Non-traumatic splenic rupture in a patient with Kasabach–Merritt syndrome

Jitesh Parmar,1 Behnam Shaygi,2 and Mike Nelson1
BMJ Case Rep. 2009; 2009: bcr08.2008.0792.Published online 2009 Mar 5. doi: 10.1136/bcr.08.2008.0792 PMCID: PMC3027749 PMID: 21686627

BACKGROUND

Haemangiomas are vascular lesions resulting from abnormal proliferation of blood vessels. They are the most common paediatric neoplasm.

Kasabach–Merritt syndrome (KMS) is a rare type of vascular lesion with peculiar characteristics. The diagnosis is based upon three basic findings: enlarging haemangioma, thrombocytopenia and consumption coagulopathy. The thrombocytopenia and consumption coagulopathy is known as Kasabach–Merritt phenomenon.1 Almost 200 cases have been reported in the literature since Kasabach and Merritt described the first case in 194020 but there has been only one case report of non-traumatic splenic rupture in a patient with KMS for whom, due to rarity of non-traumatic splenic rupture, splenctomy was not performed, and the patient died of exsanguination.2

Although splenectomy is more hazardous in patients with impaired coagulation, the situation also is more urgent in such patients since exsanguination occurs so rapidly following splenic rupture. Patients with impaired coagulation who develop evidence of splenic haemorrhage should be evaluated quickly and definitively using available imaging. Once splenic haemorrhage has been confirmed, splenectomy should be seriously considered.

The present report describes a patient with KMS who presented with acute abdominal pain, whose non-traumatic splenic rupture was confirmed by CT scan, and he was treated with emergency laparatomy plus splenectomy and blood products administration to control the coagulopathy. The clinical presentation of the case and outcome of selected treatment modalities are discussed in the light of previous studies done in connection with this subject.

CASE PRESENTATION

The patient was a 22-year-old man who was born with misshapen left arm and was later diagnosed with KMS (fig 1). This patient has had multiple treatments including medical, endovascular and even surgical resections of a part of this arteriovenous malformation. The patient had attended hospital and was admitted with acute abdominal pain with diffusely tender abdomen.

Non-traumatic splenic rupture in a patient with Kasabach–Merritt syndrome
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Figure 1
Extensive arteriovenous malformation involving whole left upper limb and left hemithorax.

As the patient was haemodynamically stable, and the exact diagnosis was not clear with a background of coagulopathy, an urgent CT scan of the abdomen/chest/pelvis was performed, and this revealed large haemoperitoneum with splenic rupture (fig 2)

INVESTIGATIONS

Investigations on admission were as follows. Full blood count: haemoglobin 6.8 g/dl; mean cell volume 69 fl, platelet count 123×109/l. Clotting: international normalised ratio 1.5, activated partial thromboplastin time ratio 1.59. Cross-match: IgG autoantibody 3+. CT scan of the abdomen/chest/pelvis: large haemoperitoneum with splenic rupture.Go to:

TREATMENT

In view of the CT scan result and coagulopathic blood picture, the patient was resuscitated with blood products and had urgent laparatomy plus splenectomy with successful control of haemorrhage. The patient was then transferred to the intensive treatment unit where he remained unstable. A re-look laparatomy was performed 8 h later, and this revealed no major haemorrhage. Only generalised oozing, especially from splenic bed, was found. This was deemed to be related to on going coagulopathy.

Despite successful control of haemorrhage surgically, the patient continued to remain unstable and required more blood products as follows: 28 units red blood cells, 14 units fresh frozen plasma, 10 units platelets, 10 units cryoprecipitate, and 2× Novo VII. This overwhelmed resources at local district hospital level, and after stabilisation the patient was transferred to a nearby tertiary care unit where he received further 10 units red blood cells, 8 units platelets, 3 units cryoprecipitate, 2 units fresh frozen plasma in the first 24 h.Go to:

OUTCOME AND FOLLOW-UP

The continued coagulopathy was treated with further blood products and systemic corticosteroid therapy. The patient was eventually discharged with full recovery after 4 weeks in hospital.Go to:

DISCUSSION

KMS is a very rare vascular malformation, and more than 80% of cases occur within the first year of life.3 With an overall mortality rate of 12%, there is a mortality rate of 30% for patients with diffuse cavernous haemangiomas when a major haemorrhage occurs.4,5 Various treatment regimens have been published, with inconsistent results. There are two major treatment objectives: the control of the coagulopathy and thrombocytopenia, as well as eradication of the haemangioma. Consequently, different treatment regimens are performed, including systemic corticosteroids,6,7 irradiation,8,9 compression,10 embolisation,11,12 antifibrinolytic agents,13,14 platelet aggregation inhibitors,15 and interferon,16,17 as well as other strategies.18,19

As previously stated there is only one case report in available literature published in 1973: a patient with known KMS presented with spontaneous splenic rupture and unfortunately died because of massive haemorrhage.

LEARNING POINTS

  • Although splenectomy is more hazardous in patients with impaired coagulation, the situation also is more urgent in such patients since exsanguination occurs so rapidly following splenic rupture.
  • Patients with impaired coagulation who develop evidence of splenic haemorrhage should be evaluated quickly and definitively.
  • There are two major treatment objectives: the control of the coagulopathy and thrombocytopenia, as well as eradication of the exsanguination site.
  • Once splenic haemorrhage has been confirmed, splenectomy should be seriously considered.