Infantile hemangiomas are tumors commonly seen in children. Few authors have reported infantile hemangiomas affecting the CNS, and there are no prior reports detailing spontaneous resolution of a histologically proven juvenile hemangioma within a dorsal root ganglion. The authors report the case of a newborn boy with a large cutaneous hemangioma in the midline of his back. Spinal MR images were obtained to rule out associated spinal cord tethering, and an intradural spinal lesion was unexpectedly discovered. Biopsy revealed an intradural infantile hemangioma within the dorsal root ganglion, and, based on this diagnosis, no resection was performed. Sixteen months following the biopsy, the cutaneous hemangioma had become involuted and the intradural hemangioma had completely resolved. The behavior of the intradural component in this case follows the natural history of many cutaneous infantile hemangiomas.
Shawn L. Hervey-Jumper, Paul E. McKeever, Stephen S. Gebarski, Karin M. Muraszko, and Cormac O. Maher
Tethered cord release
Joseph H. Piatt Jr.
Wajd N. Al-Holou, Karin M. Muraszko, Hugh J. Garton, Steven R. Buchman, and Cormac O. Maher
After primary repair of a myelomeningocele or a lipomyelomeningocele, patients can present with symptoms of secondary tethered cord syndrome (TCS). After surgical untethering, a small percentage of these patients can present with multiple repeat TCS. In patients presenting with secondary or multiple repeat TCS, the role as well the expected outcomes of surgical untethering are not well defined.
Eighty-four patients who underwent spinal cord untethering after at least 1 primary repair were retrospectively evaluated using scaled and subjective outcome measures at short-term and long-term follow-up visits. Outcomes were analyzed for predictive measures using multivariate logistic regression.
Surgical untethering was performed in 66 patients with myelomeningoceles and 18 patients with lipomyelomeningoceles. Fourteen patients underwent multiple repeat spinal cord untethering. Patients were followed up for an average of 6.2 years. Most patients had stability of function postoperatively. Motor function and weakness improved in 7 and 16% of patients at 6 months, respectively, and 6 and 19% of patients at long-term follow-up evaluation, respectively. Of the patients who presented with back pain, 75% had improvement in symptoms at 6 months postoperatively. Younger age at untethering was significantly associated with worse long-term neurological outcomes. The number of previous untethering procedures, original diagnosis, sex, anatomical level, and degree of untethering had no effect on surgical outcomes.
Patients presenting with secondary or multiple repeat TCS may benefit from surgical untethering.
Linear vein–based arteriovenous malformations
Elisa J. Kucia and Robert F. Spetzler
Cormac O. Maher, Lilliana Goumnerova, Joseph R. Madsen, Mark Proctor, and R. Michael Scott
Patients who have undergone prior myelomeningocele or lipomyelomeningocele repair may present with symptomatic retethering of the spinal cord. In some cases, symptomatic tethering may recur after previous untethering operations. The expected outcome following repeated untethering in a patient after two or more prior untethering operations is not well described.
The authors examined surgical indications, techniques, and outcomes for 30 repeated untethering operations in 22 patients who had undergone a previous repair of the primary spinal disorder and at least two subsequent untethering operations.
The mean age at repeated untethering was 12.3 years. Presenting symptoms were pain (70%), weakness (70%), urinary symptoms (57%), and sensory changes (27%). The mean duration of symptoms was 7.5 months, and a longer symptomatic interval correlated with an increased number of prior operations. Total circumferential untethering was accomplished in 11 cases (37%). Postoperative symptomatic improvement was noted most often for pain (81%), and less often for urinary symptoms (53%) and weakness (48%). Complications included postoperative cerebrospinal fluid leakage or pseudomeningocele and new postoperative lower-extremity dysesthesia in five cases (17%). An increasing number of prior untethering operations was associated with a worse result for pain relief and a greater chance of significant morbidity.
Multiple repeated untethering operations offer symptomatic relief to well-selected patients with this condition.
Cormac O. Maher, Robert J. Spinner, Caterina Giannini, Bernd W. Scheithauer, and Brian A. Crum
✓ The authors report the findings of a neuromuscular choristoma of the sciatic nerve in an otherwise healthy 18-year-old man who presented with sensorimotor symptoms and deformities of the right leg and foot. Only a few cases of this rare tumor, also known as “neuromuscular hamartoma” or “benign triton tumor,” have been reported in the surgical literature. The authors discuss the clinical presentation, radiological findings, pathological diagnosis, and surgical rationale for this case and review the associated literature.
Aqueel Pabaney, Shawn L. Hervey-Jumper, Joseph Domino, Cormac O. Maher, and Lynda J. S. Yang
Neuropathic pain is rare in children, and few reports provide adequate guidelines for treatment. The authors describe the successful treatment of tardy neuropathic pain via macrovascular decompression in a 15-year-old boy who presented with progressive pain 11 years following trauma to the upper extremity that had required surgical repair of the brachial artery. Examination revealed mild chronic median and ulnar motor neuropathy as well as recent progressive lancinating pain and a Tinel sign at the prior scar. A soft tissue mass in the neurovascular bundle at the site of previous injury was noted on MRI. Surgical exploration demonstrated an altered anatomical relationship of the previously repaired brachial artery and the median nerve, resulting in pulsatile compression of the median nerve by the brachial artery. Neurolysis and decompression of the median nerve with physical separation from the brachial artery resulted in immediate pain relief.
This is the first report of macrovascular decompression of a major peripheral nerve with complete symptom resolution. Noninvasive imaging together with a thorough history and physical examination can support identification of this potential etiology of peripheral neuralgic pain. Recognition and treatment of this uncommon problem may yield improved outcomes for children with neuropathic pain.
Wajd N. Al-Holou, Edward A. O'Hara, Aaron A. Cohen-Gadol, and Cormac O. Maher
Our current understanding of nonaccidental head injury in children is the result of decades of effort and the tireless work of numerous physicians. In 1860 Auguste Ambroise Tardieu, a French forensics expert, recognized important patterns of injury in children and identified nonaccidental trauma as the cause of these injuries. His work was ignored. In the years that followed, physicians continued to report these patterns of injury but were unable to identify the etiology. A fundamental misunderstanding of the usual cause of subdural hematoma (SDH) contributed to the confusion at that time. Early in the 20th century, neurosurgeons such as Wilfred Trotter recognized that SDHs were traumatic in origin. However, even Trotter's efforts to expose faults in the theories that SDHs primarily resulted from inflammatory or infectious processes were not accepted immediately. Eventually, the pattern of injuries in children was again recognized both by neurosurgeons, who began to identify an association between trauma-induced SDHs and retinal hemorrhages, and by radiologists, who began to note SDHs in conjunction with osseous lesions. Not until the 1950s and 1960s, however, did physicians begin to routinely identify nonaccidental trauma as the cause of these injuries. Following the recognition of child abuse, a pattern of injuries in conjunction with shaking was identified and is currently known as shaken baby syndrome. Since its identification, our understanding of this syndrome has been modified as a result of new medical research, legal challenges, and popular media forces.
Wajd N. Al-Holou, Andrew Y. Yew, Zackary E. Boomsaad, Hugh J. L. Garton, Karin M. Muraszko, and Cormac O. Maher
Arachnoid cysts are a frequent finding on intracranial imaging in children. The prevalence and natural history of these cysts are not well defined. The authors studied a large consecutive series of children undergoing MR imaging to better define both the MR imaging–demonstrated prevalence and behavior of these lesions over time.
The authors reviewed a consecutive series of 11,738 patients who were 18 years of age or younger and had undergone brain MR imaging at a single institution during an 11-year period. In the patients in whom intracranial arachnoid cysts were identified, clinical and demographic information was recorded and imaging characteristics, such as cyst size and location, were evaluated. Prevalence data were analyzed using univariate and multivariate logistic regression, linear regression, and ANOVA. All patients with sufficient data (repeat MR imaging studies as well as repeated clinical evaluation over at least 5 months) for a natural history analysis were identified. This group was assessed for any change in symptoms or imaging appearance during the follow-up interval.
Three hundred nine arachnoid cysts (2.6% prevalence rate) were identified. There was an increased prevalence of arachnoid cysts in males (p < 0.000001). One hundred eleven patients met all criteria for inclusion in the natural history analysis. After a mean follow-up of 3.5 years, 11 arachnoid cysts increased in size, 13 decreased, and 87 remained stable. A younger age at presentation was significantly associated with cyst enlargement (p = 0.001) and the need for surgery (p = 0.05). No patient older than 4 years of age at the time of initial diagnosis had cyst enlargement, demonstrated new symptoms, or underwent surgical treatment.
Arachnoid cysts are a common incidental finding on intracranial imaging in pediatric patients. An older age at the time of presentation is associated with a lack of clinical or imaging changes over time.
Jennifer Strahle, Karin M. Muraszko, Hugh J. L. Garton, Brandon W. Smith, Jordan Starr, Joseph R. Kapurch II,, and Cormac O. Maher
Syrinx size and location within the spinal cord may differ based on etiology or associated conditions of the brain and spine. These differences have not been clearly defined.
All patients with a syrinx were identified from 14,118 patients undergoing brain or cervical spine imaging at a single institution over an 11-year interval. Syrinx width, length, and location in the spinal cord were recorded. Patients were grouped according to associated brain and spine conditions including Chiari malformation Type I (CM-I), secondary CM (2°CM), Chiari malformation Type 0 (CM-0), tethered cord, other closed dysraphism, and spinal tumors. Syringes not associated with any known brain or spinal cord condition were considered idiopathic. Syrinx characteristics were compared between groups.
A total of 271 patients with a syrinx were identified. The most common associated condition was CM-I (occurring in 117 patients [43.2%]), followed by spinal dysraphism (20 [7.4%]), tumor (15 [5.5%]), and tethered cord (13 [4.8%]). Eighty-three patients (30.6%) did not have any associated condition of the brain or spinal cord and their syringes were considered idiopathic. Syringes in patients with CM-I were wide (7.8 ± 3.9 mm) compared with idiopathic syringes (3.9 ± 1.0, p < 0.0001) and those associated with tethered cord (4.2 ± 0.9, p < 0.01). When considering CM-I–associated and idiopathic syringes, the authors found that CM-I–associated syringes were more likely to have their cranial extent in the cervical spine (88%), compared with idiopathic syringes (43%; p < 0.0001). The combination of syrinx width greater than 5 mm and cranial extent in the cervical spine had 99% specificity (95% CI 0.92–0.99) for CM-I–associated syrinx.
Syrinx morphology differs according to syrinx etiology. The combination of width greater than 5 mm and cranial extent in the cervical spine is highly specific for CM-I–associated syringes. This may have relevance when determining the clinical significance of syringes in patients with low cerebellar tonsil position.