Table 1 Patient Characteristics at Presentation

Age at diagnosis

Median 7 years (IQR 20–156 months), (N = 153)

Time delay (presentation to treatment)

Median 5.6 days (IQR 2.6–14.8 days), (N = 117)

Time delay (diagnosis to treatment)

Median 1.9 (IQR 0.7–5.7 days), (N = 117)

Symptom and signs at diagnosis

153

Pain

137 (90%)

 Back pain

67 (43%)

 Limb pain

15 (10%)

 Abdominal pain

9 (6%)

 Torticollis

67 (43%)

 Irritability

38 (25%)

Tetraparesis

48 (31%)

Hemiparesis

96 (63%)

Sensory disturbances

49 (32%)

Sphincter disturbance

44 (29%)

Neurological deficit at initial presentation

104 (68%)

Neurological deficit at diagnosis

136 (89%)

Neuroimaging studies

144

MRI

83 (58%)

CT

15 (10%)

Myelography

30 (21%)

MRI and CT

14 (10%)

CT and myelography

2 (1%)

Localization

149

Cervical

21 (14%)

Cervico-thoracic

82 (55%)

Thoracic

37 (25%)

Thoraco-lumbar

4 (2.5%)

Lumbar

4 (2.5%)

Lumbo-sacral

1 (1%)

Antero-posterior localization

132

Posterior

112 (85%)

Anterior

17 (13%)

Lateral

3 (2%)

Etiology and contributing factors

150

Unknown

57 (38%)

Trivial trauma

30 (20%)

Vascular malformation

21 (14%)

 Arteriovenous malformation

10 (48%)

 Venous angioma

4 (19%)

 Angiolipoma

2 (9%)

 Hemangioma

4 (19%)

 Arteriovenous fistula

1 (5%)

Coagulation disorder

42 (28%)

 Hemophilia A

27 (64%)

 Hemophilia B

12 (29%)

 Hemophilia A and B

1 (2%)

 Coagulopathy due to cholestasis

1 (2%)

  Unknown hemophilia

1 (2%)

Treatment

151

Surgery and factor replacement

12 (8%)

Factor replacement without surgery

36 (24%)

Surgery without factor replacement

103 (68%)