Traumatic Retroclival Subdural Hematoma in a Child with Hemophilia

Journal Pre-proof TRAUMATIC RETROCLIVAL SUBDURAL HEMATOMA IN A CHILD Abat Sahlu, Kaleab Getachew, Abebe Mekonnen PII:

S1878-8750(19)32489-1

DOI:

https://doi.org/10.1016/j.wneu.2019.09.059

Reference:

WNEU 13358

To appear in:

World Neurosurgery

Received Date: 13 August 2019 Revised Date:

10 September 2019

Accepted Date: 11 September 2019

Please cite this article as: Sahlu A, Getachew K, Mekonnen A, TRAUMATIC RETROCLIVAL SUBDURAL HEMATOMA IN A CHILD, World Neurosurgery (2019), doi: https://doi.org/10.1016/ j.wneu.2019.09.059. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Elsevier Inc. All rights reserved.

TRAUMATIC RETROCLIVAL SUBDURAL HEMATOMA IN A CHILD WITH HEMOPHILIA CASE REPORT

ADDIS ABABA UNIVERSITY, COLLEGE OF HEALTH SCIENCES, SCHOOL OF MEDICINE, DEPARTMENT OF SUEGERY, NEUROSURGERY UNIT ADDIS ABABA, ETHIOPIA. April 2019 Abat Sahlu2, Kaleab Getachew1, Abebe Mekonnen 2

Abat Sahlu2, MD, Consultant Neurosurgeon, Associate professor, Addis Ababa University College of heath science Abebe Mekonnen2, MD, Consultant Neuroradiologist, Associate professor Addis Abeba University College of heath science Kaleab Getachew1, MD, Year V neurosurgery resident, Addis Abeba University College of heath science

Introduction Retroclival hematomas are rare and only represent a small subset of posterior fossa extra-axial hematomas, which as a whole constitute approximately 0.3 % of acute extraaxial hematomas.1 Most are on the cerebellar convexity. No data is available on what proportion of these SDHs are Retroclival.4 Most cases in the literature involve the pediatric population, though few cases have been reported in the adult population as well. Frequently, the etiology is related to accidental trauma, though other mechanisms have been observed, including coagulopathy, non-accidental trauma, pituitary apoplexy, and ruptured aneurysm. Still, some remain spontaneous without an identifiable cause.1 There are three compartments in the retroclival region: epidural, subdural, and subarachnoid spaces, in to which hemorrhage may occur The accurate localization of hemorrhage in the retroclival region along with the possibility of associated traumatic injury of the spine or brain should help us avoid potential significant clinical consequences and to plan the appropriate management.1,2 Bleeding disorders account for small but significant risk factor associated with intracranial haemorrhage. In conditions such as hemophilia, massive intracranial haemorrhage is often the cause of death. Hemophilia A is a congenital coagulation defect caused by an absence or low concentration of the plasma protein factor VIII which results in impairment of the first phase of coagulation.3 Hemophilia-associated hemorrhage is preventable if clotting factor replacement therapy is infused to achieve and maintain normal physiologic levels of factor VIII (FVIII) or factor IX (FIX) respectively for hemophilia A or B. Even the well-established practice of infusing FVIII or FIX prophylactically to prevent spontaneous bleeding uses dosing strategies insufficient to prevent all potential traumainduced hemorrhages. Therefore, all individuals with hemophilia are, under very adverse circumstances, at risk for severe bleeding that may quickly evolve to life threatening circumstances requiring emergency care. 5

Case Presentation An 11 years old male child, who is a known hemophiliac with regular follow up at the pediatric hematology unit, presented 2 days after sustaining a fall down accident while he was playing with his peers. At presentation he was complaining of severe headache, photophobia and double visions. He had one episode of vomiting otherwise he denied any history of seizures or weakness He is a known hemophiliac with regular follow up and on factor VIII replacement on a regular basis. Physical examination at presentation showed stable vitals, there was bruise on the right forehead. Neurologic exam revealed bilateral abduscent nerve palsy otherwise GCS was 15/15, pupils were bilaterally equal sized and reactive to light and power was 5/5 in all extremities. He was scanned with an Unenhanced Brain and Cervical spine CT scan which revealed a slightly hyperdense extra axial collection in the retroclival region extending to the left anterior cerebellar hemisphere and included upper cervical spinal canal, which was consistent with subdural hematoma; sub acute stage. (Figure 1) There was no intracranial hemorrhage seen in the supratentorial compartment. Bone windows did not reveal any fracture of the clivus. Brain and Cervical spine MRI done five days after admission a crescent shaped extra axial collection along the anterior and posterolateral aspect of the left cerebellar hemisphere extending to pre pontine extra axial space and anterior upper cervical spinal canal subdural space. The collection measures 6mm in thickness at maximum point of collection and has T1 shortening (hyper intense on T1W) and T2 prolongation on T2W and FLAIR sequences.(Figure 2) The spinal portion of the hematoma was spanning from foramen magnum to C7 level. The collection causes no significant mass effect on adjacent structures. (Figure 3) Otherwise no features of ligamentous injury or AOD were noted. Coagulation profile revealed slightly prolonged PT and PTT. INR was 1.35. Other hematologic work up did not reveal any abnormalities. Factor level determination was not done (not available) He was admitted to the pediatric causality ward, and was given 1350 IU of factor VIII concentrate for the first 7 days to achieve the 100% desired factor VIII activity. After the first week of treatment patient received half of the first week dose, 25 IU/KG, 675 IU for the next 2 weeks. He was followed for any neurologic deterioration and worsening of pre existing symptoms or the emergence of new ones. Multidisciplinary management protocol was followed involving Pediatrics/Hematology and Neurosurgery. His headache and photophobia progressively improved but double vision persisted. On progressive evaluation the severe headache which was present initially was resolving and patient showed significant improvement and was discharged from the emergency ward and sent back to the hematology and neurosurgery clinic. Evaluation at two weeks follow up clinic revealed significant improvement of the abduscent palsy and control CT scan showed complete resolution of the hematoma.

Discussion SDHs of the posterior fossa are rare and there have been very few reports of Retroclival SDH. Most cases of retroclival hematomas are epidural and posttraumatic.4 Actual bleeding in the subdural or epidural region is even less common with most cases occurring in the epidural space in children in conjunction with blunt trauma and skull base fracture.6 Due to their strategic location and rarity, retroclival hematomas pose a challenge in diagnosis and management.7 Bleeding disorders account for small but significant risk factor associated with intracranial haemorrhage. In conditions such as hemophilia, massive intracranial haemorrhage is often the cause of death. Hemophilia A and Hemophilia B are hereditary deficiencies of factor VIII and factor IX, respectively, two glycoproteins required for normal blood clotting. Among affected persons, spontaneous bleeding or bleeding at the site of an injury is common and, if uncorrected, can lead to severe disability or death.3 Hemophilia A is almost exclusively found in males and is an X-linked recessive disorder in which there is a deficiency of factor VIII. 8 Possibly the most frequently encountered emergent hemorrhagic event in hemophilia management is CNS bleeding. Most of these events, which involve bleeding inside the skull or spinal canal, are caused by trauma. However, since patients with hemophilia can experience bleeding even weeks after a minor head injury, a history of head trauma may be hard to determine, particularly in children. The risk of traumatic events affects the entire spectrum of hemophilia phenotypes. For people with mild hemophilia, risk of intracranial hemorrhage is at least 50% that of those with severe disease.5 Our literature review revealed 18 cases of retroclival subdural hematomas in the pediatric population. Six cases were traumatic out of which 2 have undergone surgical evacuation of the hematoma. The long term outcome of these traumatic cases was favorable except for the demise of one patient. One eight years old child with both retroclival epidural and subdural hematoma following a motor vehicle accident was reported.1 The remaining twelve cases in the pediatric population were reported in association with abusive head trauma.9 Table 1, below, summarizes the literatures available on pediatric retroclival hematoma The literature review also revealed 24 cases of retroclival subdural hematoma in the adult population.1,3,6,10,11,12,13 Table 2, below, summarizes the literatures retrieved regarding retroclival hematomas in the adult population There were only two published studies in patients with hemophilia and retroclival subdural hematoma both with fatal outcome .3,8 The first was a 17 years old male with severe hemophilia A who was on a home based treatment with a virus inactivated factor VIII concentrate. He sustained a fall down accident after slipping on a patch of ice and hitting his head, he was initially seen in an emergency department and CT was normal and he was neurologically intact after which he was released from the emergency department only to be found unconscious at home

5 days after his trauma. Repeat CT showed a retroclival subdural hematoma and the patient was declared brain dead 12 hours after admission.8 The second case was a 20 years old patient with a past history of hemophilia who presented to the ED with a complaint of severe headache and history of assault. CT at the time of admission showed a left frontal ICH and a left side Supratentorial ASDH with no evidence of clival hematoma. The patient was admitted to an ICU but his condition kept on deteriorating until he died and autopsy showed a subdural hematoma that extended in the posterior fossa anteriorly from the clivus to the upper spinal subdural space.3 The most common etiology of RCH is a traumatic event that induces hyper mobility of the neck. Either hyperflexion or hyperextension can lead to soft tissue injury or fractures, causing a retroclival hematoma. The preponderance of reported pediatric cases relative to adult cases may be attributed to the anatomical differences at the craniocervical junction. Compared to adults, children possess certain features (large head-to-body proportion, small occipital condyles, shallow facet joints, and weak cervical muscles) that increase the mobility of the spine and augment the risk for injury.1 Children also have a higher fulcrum of flexion compared to adults.2 The tectorial membrane is firmly attached to the posterior aspect of the body of the axis and attaches to the basilar groove of the occipital bone at the level of the foramen magnum where it blends with the cranial dura mater. In REDH, traction applied during stripping of the tectorial membrane can shear nearby vessels of the basilar venous plexus and dorsal meningeal branch of the meningohypophyseal trunk allowing blood to fill the retroclival epidural space. It is also thought that a less common mechanism for development of REDH involves fracture of the clivus as opposed to disruption of the tectorial membrane.2 The cause of RSDH is unknown but shearing forces may lead to hemorrhage via rupture of the bridging petrosal and small veins near the foramen magnum; the tectorial membrane is usually unharmed, remaining attached to the clivus; this feature is an important characteristic which differs from REDH.1 Neuroimaging may be carried out by either CT or MRI. CT is usually the initial study of choice in the setting of significant trauma in children. CT imaging should be reviewed in sagittal and axial planes and both in soft tissue and bone windows to determine the presence of soft tissue and bone injury. The shape of the hemorrhage or collection does not definitively help with determining the location, as is often possible in the supratentorial compartment.2 The diagnosis of the location of a retroclival hematoma as subdural or extradural is difficult with a CT scan alone.4 These hematomas may be overlooked on axial CT due to beam hardening artifacts in the posterior fossa, requiring reformatted CT images or MRI to elucidate the diagnosis and assess for ligamentous damage.1 The typical CT findings of RSDH are the presence of hyperdense material posterior to the clivus. The hemorrhage is not confined to the C2 vertebral body level in contradistinction to REDH and may extend cranially to the brain and also inferiorly to the spinal subdural space.2

This is because the posterior [inner] layer of the clival dura is continuous with the spinal dura, while the anterior (periosteal) layer is continuous with the upward extension of the posterior longitudinal ligament, that is, the tectorial membrane.4 The tectorial membrane is intact. CT may demonstrate hemorrhage overlying the intact tectorial membrane when reviewed on the sagittal soft tissue reformats. The simultaneous presence of subdural blood in the posterior fossa, for instance along the tentorium and retrocerebellar region, would be more suggestive of RSDH. MRI is superior in evaluating ligamentous injury. The standard sequences include sagittal and axial T1- and T2- weighted MRI sequences as well as T2-weighted short-TI inversion recovery (STIR) sequences. The T2-weighted and the STIR pulse sequences are important in determining the integrity of the ligaments. The evaluation of the tectorial membrane, transverse, atlantocipital, and apical ligaments at the CCJ is best done with MRI. In RSDH the tectorial membrane is well delineated as a continuous T2hypointense structure, confirming its integrity. The signal characteristic of blood itself varies depending on the age and evolution on both T1- and T2 weighted pulse sequences. RSDH unlike REDH is not bounded by the margins of the tectorial membrane, which is typically intact, and may extend or redistribute into the spinal subdural space.2 Clinical presentation can be variable. Neurological impairment may be related to stretching, direct compression, or contusion of surrounding nerves and brain parenchyma. The most frequently injured cranial nerve is the sixth cranial nerve (unilateral or bilateral).1 Other affected nerves include the optic, oculomotor, trigeminal, facial, glossopharyngeal, and hypoglossal nerves. Patients may also exhibit hemiparesis or quadriparesis. The rare extreme cases include brain stem contusion with cardiorespiratory compromise and progressive hydrocephalus.1,3,8,12,14 Many patients with hemophilia have a delay of several days to a week in the onset of signs following an injury with trivial nature of the mechanism of trauma and rapidly fatal deterioration also being common in head injured hemophiliacs. This feature suggests that the source of bleeding is small and the accumulation of blood is slow.8 When hemophiliacs bleed, they do not bleed faster than normal, they bleed longer because the coagulation process does not function properly.3 Negative initial CT scans are not unusual and persistent symptoms or neurologic deterioration warrant repeat imaging in this group of patients.8 No other specific clinical signs or symptoms have been reported. This perhaps reflects the cause for RSDH as an entity being relatively underreported or understudied.2 The presence of ligamentous instability and brain injury or spinal cord injury will determine the appropriate management.1 Expectant management is a reasonable method of treatment in the setting of a normal neurological examination and spontaneous resolution of blood products is the expectant course. Mass effect on the brainstem and cranial nerves necessitates surgical evacuation, although this is rarely required.

The prognosis is generally good when present in isolation. Except for the rare cases that lead to death, the majority of patients exhibit good outcomes with minimal long-term neurological deficits with conservative management.1,2 Head trauma-related ICH in patients with bleeding disorders has been best described in patients with hemophilia. ICH is the leading cause of mortality from bleeding in this population, and the reported prevalence of head trauma-related ICH in patients with hemophilia ranges from 2% to 16%.ICH is a life threatening complication of hemophilia. The correction of factor VIII to haemostatic level alone is inadequate in the majority of cases, and there is sudden deterioration in the patient's condition and death.3 An important factor in the treatment of hemophiliacs with head injury is the direct and continuous participation of both hematologist and neurosurgeon. The combined expertise of both disciplines is necessary to achieve a successful outcome for these patients. If the two specialties are not available for consultation, the patient should be transferred to a facility with such expertise.8 In addition to CFC replacement and neurosurgery, aggressive airway management is essential for comatose or incapacitated patients and may also be required as adjunctive management for injury-associated increased intracranial pressure.5 CFC replacement dosing in these group of patients is governed by three principles. Infuse enough to ensure a normal physiologic level initially (at least 100% within the normal range), Infuse frequently enough to ensure that the FVIII level never falls to a non-physiologic level (i.e., a minimum of 50% with a 10-20% “cushion” for confidence) and last monitor FVIII levels as frequently as feasible to ensure that these physiologic levels are being achieved. For hemophilia A, this generally implies an initial acute dose of FVIII of approximately 50 IU/kg followed by repeated bolus dosing every 8-12 hours. The exact frequency depends on FVIII measurements. If neurosurgery is required, the second dose (and perhaps subsequent doses as well) will likely be needed much sooner because of high FVIII clearance during surgery. Correspondingly, increased FVIII monitoring will be needed for such clinical scenarios. The duration of CFC replacement therapy following CNS bleeding is less well-defined than dosing. Many investigators attempt to maintain circulating factor levels above 50% at all times up to three weeks following a CNS bleed in order to minimize the re-bleed risk. A longer duration may be needed if an extensive neurosurgical procedure was required to treat the patient. These CFC infusion recommendations make the critical assumption that no FVIII inhibitor is present. In circumstances in which observed haemostatic effect is less than expected, having confirmation about inhibitor status can be most important. When an inhibitor is known to be present in an individual with an acute CNS bleed alternative haemostatic replacement strategies will be needed. This can entail infusion of higher doses of FVIII or of the so-called “bypassing agents” such as activated prothrombin concentrates (aPCCs) or recombinant factor VIIa (rFVIIa).3,5,8

Conclusion Retroclival SDHs are rare and could be posttraumatic or spontaneous. Though an uncommon entity, retroclival hematomas are an important diagnostic finding to make due to their potential for significant neural compression as well as their association with underlying osseous and ligamentous injury, especially in the pediatric population. Fatal intracranial hemorrhage is a common cause of death in patients with hemophilia A and usually follows trivial injury. Absence of findings on initial CT scans is not sufficient to rule out significant intracranial injury and repeat CTs should be used for patients with persistent symptoms or new signs of neurologic deterioration. Most cases of RSDH exhibit a benign clinical course with conservative management, but significant and profound morbidity and mortality have been reported. Surgical management is dictated based on the presence of hydrocephalus, brainstem compression, and occipitocervical instability. Alongside the neurosurgical management medical management with CFC replacement and close observation in a monitored setup is of paramount importance.

Table 1 Literature review of pediatric RSDH Literatur Year Age sex Mechanism e Ahn 2005 4y M Fall, from a four story window Myers 1995 17y M Hemophilia, Fall down

Physical findings

Surgery

Lt side Hemiparesis

No

Long term deficits None

No

Died

No

None

Casey

2009

18y

M

Trivial injury

Comatose, No brainstem reflexes GCS- 13

Sridhar

2010

18y

M

Fall from a two wheeler

Bilateral 6th nerve palsy

Yes

None

Dayarant e

2015

6y

F

Fall from a bicycle

Quadriparesis

Yes

None

Nguyen

2016

8y

M

MVA

No

None

Silvera

2014

3m

M

Abusive

GCS-14, Bilateral 6th nerve palsy ***

***

***

1m

F

Abusive

***

***

***

3m

M

Abusive

***

***

***

1m

M

Abusive

***

***

***

36m

M

Abusive

***

***

***

30m 7m

M F

Abusive Abusive

*** ***

*** ***

*** ***

7m 3m

F M

Abusive Abusive

*** ***

*** ***

*** ***

4m

M

Abusive

***

***

***

4m

M

Abusive

***

***

***

30m

F

Abusive

***

***

***

M-Male, F-Female, GCS-Glasgow coma scale, MVA-Motor vehicle accident, *** No data

Table 2 Literature review of adult retroclival subdural hematoma Literatur Yea Age Se Mechanism Physical Exam Surgery e r x Narvid

201 5

58

M

64

F

64

M

67

M

Spontaneou s Spontaneou s Spontaneou s Spontaneou s Pituitary Apoplexy

Azizyan

201 5

Mea n 55

8M 2F

Mohame d

201 3

37

M

Pituitary Apoplexy

Krishnan

201 3

59

F

Low platelet count

Schievink 200 1

49

F

Spontaneou s

Sridhar

201 0

19

M

Van Rijn

200 3

72

Kim

201 2

Brock Yadav

NF

No

Long term deficits None

Other features

NF

No

None

IVH

Diplopia

No

None

***

Unresponsive in the ED 9 out of 10 exhibited opthalmoplegia

No

None

IVH

8 out of 10 surgery for Pituitary adenoma (did not address RCH) Surgery for Pituitary

***

***

***

IVH

Left 3rd nerve palsy, Temporal field cut, Decrease acuity Flexing both limbs to pain Extensor plantars NF

No

Partially improved 3rd nerve palsy Died

No

None

***

Fall from a moving bus

NF

No

None

***

M

Spontaneou s

***

***

83

F

None

***

201 0

52

F

Ruptured Pcomm aneurysm Infra clinoid aneurysm

Bilateral 6th *** nerve palsy, Leg paresis Confusion Coil embo for aneurysm rd Rt side 3 and Aneurysm 4th Nerve palsy clipping

None

***

201 5

20

M

Hemophilia Assault

Seizure, Minor external injuries

Died

Frontal ICH, ASDH,

No

Left Convexit y SDH

SAH Deepak

201 8

30

F

Caraman ti

201 9

51

M

On anticoagulan t (Warfarin) Wood log fell on head of patient

Isolated bilateral 6th and left 12th nerve palsy Rt. Leg paresis (power- 4/5) +ve Lasegue sign on the Rt. side

***

***

***

L4-S1 Improved Spinal hemilamine weaknes cord ctomy for s and injury Spinal cord resolution with L4SDH, of RCH S1 SDH, bilateral Craniotomy on 30 for ASDH, days intra RCH not follow up cranial treated ASDH surgically M-Male, F-Female, ED- Emergency Department, RCH-Retroclival hematoma, SDHSubdural hematoma, ASDH- Acute subdural hematoma, Pcomm- Posterior communicating artery, ICH-Intracranial hematoma, *** No data

Figure 1A- Axial CT scan of patient showing a hyper dense well defined retroclival collection with extension to the left tentorium.

Figure 1B-Sagittal CT scan of patient with a hyperdense retroclival hematoma extending from dorsum sella to the inferior part of C2 vertebral body

Figure 1C- coronal CT scan of patient showing associated hyperdense collection in the left posterior fossa, cerebellar convexity

A

B

C

Figure 2- Axial T1W (A) and T2W (B) and Coronal FLAIR (C) MRI of patient showing a crescent shaped extraxial collection along the anterior and posterolateral aspect of the left cerebellar hemisphere extending to prepontine extraxial space. The collection measures 6mm in thickness at maximum point of collection and has T1 shortening (hyper intense on T1W) and prolongation on T2W and FLAIR sequences

A

B

C Figure 3- T2W sagittal brain (A), T1W sagittal C-spine (B) and T2W sagittal C-spine (C) MRI of the patient with retroclival subdural hematoma with the spinal canal portion of the collection spanning from foramen magnum to C7 level. The collection causes no significant mass effect on adjacent structures.

A

B Figure 1- Axial (A) and Sagittal (B) Computed tomography (CT) of the patient showing retroclival subdural hematoma

A

B

C Figure 2- Axial T1W (A), Axial T2W (B) and Coronal FLAIR (C) MRI of the patient with retroclival subdural hematoma with right cerebellar extension.

A

B

C Figure 3- T2W sagittal brain (A), T1W sagittal C-spine (B) and T2W sagittal C-spine (C) MRI of the patient with retroclival subdural hematoma extending to the cervical spine

Abbreviations AAD------------------------------------------------------------------------------Atlanto Axial Dislocation AAU-------------------------------------------------------------------------------Addis Ababa University AEDH-------------------------------------------------------------------------Acute Epidural Hematoma AOD-------------------------------------------------------------------------Atlanto Occipital Dislocation APCCs-----------------------------------------------------------Activated Prothrombin Concentrates ASDH------------------------------------------------------------------------Acute Subdural Hematoma CCJ------------------------------------------------------------------------------Cranio Cervical Junction CFC--------------------------------------------------------------------------Clotting Factor Concentrate CT---------------------------------------------------------------------------------Computed Tomography ED---------------------------------------------------------------------------------Emergency Department EDH------------------------------------------------------------------------------------Epidural Hematoma GCS---------------------------------------------------------------------------------Glasgow Coma Scale ICH---------------------------------------------------------------------------------Intracranial Hematoma ICU--------------------------------------------------------------------------------------Intensive Care Unit INR-----------------------------------------------------------------------International Normalized Ratio IU-------------------------------------------------------------------------------------------International Unit MRI------------------------------------------------------------------------Magnetic Resonance imaging PT-----------------------------------------------------------------------------------------Prothrombin Time PTT-------------------------------------------------------------------------Partial Thromboplastin Time RCH-------------------------------------------------------------------------------Retro Clival Hematoma REDH-------------------------------------------------------------------Retroclival Epidural Hematoma RSDH------------------------------------------------------------------Retroclival Subdural Hematoma SDH-----------------------------------------------------------------------------------Subdural Hematoma STIR-----------------------------------------------------------------------Short T-1 Inversion Recovery

Disclosure All authors declare no conflict of interest