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Chronic disseminated intravascular coagulation presenting as renal mass

Manjiri Somashekhar, Padmalatha S. Kadamba, Mugdha Wakodkar Department of Paediatric Surgery, MS Ramaiah Medical and Teaching Hospital, Bangalore, India

Correspondence: Dr. Manjiri Somashekhar, Department of Paediatric Surgery, MS Ramaiah Medical and Teaching Hospital, Bangalore, India. E-mail: [email protected]

Abstract

Disseminated intravascular coagulation (DIC) is a complex clinical syndrome, described as a sequential activation of the coagulation and fibrinolytic system. Trauma and sepsis are some of the known precipitating factors. We report a case of nonovert disseminated intravascular coagulation presenting as a huge renal mass in a 3-year-old child, suspected to be a Wilms’ tumor. On imaging studies, it was found to be a renal hematoma. Laboratory investigations revealed an underlying chronic disseminated intravascular coagulation caused by sepsis. The child recovered with conservative treatment; follow up investigations showed resolution of renal hematoma with renal function returning to base line. Clinical presentation of Chronic DIC is variable. Laboratory investigations usually help to diagnose the condition and also to monitor the progress of the treatment. The treatment of the triggering cause is the cornerstone of the management of this condition.

Keywords: Chronic disseminated intravascular coagulation, Wilm’s tumor, renal mass

INTRODUCTION addition, a wound discharging pus was noticed since 8 days over his right leg. On examination, the child Chronic disseminated intravascular coagulation was febrile, tachypneic, pale, icteric and edematous. (DIC), also known as compensated disseminated Abdomen was distended with visible dilated veins and intravascular coagulation, results from a persistent everted umbilicus. A huge 15 × 10 cm firm, tender and weak or intermittent activating stimulus. Under such fixed mass was palpable occupying the whole of right conditions, destruction and production of coagulation side of the abdomen. It was suspected to be a renal mass, factors and platelets are balanced.[1] Chronic DIC probably Wilms’ tumor. Auscultation of chest revealed is usually associated with carcinomatosis, retained bilateral basal fine crepitations, tachycardia and systolic dead fetus, liver disease, aneurysm or hemangioma.[2] murmur. His right leg was swollen in its entirety and Sepsis usually causes acute disseminated intravascular a distinct 2 × 1 cm tender, sinus discharging pus was coagulation but nonovert chronic DIC is also observed. seen over the distal one third of his leg. Hematuria was [1] Liver hematoma, subdural hematoma[3,4] and post- noticed on catheterization. His laboratory investigations biopsy renal hematoma due to chronic disseminated revealed the following: hemoglobin of 2 gm%, packed intravascular coagulation have been reported.[5] We are cells volume 28.3 mg/cc, total count of 16,000 cells/ reporting an unusual presentation of chronic DIC in the cc, platelet 1.82 lacs/cmm, microcytic hypochromic form of a renal mass. anemia in peripheral smear as well as neutrophilic leukocytosis 80%, and band form cells with retic count Case report 3%. Prothrombin time was 18.8 s (control 16.2 s) and activated partial thromboplastin time 48 s (control 26.5 A 3-year-old male child was admitted with complaints s), fibrin degradation products (FDP) were >2000 ng/ of high-grade continuous fever in addition to painful ml. Liver function tests revealed total bilirubin of 2.0 swelling of right leg, jaundice and abdominal distention, mg/dl, direct bilirubin 1.80 mg/dl, AST 79 mg/dl, ALT gradually progressing over 20 days. Prior to this, there 44 mg/dl, alkaline phosphatase 247 mg/dl, and serum was a history of trivial trauma over the abdomen. In albumin was 1.9gms/dl. Renal function was slightly

J Indian Assoc Pediatr Surg / Oct-Dec 2008 / Vol 13 / Issue 4 144 Somashekhar et al.: Chronic DIC masquerading as renal mass deranged with serum creatinine of 1.2 mg/dl. Serum due to trauma and chronic osteomyelitis. electrolyte levels were within normal limits: Na 127 meq/L, K 4.9 meq/L and Cl 104 meq/L. Ultrasound DISCUSSION [Figure 1] of the abdomen demonstrated a heterogeneous, lamellated, avascular mixed echogenic mass in the DIC is a complex syndrome in which there is pathological right perinephric region measuring 13 × 6 × 6.5 cm generation of thrombin and diffuse intravascular clot with urinary bladder having some echogenic material, formation.[6] It is known to occur as acute decompensated probably blood clots. Computerized tomography scan or chronic compensated form. The underlying disease Figure 1 showed evidence of contusion of the right that sparks off DIC determines the clinical presentation. kidney with perinephric hematoma which was confined In acute decompensated DIC, there is a sudden massive to renal capsule displacing the ureter anteriorly X-ray exposure of tissue factor over a brief time period; it is of the right leg exhibited changes of osteomyelitis beyond the capacity of control mechanisms and there is in distal one-third of the tibia. Pus from the wound no time for generation of coagulation factors.[7] Chronic was sterile. Biopsy of the bone confirmed chronic DIC, also known as compensated DIC, results from a non-specific osteomyelitis. The patient was managed persistent weak or intermittent activating stimulus. conservatively with broad spectrum intravenous Under such conditions, destruction and production of antibiotics for three weeks. Two units of packed red coagulation factors and platelets are balanced.[1] cells, three units of fresh frozen plasma and two units of cryoprecipitate were transfused over a period of 10 days. Bleeding is a universal manifestation of DIC, but Sinus over the right leg was explored, sequestrectomy most of the morbidity and mortality of DIC is due to was done and the limb was immobilized. Follow up microvascular thrombosis.[7] Thrombosis is witnessed investigations showed improvement in hemoglobin essentially in the form of renal failure, coma, liver and coagulation parameters. He was symptomatically failure, respiratory failure, skin necrosis, gangrene better, with a gradual decrease in abdominal distention and venous thromboembolism.[2] In chronic DIC, there and tenderness. A small palpable mass was present in may be very minimal or no clinical features or there the right lumbar region. The urine output was adequate may be only laboratory evidence of DIC.[7] Thus, we without hematuria. The child was taking orally and see marked heterogenicity in clinical manifestations, was ambulant with cast in situ. He was discharged and the etiologic factor is the major predictor of the at the end of 4 weeks and followed up after 2 weeks. clinical events.[1] He looked healthy, with a very small nontender mass palpable in the right lumbar region. Follow up In our case, perhaps an unspecified trivial trauma has ultrasound still demonstrated a 8 × 3 × 5 cm right instigated osteomyelitis of the tibia which remained perinephric hematoma. Intravenous urography revealed undiagnosed till there was sepsis. A small post- normally functioning kidneys. Thrombus was seen in traumatic right renal hematoma has gradually enlarged the inferior vena cava between the liver and common due to chronic DIC. iliac veins with multiple retroperitoneal collaterals. He further received 6 weeks treatment with aspirin and oral Though the pathophysiology is identical to acute DIC, antibiotics for thrombus and osteomyelitis, respectively. clinical picture and laboratory findings in chronic DIC Final diagnosis was of chronic DIC triggered by sepsis may be wavering. Here, nearly all of the global tests like platelet count, fibrinogen, PT and PTT may be normal. FDP, fibrinopeptide A and D dimer are usually raised and diagnostic.[7] Chronic DIC is usually associated with carcinomatosis, retained dead fetus, liver disease, aneurysm or hemangioma.[2] Sepsis usually causes acute DIC but nonovert chronic DIC is also observed.[1] Liver hematoma, subdural hematoma[3,4] and post-biopsy renal hematoma due to chronic DIC have been reported.[5]

Critical point in the management of DIC is to eradicate the primary disease and treat the concomitant causes.[8] Patients usually require treatment for thrombotic obstruction of the vasculature and subsequent multiorgan failure.[8] Theoretically, interruption of coagulation should be of benefit in patients with DIC. Heparin has been shown to have a beneficial effect in small, uncontrolled studies of Figure 1: CT scan showing right renal hematoma patients with DIC, but not in controlled clinical trials.[9]

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The cornerstone of management of DIC is the treatment 4. Ng PC, Fok TF, Lee CH, Wong W, Cheung KL. Massive subdural of the underlying disorder. Most often, these cases can hematoma: An unusual complication of septicemia in preterm very low birth weight infants. J Paediatr Child Health 1998;34:296-8. be managed with blood and coagulation factors if the 5. Dara T, Lohr J. Disseminated Intravascular Coagulation following underlying cause is treated as in our case. percutaneous renal biopsy. Am J Nephrol 1991;11:343-4. 6. Hassell K, Colorado. Disseminated Intravascular Coagulation, Medline Online Review and Database. Snow Tiger Medical REFERENCES Database. 2004. 7. Bakhshi S, Arya LS. Diagnosis and treatment of disseminated 1. Rodgers G. Wintrobe’s clinical hematology, 11th ed. Vol. 2, Chapter intravascular coagulation. Indian Pediatr 2003;40:721-30. 60. 2004. p. 1671-757. 8. Gullo A. Disseminated intravascular coagulation in the critical 2. Baglin T. Fortnightly review: Disseminated intravascular illness. Minerva Anesthesiol 2001;67:831-8. coagulation: Diagnosis and treatment. BMJ 1996;312:683-7. 9. Levi M, Ten Cate H, Disseminated intravascular coagulation. N 3. Pollak L, Schiffer J, Leonov Y, Zaidenstein R. Acute subdural Engl J Med 1999;341:586-92. hematoma following disseminated intravascular coagulation associated with an obstetric catastrophe. Isr J Med Sci Source of Support: Nil, Conflict of Interest: None declared. 1995;31:489-91.

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