Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is classified under trigeminal autonomic cephalalgias. This rare headache syndrome is infrequently associated with secondary pathologies. In this paper the authors report on a patient with paroxysmal left retroorbital pain with associated autonomic symptoms of ipsilateral conjunctival injection and lacrimation, suggestive of SUNCT syndrome. After failed medical treatment an MRI sequence was obtained in this patient, demonstrating an epidermoid tumor in the left cerebellopontine angle. The patient's symptoms completely resolved after a gross-total resection of the tumor. This case demonstrates the effectiveness of resection as definitive treatment for SUNCT syndrome associated with tumoral compression of the trigeminal nerve. Early MRI studies should be considered in all patients with SUNCT, especially those with atypical signs and symptoms.
Shaun D. Rodgers, Bryan J. Marascalchi, Russell G. Strom and Paul P. Huang
Stephen P. Kalhorn, Russell G. Strom and David H. Harter
Hydrocephalus caused by stenosis of the foramen of Monro is rare. The authors describe a 28-year-old female patient with bilateral foraminal stenosis treated using endoscopic septostomy and unilateral foraminal balloon plasty (foraminoplasty). The patient's hydrocephalus and symptoms resolved postoperatively. Endoscopic strategies may be employed as first-line therapy in this condition.
Robert E. Elliott, Stephen A. Sands, Russell G. Strom and Jeffrey H. Wisoff
Controversy persists concerning the optimal treatment of craniopharyngiomas in children, and no standard outcome metric exists for comparison across treatment modalities, nor is there one that adequately reflects the multisystem dysfunction that may arise.
The authors retrospectively analyzed the records of 86 consecutive children who underwent a uniform treatment paradigm of attempted radical resection performed by a single surgeon. Excluding 3 perioperative deaths and 3 patients with inadequate follow-up, 80 children (34 girls and 46 boys; mean age 9.56 years; mean follow-up 9.6 years) composed the study group (53 primary and 27 previously treated/recurrent tumors). Building on existing classification schemes proposed by De Vile for hypothalamic dysfunction and Wen for overall functional outcome, the authors devised a more nuanced classification system (Craniopharyngioma Clinical Status Scale [CCSS]) that assesses outcome across 5 axes, including neurological examination, visual status, pituitary function, hypothalamic dysfunction, and educational/occupational status at last follow-up (there is a 4-tiered grading scale in each domain, with increasing values reflecting greater dysfunction).
There was a significant increase in pituitary dysfunction following treatment—consistent with the high rates of diabetes insipidus and hypopituitarism common to the surgical management of craniopharyngiomas—and less dramatic deterioration in hypothalamic function or cognitive domains. Significant improvement in vision was also demonstrated, with no significant overall change in neurological status. Preoperative CCSS scores predicted postoperative outcome better than clinical characteristics like patient age, sex, tumor size, and the location or presence of hydrocephalus.
Preoperative CCSS scores predicted outcome with higher accuracy than clinical or imaging characteristics. In lieu of randomized trials, the CCSS may provide a useful outcome assessment tool for comparison across treatment paradigms and surgical approaches. Long-term follow-up is critical to the analysis of outcomes of craniopharyngioma treatment, given the often-delayed sequelae of all therapies and the high recurrence rates of these tumors.
Russell G. Strom, Junseok Bae, Jun Mizutani, Frank Valone III, Christopher P. Ames and Vedat Deviren
Lateral interbody fusion (LIF) with percutaneous screw fixation can treat adult spinal deformity (ASD) in the coronal plane, but sagittal correction is limited. The authors combined LIF with open posterior (OP) surgery using facet osteotomies and a rod-cantilever technique to enhance lumbar lordosis (LL). It is unclear how this hybrid strategy compares to OP surgery alone. The goal of this study was to evaluate the combination of LIF and OP surgery (LIF+OP) for ASD.
All thoracolumbar ASD cases from 2009 to 2014 were reviewed. Patients with < 6 months follow-up, prior fusion, severe sagittal imbalance (sagittal vertical axis > 200 mm or pelvic incidence-LL > 40°), and those undergoing anterior lumbar interbody fusion were excluded. Deformity correction, complications, and outcomes were compared between LIF+OP and OP-only surgery patients.
LIF+OP (n = 32) and OP-only patients (n = 60) had similar baseline features and posterior fusion levels. On average, 3.8 LIFs were performed. Patients who underwent LIF+OP had less blood loss (1129 vs 1833 ml, p = 0.016) and lower durotomy rates (0% vs 23%, p = 0.002). Patients in the LIF+OP group required less ICU care (0.7 vs 2.8 days, p < 0.001) and inpatient rehabilitation (63% vs 87%, p = 0.015). The incidence of new leg pain, numbness, or weakness was similar between groups (28% vs 22%, p = 0.609). All leg symptoms resolved within 6 months, except in 1 OP-only patient. Follow-up duration was similar (28 vs 25 months, p = 0.462). LIF+OP patients had significantly less pseudarthrosis (6% vs 27%, p = 0.026) and greater improvement in visual analog scale back pain (mean decrease 4.0 vs 1.9, p = 0.046) and Oswestry Disability Index (mean decrease 21 vs 12, p = 0.035) scores. Lumbar coronal correction was greater with LIF+OP surgery (mean [± SD] 22° ± 13° vs 14° ± 13°, p = 0.010). LL restoration was 22° ± 13°, intermediately between OP-only with facet osteotomies (11° ± 7°, p < 0.001) and pedicle subtraction osteotomy (29° ± 10°, p = 0.045).
LIF+OP is an effective strategy for ASD of moderate severity. Compared with the authors' OP-only operations, LIF+OP was associated with faster recovery, fewer complications, and greater relief of pain and disability.
Daniel Refai, James A. Botros, Russell G. Strom, Colin P. Derdeyn, Aseem Sharma and Gregory J. Zipfel
The clinical characteristics and overall outcome in patients with spontaneous isolated convexity subarachnoid hemorrhage (SAH) are not well described in the literature. The purpose of this study was to examine the mode of presentation, common origins, radiographic findings, and clinical course in a large case series of such patients.
A retrospective single-center chart review of all patients in whom nontraumatic primary convexity SAH was diagnosed between 2002 and 2007 was performed. Twenty patients were identified and analyzed for presenting symptoms, radiological and laboratory findings, hospital course, and outcome.
There were 15 women and 5 men in our series, and the mean age was 52 years (range 18–86 years). The most common presenting symptom was headache, with 15 patients experiencing it as a chief complaint. Other frequent manifestations included altered mental status (8 patients), focal neurological deficits (7), and seizure (4 patients). An underlying cause of the hemorrhage was identified in 13 cases, whereas the remainder went unresolved. Of the known causes, 5 were due to posterior reversible encephalopathy syndrome, 3 were caused by thrombocytopenia or anticoagulation, and the remainder were isolated cases of lupus vasculitis, drug-induced vasculopathy, postpartum cerebral angiopathy, hypertensive microangiopathy, and Call–Fleming syndrome. All patients with unknown disease origins had favorable outcomes, whereas 8 of 13 patients with an identifiable underlying disorder experienced favorable outcomes.
Spontaneous isolated convexity SAH is rarely caused by aneurysm rupture, has a distinct mode of presentation, and generally carries a more favorable prognosis than that of aneurysmal SAH.