. 1. Posthemorrhagic shock,

1st rupture of left ileopsoas hematoma – July 1997

Fig. 2. 2nd rupture left ileopso-

as hematoma, surgical removal

and drainage – April 1998

barium enema failed to asses the fistula. Surgical excision or percutaneous drainage of the HPT were considered but deemed much too risky in our situation of FVIII shortage. Antibiotic therapy was performed almost continuously.

In December 2002 macroscopic hematuria complicated the evolution. In February 2003 an inguino-crural abscess was assessed.

March 2003 marked the externalization of the retroperitoneal abscess on the abdominal wall, precipitating the surgical intervention (Fig. 4–6).

Fig. 3. Bowel adherence and air within the left pelvic pseudo- tumor, intestinal hemorrhage - November 2002

Fig. 4. Bowel

fistula, contrast

substance and air

within the pseu-

dotumor – March

2003

On 22 March 2003 under FVIII substitution (80,000 IU) a diversion colostomy and anus praeter were performed; retroperitoneal and thigh drainage were inserted. An amount of more than 1000ml bloody pus was discharged, followed by immediate clinical improvement. Under continuous secondary prophylaxis (FVIII 50,000 IU) repositioning of drains was intermittently required. In June 2003 the drainage was discharged. In September 2003 closure of the externalized fistula on the abdominal wall was obtained.

The present status is satisfactory: the patient is nonfebrile since April 2003; he has a weight gain of 14 kg; the locomotor performance has improved; the total clinical joint score has dropped to 25; the dimension of the ileopsoas HPT has reduced, the present diameter of HPT being of 18 mm; the external segment of fis- tula has closed; the fistulation route of HPT is still present but there are no signs of communication with the bowel (Fig. 7).

Fig. 5. Externalization of fistula on the abdominal wall - March 2003

Fig. 6. Inguino-crural involvement,

abscess in the left tigh - March 2003

Discussion

In countries where adequate substitution is performed, the occurrence of HPT is rare (<1%); they usually complicate the evolution of patients with high level in- hibitors. In countries where the accessibility to appropriate replacement therapy is limited, the prevalence of HPT is quite frequent (10% or even more) [1]. Fistula for- mation is a common complication of HPT, usually in the form of cutaneous fistulae;

segment, no signs of com- munication with the bowel – November 2003

Fig. 8. Reduced HPT dimen- sion, ø18 mm – November 2003

Fig. 9. Anus praeter –

November 2003

Embolization alone has only a temporary effect. Irradiation of unresectable lesions can further damage the walls of the bowel fistula. Often, more conservative approa- ches are preferred. However, residual HPT always remains a potential focus for recur- rence with growth and infection [3, 4, 5]. Despite adequate clotting factor replace- ment bleeding may develop in the dead space remaining after the HPT is resected.

Complete resection of the HPT should be the goal, but, like in our case, may not be possible. A new surgical intervention with the interposition of omentum between PT and the bowel fistula could represent a salvage solution for our patient [6].

Conclusions

Because of many and diverse shortcomings, HPT remain a severe life threatening burden for our patients with hemophilia. A successful outcome was achieved in the presented patient due to a persistent, multidisciplinary approach; his long term prog- nosis is depending on the potential risks of bleeding and infection.

Acknowledgement. We thank Prof. Wolfgang Schramm for the generous support in FVIII concentrates, allowing the surgical intervention.

References

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