Pediatric Neurology xxx (2015) 1e2
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Visual Diagnosis
Cavernous Sinus Thrombosis With Occlusion of Internal Carotid Artery in a Young Child With Acute Lymphoblastic Leukemia Payal Malhotra DNB, Sandeep Jain DNB, FIAP, Gauri Kapoor MD, PhD * Department of Pediatric Hematology and Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India
This 14-year-old boy with acute lymphoblastic leukemia (ALL) was referred to our institute on day 17 of induction chemotherapy with complaints of sudden onset of severe
FIGURE. (A) Magnetic resonance venogram: loss of flow in right cavernous sinus (black arrow)*. (B) Magnetic resonance T1-weighted image: loss of flow void in right internal carotid artery and thrombosed cavernous sinus (black arrow).* (C) T2-weighted magnetic resonance imaging: right maxillary sinusitis. (D) Magnetic resonance venogram: loss of flow in the left transverse and sigmoid sinuses (arrow). (E) Magnetic resonance angiogram: nonvisualization of right internal carotid artery (black arrow).* (F) Magnetic resonance imaging (contrast): multiple right cerebral infarcts (arrows). *White arrows represent normal flow.
Sources of support: None. Conflict of interest: None.
* Communications should be addressed to: Dr. Kapoor; Department of Pediatric Hematology and Oncology; Rajiv Gandhi Cancer Institute and Research Centre; Sector 5; Rohini, Delhi-110085, India. E-mail address: [email protected] 0887-8994/$ e see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2015.08.017
headache and diplopia. In addition, he had a high-grade fever and loose stools with melena. Central nervous system examination revealed normal cognitive function without any sensory or motor deficit. Cranial nerve examination revealed normal-sized pupils with intact direct and consensual light reflex and right ptosis and lateral rectus palsy. The other cranial nerves and systemic examination were unremarkable. Differential diagnoses of intracranial infection, thrombosis, and bleed were considered. A hemogram revealed hemoglobin 8 g/dL, total leukocyte count 1 103/mL, absolute neutrophil count 44/ mL, and platelet count 44 103/mL; sepsis screen and cerebrospinal fluid analysis were unremarkable. An urgent magnetic resonance venogram (Figure) revealed thrombosis in the left transverse and sigmoid sinuses, right cavernous sinus, and internal carotid artery with multiple cerebral infarcts, maxillary sinusitis. Pulmonary aspergillosis was demonstrated on computed tomography scan of the chest (not shown). Management included broadspectrum antibiotics, anti-aspergillus therapy, lowmolecular-weight heparin, and other supportive measures. Clinically there was neurological progression, initially leading to complete ophthalmoplegia of the right eye with loss of pupillary function and all extraocular movements followed by gradual and near-complete recovery. Hyperleukocytosis, drugs (steroids and L-asparaginase), and sepsis make ALL a prothrombotic condition for cerebral thrombosis (as in this case).1 Clinically symptomatic cases are reported in fewer than 5% of children with ALL, usually involving the dural sinuses or cortical veins.2 Additional involvement of the cavernous sinus and internal carotid artery was observed in our patient. Hematogenous dissemination of sinopulmonary infection probably led to cavernous sinus thrombosis with secondary occlusion of internal carotid artery and infarcts in its territory. The clinical manifestations of cavernous sinus thrombosis may be subtledsuch as persistent headache, vomiting, decreased visual acuity, or transient weakness of limbsdor frankdwith exophthalmos, ophthalmoplegia,
2
P. Malhotra et al. / Pediatric Neurology xxx (2015) 1e2
seizures, or focal neurological deficits. There may be varying involvement of cranial nerves and autonomic fibers within the lateral wall of the cavernous sinus. Magnetic resonance venography is the preferred imaging method because it shows an absence of venous flow in the affected sinus, which may well be missed on conventional imaging of the brain.3 Because radiological changes may precede clinical signs, a high index of suspicion and timely imaging in predisposed patients aid early diagnosis and prompt management. Unlike arterial thrombosis, where clot formation depends on endothelial damage and platelet activity, venous thrombosis results from activation of the coagulation cascade. Treatment includes supportive measures to relieve raised intracranial pressure and
specific measures targeting the thrombotic process. Lowmolecular-weight heparin is recommended to prevent extension of thrombus, subsequent embolism, and support thrombus resolution.1 References 1. Athale UH, Siciliano SA, Crowther M, et al. Thromboembolism in children with acute lymphoblastic leukaemia treated on Dana-Farber Cancer Institute protocols: effect of age and risk stratification of disease. Br J Haematol. 2005;129:803-810. 2. Absoud M, Hikmet F, Dey P, et al. Bilateral cavernous sinus thrombosis complicating sinusitis. J R Soc Med. 2006;99:474-476. 3. Razek AA, Castillo M. Imaging lesions of the cavernous sinus. Am J Neuroradiol. 2009;30:444-452.
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Visual Diagnosis
Cavernous Sinus Thrombosis With Occlusion of Internal Carotid Artery in a Young Child With Acute Lymphoblastic Leukemia Payal Malhotra DNB, Sandeep Jain DNB, FIAP, Gauri Kapoor MD, PhD * Department of Pediatric Hematology and Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India
This 14-year-old boy with acute lymphoblastic leukemia (ALL) was referred to our institute on day 17 of induction chemotherapy with complaints of sudden onset of severe
FIGURE. (A) Magnetic resonance venogram: loss of flow in right cavernous sinus (black arrow)*. (B) Magnetic resonance T1-weighted image: loss of flow void in right internal carotid artery and thrombosed cavernous sinus (black arrow).* (C) T2-weighted magnetic resonance imaging: right maxillary sinusitis. (D) Magnetic resonance venogram: loss of flow in the left transverse and sigmoid sinuses (arrow). (E) Magnetic resonance angiogram: nonvisualization of right internal carotid artery (black arrow).* (F) Magnetic resonance imaging (contrast): multiple right cerebral infarcts (arrows). *White arrows represent normal flow.
Sources of support: None. Conflict of interest: None.
* Communications should be addressed to: Dr. Kapoor; Department of Pediatric Hematology and Oncology; Rajiv Gandhi Cancer Institute and Research Centre; Sector 5; Rohini, Delhi-110085, India. E-mail address: [email protected] 0887-8994/$ e see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2015.08.017
headache and diplopia. In addition, he had a high-grade fever and loose stools with melena. Central nervous system examination revealed normal cognitive function without any sensory or motor deficit. Cranial nerve examination revealed normal-sized pupils with intact direct and consensual light reflex and right ptosis and lateral rectus palsy. The other cranial nerves and systemic examination were unremarkable. Differential diagnoses of intracranial infection, thrombosis, and bleed were considered. A hemogram revealed hemoglobin 8 g/dL, total leukocyte count 1 103/mL, absolute neutrophil count 44/ mL, and platelet count 44 103/mL; sepsis screen and cerebrospinal fluid analysis were unremarkable. An urgent magnetic resonance venogram (Figure) revealed thrombosis in the left transverse and sigmoid sinuses, right cavernous sinus, and internal carotid artery with multiple cerebral infarcts, maxillary sinusitis. Pulmonary aspergillosis was demonstrated on computed tomography scan of the chest (not shown). Management included broadspectrum antibiotics, anti-aspergillus therapy, lowmolecular-weight heparin, and other supportive measures. Clinically there was neurological progression, initially leading to complete ophthalmoplegia of the right eye with loss of pupillary function and all extraocular movements followed by gradual and near-complete recovery. Hyperleukocytosis, drugs (steroids and L-asparaginase), and sepsis make ALL a prothrombotic condition for cerebral thrombosis (as in this case).1 Clinically symptomatic cases are reported in fewer than 5% of children with ALL, usually involving the dural sinuses or cortical veins.2 Additional involvement of the cavernous sinus and internal carotid artery was observed in our patient. Hematogenous dissemination of sinopulmonary infection probably led to cavernous sinus thrombosis with secondary occlusion of internal carotid artery and infarcts in its territory. The clinical manifestations of cavernous sinus thrombosis may be subtledsuch as persistent headache, vomiting, decreased visual acuity, or transient weakness of limbsdor frankdwith exophthalmos, ophthalmoplegia,
2
P. Malhotra et al. / Pediatric Neurology xxx (2015) 1e2
seizures, or focal neurological deficits. There may be varying involvement of cranial nerves and autonomic fibers within the lateral wall of the cavernous sinus. Magnetic resonance venography is the preferred imaging method because it shows an absence of venous flow in the affected sinus, which may well be missed on conventional imaging of the brain.3 Because radiological changes may precede clinical signs, a high index of suspicion and timely imaging in predisposed patients aid early diagnosis and prompt management. Unlike arterial thrombosis, where clot formation depends on endothelial damage and platelet activity, venous thrombosis results from activation of the coagulation cascade. Treatment includes supportive measures to relieve raised intracranial pressure and
specific measures targeting the thrombotic process. Lowmolecular-weight heparin is recommended to prevent extension of thrombus, subsequent embolism, and support thrombus resolution.1 References 1. Athale UH, Siciliano SA, Crowther M, et al. Thromboembolism in children with acute lymphoblastic leukaemia treated on Dana-Farber Cancer Institute protocols: effect of age and risk stratification of disease. Br J Haematol. 2005;129:803-810. 2. Absoud M, Hikmet F, Dey P, et al. Bilateral cavernous sinus thrombosis complicating sinusitis. J R Soc Med. 2006;99:474-476. 3. Razek AA, Castillo M. Imaging lesions of the cavernous sinus. Am J Neuroradiol. 2009;30:444-452.
