Gregory G. Heuer, Erin S. Schwartz and Phillip B. Storm
Jennifer Hong, Jared M. Pisapia, Zarina S. Ali, Austin J. Heuer, Erin Alexander, Gregory G. Heuer and Eric L. Zager
Neurogenic thoracic outlet syndrome (nTOS) is an uncommon compression syndrome of the brachial plexus that presents with pain, sensory changes, and motor weakness in the affected limb. The authors reviewed the clinical presentations and outcomes in their series of pediatric patients with surgically treated nTOS over a 6-year period.
Cases of nTOS in patients age 18 years or younger were extracted for analysis from a prospective database of peripheral nerve operations. Baseline patient characteristics, imaging and neurophysiological data, operative findings, and outcomes and complications were assessed.
Twelve patients with 14 cases of nTOS surgically treated between April 2010 and December 2016 were identified. One-third of the patients were male, and 2 male patients underwent staged, bilateral procedures. Disabling pain (both local and radiating) was the most common presenting symptom (100%), followed by numbness (35.7%), then tingling (28.6%). The mean duration of symptoms prior to surgery was 15.8 ± 6.6 months (mean ± SD). Sports-related onset of symptoms was seen in 78.6% of cases. Imaging revealed cervical ribs in 4 cases, prominent C-7 transverse processes in 4 cases, abnormal first thoracic ribs in 2 cases, and absence of bony anomalies in 4 cases. Neurophysiological testing results were normal in 85.7% of cases. Conservative management failed in all patients, with 5 patients reporting minimal improvement in symptoms with physical therapy. With a mean follow-up after surgery of 22 ± 18.3 months (mean ± SD), pain relief was excellent (> 90%) in 8 cases (57.1%), and good (improved > 50%) in 6 cases (42.9%). On univariate analysis, patients who reported excellent pain resolution following surgery at long-term follow-up were found to be significantly younger, and to have suffered a shorter duration of preoperative symptoms than patients who had worse outcomes. Lack of significant trauma or previous surgery to the affected arm was also associated with excellent outcomes. There were 4 minor complications in 3 patients within 30 days of surgery: 1 patient developed a small pneumothorax that resolved spontaneously; 1 patient suffered a transient increase in pain requiring consultation, followed by hiccups for a period of 3 hours that resolved spontaneously; and 1 patient fell at home, with transient increased pain in the surgically treated extremity. There were no new neurological deficits, wound infections, deep vein thromboses, or readmissions.
Pediatric nTOS commonly presents with disabling pain and is more frequently associated with bony anomalies compared with adult nTOS. In carefully selected patients, surgical decompression of the brachial plexus results in excellent pain relief, which is more likely to be seen in younger patients who present for early surgical evaluation.
Gregory G. Heuer, Michelle J. Smith, J. Paul Elliott, H. Richard Winn and Peter D. Leroux
Object. Increased intracranial pressure (ICP) is well known to affect adversely patients with head injury. In contrast, the variables associated with ICP following aneurysmal subarachnoid hemorrhage (SAH) and their impact on outcome have been less intensely studied.
Methods. In this retrospective study the authors reviewed a prospective observational database cataloging the treatment details in 433 patients with SAH who had undergone surgical occlusion of an aneurysm as well as ICP monitoring. All 433 patients underwent postoperative ICP monitoring, whereas only 146 (33.7%) underwent both pre- and postoperative ICP monitoring.
The mean maximal ICP was 24.9 ± 17.3 mm Hg (mean ± standard deviation). During their hospital stay, 234 patients (54%) had elevated ICP (> 20 mm Hg), including 136 of those (48.7%) with a good clinical grade (Hunt and Hess Grades I–III) and 98 (63.6%) of the 154 patients with a poor grade (Hunt and Hess Grades IV and V) on admission. An increased mean maximal ICP was associated with several admission variables: worse Hunt and Hess clinical grade (p < 0.0001), a lower Glasgow Coma Scale (GSC) motor score (p < 0.0001); worse SAH grade based on results of computerized tomography studies (p < 0.0001); intracerebral hemorrhage (p = 0.024); severity of intraventricular hemorrhage (p < 0.0001); and rebleeding (p = 0.0048). Both intraoperative cerebral swelling (p = 0.0017) and postoperative GCS score (p < 0.0001) were significantly associated with a raised ICP. Variables such as patient age, aneurysm size, symptomatic vasospasm, intraoperative aneurysm rupture, and secondary cerebral insults such as hypoxia were not associated with raised ICP. Increased ICP adversely affected outcome: 71.9% of patients with normal ICP demonstrated favorable 6-month outcomes postoperatively, whereas 63.5% of patients with ICP between 20 and 50 mm Hg and 33.3% with ICP greater than 50 mm Hg demonstrated favorable outcomes. Among 21 patients whose raised ICP did not respond to mannitol therapy, all experienced a poor outcome and 95.2% died. Among 145 patients whose elevated ICP responded to mannitol, 66.9% had a favorable outcome and only 20.7% were dead 6 months after surgery (p < 0.0001). According to results of multivariate analysis, however, ICP was not an independent outcome predictor (odds ratio 1.26, 95% confidence interval 0.28–5.68).
Conclusions. Increased ICP is common after SAH, even in patients with a good clinical grade. Elevated ICP post-SAH is associated with a worse patient outcome, particularly if ICP does not respond to treatment. This association, however, may depend more on the overall severity of the SAH than on ICP alone.
Joel A. Bauman, Douglas A. Hardesty, Gregory G. Heuer and Phillip B. Storm
An alternative method of bone grafting for pediatric posterior cervical and occipitocervical fixation is presented in detail. Full-thickness autografts from small craniectomies of the occipital bone are used to augment posterior segmental fusion in pediatric patients. Twelve patients have been treated successfully without bone graft donor site complications. The technical differences from previously reported uses of calvarial autograft in spine fusion are reviewed.
Report of two cases and review of the literature
Gregory G. Heuer, Michael F. Stiefel, Robert L. Bailey and James M. Schuster
✓Spinal ependymomas are a common type of primary spinal cord neoplasm that frequently occurs in the lumbar spine. The authors report on two patients who presented with acute neurological decline after hemorrhage into ependymomas of the filum terminale. Both were transferred to the authors' institution because of diagnostic uncertainty and a concern about possible intradural vascular abnormalities. Both patients underwent lumbar laminectomies for tumor resection. The pathological finding in each case was myxopapillary ependymoma. Both patients made a significant recovery and were ambulatory and continent at follow-up review. These cases illustrate the rare but clinically significant incidence of acute neurological decline caused by hemorrhagic cauda equina ependymomas, including the potential for delayed diagnosis and treatment.
Gregory G. Heuer, Kareem A. Zaghloul, Richard Roberts, Michael F. Stiefel and Phillip B. Storm
✓ Coil migration is a rare but potentially serious complication of endovascular procedures. Occasionally coils can be retrieved via endovascular techniques. The authors describe the microsurgical management of a case in which endovascular techniques failed. A 2-year-old girl with pulmonary atresia and a Blalock–Taussig shunt underwent attempted endovascular closure of the shunt with Gianturco steel coils. During deployment, a coil was lost in the aorta and an angiogram showed that it had lodged in the proximal M1 segment of the middle cerebral artery. The coil could not be retrieved by endovascular techniques, and the patient was taken to the operating room to undergo a craniotomy. After the sylvian fissure was split, the coil was visible through the vessel wall. Temporary clips were placed on the proximal M1 and the proximal M2 segments, trapping the coil. A small arteriotomy was performed, the coil was removed, and the arteriotomy was closed. A cerebral angiogram showed excellent perfusion with no dissections. The patient’s motor examination demonstrated a mild hemiparesis on the left with no tremulousness. Coil migration can be treated by microsurgical techniques in pediatric patients with a good clinical outcome.
Zarina S. Ali, Eric L. Zager, Gregory G. Heuer and Sherman C. Stein
Shih-Shan Lang, Joel A. Bauman, Michael W. Aversano, Matthew R. Sanborn, Arastoo Vossough, Gregory G. Heuer and Phillip B. Storm
Electrolyte and endocrinological complications of endoscopic third ventriculostomy (ETV) are infrequent but serious events, likely due to transient hypothalamic-pituitary dysfunction. While the incidence of diabetes insipidus is relatively well known, hyponatremia is not often reported. The authors report on a series of 5 patients with post-ETV hyponatremia.
The records of patients undergoing ETV between 2008 and 2010 were reviewed. All ETVs were performed with a rigid neuroendoscope via a frontal bur hole, standard third ventricle floor blunt perforation, Fogarty catheter dilation, and intermittent normal saline irrigation. Postoperative MR images were evaluated for endoscope tract injury as well as the trajectory from the bur hole center to the fenestration site.
Thirty-two patients (20 male and 12 female) underwent ETV. Their median age was 6 years (range 3 weeks–28 years). Hydrocephalus was most commonly due to nontumoral aqueductal stenosis (43%), nontectal tumor (25%), or tectal glioma (13%). Five patients (16%) had multicystic/loculated hydrocephalus. Five patients (16%) developed hyponatremia between 1 and 8 days following ETV, including 2 patients with seizures (1 of whom was still hospitalized at the time of the seizure and 1 of whom was readmitted as a result of the seizure) and 3 patients who were readmitted because of decline in their condition following routine discharge. No hypothalamic injuries were noted on imaging. Univariate risk factors consisted of age of 2 years or less (p = 0.02), presence of cystic lesions (p = 0.02), and ETV trajectory angle 10° or more from perpendicular (p = 0.001).
Endoscopic third ventriculostomy is a well-tolerated procedure but can result in serious complications. Hyponatremia is rare and may be more likely in younger patients or those with cystic loculations. Patients with altered craniometry may be at particular risk with a rigid endoscopic approach requiring greater manipulation of subforniceal or hypothalamic structures.
Shih-Shan Lang, Lauren A. Beslow, Robert L. Bailey, Arastoo Vossough, Joanna Ekstrom, Gregory G. Heuer and Phillip B. Storm
The true postoperative incidence of arteriovenous malformation (AVM) recurrence in the pediatric population remains largely unreported. Some literature suggests that delayed imaging studies should be obtained at 6 months to 1 year after negative findings on a postoperative angiogram. The aim of this study was to describe the timing of AVM recurrences after resection and the neuroimaging modalities on which the recurrences were detected.
This study was performed in a retrospective cohort of all pediatric patients treated surgically for AVM resection by a single neurosurgeon between 2005 and 2010. Patients were followed after resection with MR angiography (MRA) or conventional angiography, when possible, at various time points. A visual scale for compactness of the initial AVM nidus was used, and the score was correlated with probability of recurrence after surgery.
A total of 28 patients (13 female, 15 male) underwent an AVM resection. In 18 patients (64.3%) an intraoperative angiogram was obtained. In 4 cases the intraoperative angiogram revealed residual AVM, and repeat resections were performed immediately. Recurrent AVMs were found in 4 children (14.3%) at 50, 51, 56, and 60 weeks after the initial resection. Recurrence risk was 0.08 per person-year. No patient with normal results on an angiogram obtained at 1 year developed a recurrence on either a 5-year angiogram or one obtained at 18 years of age. All patients with recurrence had a compactness score of 1 (diffuse AVM); a lower compactness score was associated with recurrence (p = 0.0003).
All recurrences in this cohort occurred less than 15 months from the initial resection. The authors recommend intraoperative angiography to help ensure complete resection at the time of the surgery. Follow-up vascular imaging is crucial for detecting recurrent AVMs, and conventional angiography is preferred because MRA can miss smaller AVMs. One-year follow-up imaging detected these recurrences, and no one who had negative results on an angiogram obtained at 1 year had a late recurrence. However, not all of the patients have been followed for 5 years or until 18 years of age, so longer follow-up is required for these patients. A lower compactness score predicted recurrent AVM in this cohort.