Abstract

We report a patient of sickle cell disease, who presented to us with a splenic abscess. She developed a lienocolonic fistula following percutaneous drainage of the splenic abscess. Apart from this unprecedented complication she developed a pathological fracture of the humerus during the same hospital stay. Diagnosis and management related issues of multiple morbidities in a patient of sickle cell disease have been discussed.

Introduction

Splenic infarction leading to splenic abscess is a known complication of sickle cell disease.1 It presents clinically with pain in abdomen and fever. An abdominal USG and CT scan help in diagnosis and provide guidance for a percutaneous drainage of the splenic abscess. A splenectomy or percutaneous drainage of abscess with antibiotic therapy is mandatory for treatment.

Case Report

A 14-year-old girl was referred to us as a known case of sickle cell disease (SCD) with complaints of, pain in upper abdomen and fever with chills since two weeks. On examination, the patient was asthenic, febrile and pale. She had a pulse rate of 110 per min and a normal blood pressure. Abdominal examination revealed a large and tender splenomegaly. Her laboratory investigations revealed haemoglobin of 7.4 gm percent and total count of 12,800 per cu.mm. Peripheral smear showed anisocytosis and target cells with adequate platelet count. Haemoglobin electrophoresis demonstrated an SS pattern.

Ultrasonography (USG) of the abdomen showed an enlarged spleen with hypoechoic area showing internal echoes along with air densities suggestive of splenic abscess. About 180 ml of pus was drained by USG guided aspiration. The pus on culture grew Proteus mirabilis organisms sensitive to cefotaxim. The patient was administered antibiotics and was transfused two units of whole blood. A computed tomographic (CT) scan of abdomen revealed a huge residual splenic abscess of 16 x 12 x 14 cm size with air fluid level (Fig. 1). USG guided percutaneous pigtail drain was inserted and around 1200 ml of pus was drained. Drainage reduced to minimal in one week and the patient improved symptomatically. Follow up USG revealed regression of abscess cavity with no residual collection.

A sinogram was done, for radiological demonstration of the cavity, which demonstrated the passage of dye into the descending colon suggestive of a fistulous communication (Fig. 2). An abdominal CT scan also revealed the drain entering the colon before reaching the splenic abscess. On colonoscopy the pigtail drain was seen presenting itself in the upper part of the descending colon and exiting at the splenic flexure about 15 cm beyond the point of entry into the colon. The colon was essentially normal. The catheter was pulled out under colonoscopic vision. The patient had no abdominal complaints or signs after the procedure. However she developed pain in the right shoulder around this time. There was no history of trauma. Local examination revealed a tender swelling in the right upper arm. An X-ray of right shoulder revealed a pathological fracture of the proximal end of humerus (Fig. 3). The patient was treated with analgesics, third generation cephalosporins, gentamicin and immobilization. She became aysmptomatic and was discharged two weeks later and was advised to take antibiotics orally for a month. Six weeks later a USG of abdomen did not reveal any residual collection in the spleen and her humeral fracture was showing radiological signs of healing. At one year follow up the patient was asymptomatic with no evidence of splenic collection on USG and her humeral fracture had healed.

Fig. 1 :Abdominal CT scan showing a large splenic abscess with air fluid level.	Fig. 2 :Sinogram showing the pigtail drain and the presence of dye in the spleen and the descending colon.	Fig. 3 :X-ray of right shoulder showing pathological fracture of proximal humerus.

Discussion

A lienocolonic fistula has not been reported previously in patients of SCD. Cases of colosplenic fistulas secondary to Crohn’s colitis, immunoblastic lymphoma, and melanoma have been reported, but in those cases the colon was the primary site of pathology. Very few cases of leinocolonic fistula formation following splenic abscess have been reported. A case of lienocolonic fistula due to splenic abscess in a diabetic man with ectopia vesicae and in a patient of polycythaemia vera has been reported.2,3 This is perhaps the only reported case of lienocolonic fistula formation following percutaneous pigtail insertion for drainage of splenic abscess.

Spontaneous lienocolonic fistula can occur in a case of splenic abscess.2 It is thus mandatory to do a colonoscopy to confirm the causal role of the drain. Colonoscopy also aids in removing the catheter under vision. In the present case, as the abscess had completely drained and the fistulous tract was narrow it healed without much problems. However, awareness of this complication should help to plan the placement of the percutaneous drain appropriately to avoid such an occurrence.

In sickle cell disease pathological fracture of bone can occur due to bone infarction or due to osteomyelitis. In some cases infection may superimpose on infarction. It has been observed that pathological fractures occur more frequently in acute rather than chronic osteomyelitis in young patients with SCD.4 Investigations like culture from bone aspirate, magnetic resonance imaging (MRI) and sequential radionuclide bone marrow and bone scans can help in distinguishing the cause of the osseous lesion.5,6 However in the present case no such investigations were done. As the patient had a co-existing source of sepsis the fracture was treated as pathological fracture due to osteomyelitis on clinical grounds.

Vaso-occlusion by sickled cells leads to multiple organ infarction. Sepsis usually follows due to increased susceptibility of these patients to infections due to hyposplenism. This leads to a wide variety of clinical problems, which may present to various surgical and medical disciplines. A team approach and close collaboration is thus essential.

References

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2. Cowie MR, Hoffbrand BI, Grant DS. Lienocolonic fistula following splenic abscess. J R Soc Med 1992; 85 (10) : 636-7.
3. Means JR, Villella ER, Stahlfeld KR. Splenocolic fistula in a patient with polycythemia vera. Am J Surg 2003; 185 (2) : 173-4.
4. Ebong WW. Pathological fracture complicating long bone osteomyelitis in patients with sickle cell disease. J Pediatr Orthop 1986; 6 (2) : 177-81.
5. Wong AL, Sakamoto KM, Johnson EE. Differentiating osteomyelitis from bone infarction in sickle cell disease. Pediatr Emerg Care 2001; 17 (1) : 60-3.
6. Skaggs DL, Kim SK, Greene NW, Harris D, Miller JH.Differentiation between bone infarction and acute osteomyelitis in children with sickle-cell disease with use of sequential radionuclide bone-marrow and bone scans. J Bone Joint Surg Am 2001; 83-A (12) : 1810-3.

*Associate Professor and Unit Head; **Senior Registrar, Department of Surgery, Government Medical College and Hospital, Nagpur, India.