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Coleman P. Riordan and R. Michael Scott

OBJECTIVE

In patients with syringomyelia and type I Chiari malformation (CM-I) who have required reoperation because of persistent, recurrent, or expanding syrinx, the senior author placed a stent from the fourth ventricle to the cervical subarachnoid space in hopes of promoting circulation of CSF out of the ventricle and away from the central canal of the spinal cord. This study was undertaken to determine the long-term success of this operative stratagem in eliminating the syrinx, as well as to document the complications that occurred following stent placement. The technique utilized for placement of fourth ventricle stents is presented.

METHODS

The surgical database of the senior author was reviewed to identify all patients who underwent stent placement at a reexploration of a suboccipital decompression for a CM-I conducted for a recurrent or ineffectively treated syringomyelia. The clinical and radiological data of these patients were analyzed to determine long-term efficacy and complications of the procedure.

RESULTS

Fourteen patients (average age 10.7 ± 5.2 years, range 2.6–20.1 years) were identified who met these inclusion criteria. They each presented with recurrent, residual, or expanding syringomyelia following a prior decompression for a CM-I. The reoperation with stent placement was complicated by late stent dislodgement and recurrence or persistence of the syrinx in 2 patients (14%) and by neurological deficit in 1 patient (7%). There was 1 perioperative CSF leak (7%). In 1 other patient (7%), the stent dislodged after surgery but required no further intervention, as the syrinx remained collapsed. Two patients (14%) required late reoperation for stent replacement when syrinxes recurred. At the most recent imaging follow-up, the stent was positioned appropriately in 12 patients (86%; average follow-up 6.9 years, range 0.5–18.1 years), and the recurrent or residual syrinx was eliminated or reduced in size by 75% or greater in 13 patients (93%).

CONCLUSIONS

The placement of a stent from the fourth ventricle to the cervical subarachnoid space was a highly effective treatment for patients with recurrent, residual, or expanding syringomyelia following an initial decompression of an associated CM-I. The sole neurological complication in this series was related to lysis of arachnoid scar rather than stent placement itself, but inability to maintain fixation of the stent in situ led to further surgery to replace the stent in 2 patients.

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Coleman P. Riordan and R. Michael Scott

OBJECTIVE

In patients with syringomyelia and type I Chiari malformation (CM-I) who have required reoperation because of persistent, recurrent, or expanding syrinx, the senior author placed a stent from the fourth ventricle to the cervical subarachnoid space in hopes of promoting circulation of CSF out of the ventricle and away from the central canal of the spinal cord. This study was undertaken to determine the long-term success of this operative stratagem in eliminating the syrinx, as well as to document the complications that occurred following stent placement. The technique utilized for placement of fourth ventricle stents is presented.

METHODS

The surgical database of the senior author was reviewed to identify all patients who underwent stent placement at a reexploration of a suboccipital decompression for a CM-I conducted for a recurrent or ineffectively treated syringomyelia. The clinical and radiological data of these patients were analyzed to determine long-term efficacy and complications of the procedure.

RESULTS

Fourteen patients (average age 10.7 ± 5.2 years, range 2.6–20.1 years) were identified who met these inclusion criteria. They each presented with recurrent, residual, or expanding syringomyelia following a prior decompression for a CM-I. The reoperation with stent placement was complicated by late stent dislodgement and recurrence or persistence of the syrinx in 2 patients (14%) and by neurological deficit in 1 patient (7%). There was 1 perioperative CSF leak (7%). In 1 other patient (7%), the stent dislodged after surgery but required no further intervention, as the syrinx remained collapsed. Two patients (14%) required late reoperation for stent replacement when syrinxes recurred. At the most recent imaging follow-up, the stent was positioned appropriately in 12 patients (86%; average follow-up 6.9 years, range 0.5–18.1 years), and the recurrent or residual syrinx was eliminated or reduced in size by 75% or greater in 13 patients (93%).

CONCLUSIONS

The placement of a stent from the fourth ventricle to the cervical subarachnoid space was a highly effective treatment for patients with recurrent, residual, or expanding syringomyelia following an initial decompression of an associated CM-I. The sole neurological complication in this series was related to lysis of arachnoid scar rather than stent placement itself, but inability to maintain fixation of the stent in situ led to further surgery to replace the stent in 2 patients.

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Coleman P. Riordan, Darren B. Orbach, Edward R. Smith and R. Michael Scott

OBJECTIVE

The most significant adverse outcome of intracranial hemorrhage from an arteriovenous malformation (AVM) is death. This study reviews a single-center experience with pediatric AVMs to quantify the incidence and characterize clinical and radiographic factors associated with sudden death from the hemorrhage of previously undiagnosed AVMs in children.

METHODS

A single-center database review of the period from 2006 to 2017 identified all patients with a first-time intracranial hemorrhage from a previously undiagnosed AVM. Clinical and radiographic data were collected and compared between patients who survived to hospital discharge and those who died at presentation.

RESULTS

A total of 57 patients (average age 10.8 years, range 0.1–19 years) presented with first-time intracranial hemorrhage from a previously undiagnosed AVM during the study period. Of this group, 7/57 (12%) patients (average age 11.5 years, range 6–16 years) suffered hemorrhages that led directly to their deaths. Compared to the cohort of patients who survived their hemorrhage, patients who died were 4 times more likely to have an AVM in the posterior fossa. No clear pattern of antecedent triggering activity (sports, trauma, etc.) was identified, and 3/7 (43%) experienced cardiac arrest in the prehospital setting. Surviving patients were ultimately treated with resection of the AVM in 42/50 (84%) of cases.

CONCLUSIONS

Children who present with hemorrhage from a previously undiagnosed intracranial AVM had a 12% chance of sudden death in our single-institution series of pediatric cerebrovascular cases. Clinical triggers of hemorrhage are unpredictable, but subsequent radiographic evidence of a posterior fossa AVM was present in 57% of fatal cases, and all fatal cases were in locations with high risk of potential herniation. These data support a proactive, aggressive approach toward definitive treatment of AVMs in children.