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Mark R. Proctor and R. Michael Scott

Object

Split cord malformations (SCMs) are relatively rare forms of occult spinal dysraphism (OSD) and tethered spinal cord syndrome. The majority of these cases present in early childhood, with neurocutaneous stigmata being an early presenting feature. Prophylactic detethering surgery is advocated by most neurosurgeons due to the risk of neurological deterioration over time caused by patient growth and activity. However, unlike other forms of OSD, the course of SCM progression after surgery is not well understood, and little has been published about long-term follow-up results. In this study the authors review the results obtained in 16 patients in whom the senior author performed surgery over a 13-year period (average length of follow up almost 8 years).

Methods

Presentation, surgical approach, and outcome are evaluated, and the long-term outcome of neurological status, pain, bowel/bladder disturbance, and spinal deformities are emphasized.

Conclusions

The primary conclusion is that patients with SCM generally tolerate surgery well and experience few complications. Neurological deterioration is rare except in cases in which retethering occurs, (two patients in this series). Although impaired bowel and bladder function was stabilized or improved and pain was reliably relieved postoperatively, preexisting vertebral column deformities usually progressed after surgery and, in most cases, required spinal fusion.

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S. Alex Rottgers, Subash Lohani and Mark R. Proctor

OBJECTIVE

Historically, bilateral frontoorbital advancement (FOA) has been the keystone for treatment of turribrachycephaly caused by bilateral coronal synostosis. Early endoscopic suturectomy has become a popular technique for treatment of single-suture synostosis, with acceptable results and minimal perioperative morbidity. Boston Children's Hospital has adopted this method of treating early-presenting cases of bilateral coronal synostosis.

METHODS

A retrospective review of patients with bilateral coronal craniosynostosis who were treated with endoscopic suturectomy between 2005 and 2012 was completed. Patients were operated on between 1 and 4 months of age. Hospital records were reviewed for perioperative morbidity, length of stay, head circumference and cephalic indices, and the need for further surgery.

RESULTS

Eighteen patients were identified, 8 males and 10 females, with a mean age at surgery of 2.6 months (range 1–4 months). Nine patients had syndromic craniosynostosis. The mean duration of surgery was 73.3 minutes (range 50–93 minutes). The mean blood loss was 40 ml (range 20–100 ml), and 2 patients needed a blood transfusion. The mean duration of hospital stay was 1.2 days (range 1–2 days). There was 1 major complication in the form of a CSF leak. The mean follow-up was 37 months (range 6–102 months). Eleven percent of nonsyndromic patients required a subsequent FOA; 55.6% of syndromic patients underwent FOA. The head circumference percentiles and cephalic indices improved significantly.

CONCLUSIONS

Early endoscopic suturectomy successfully treats the majority of patients with bilateral coronal synostosis, and affords a short procedure time, a brief hospital stay, and an expedited recovery. Close follow-up is needed to detect patients who will require a secondary FOA due to progressive suture fusion or resynostosis of the released coronal sutures.

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Kathryn V. Isaac, John G. Meara and Mark R. Proctor

OBJECTIVE

Comparative effectiveness studies are needed for analyzing the clinical outcomes of treatment of sagittal craniosynostosis (SC) with endoscopic suturectomy (ES) or cranial vault remodeling (CVR).

METHODS

From 2004–2015, patients were consecutively reviewed and data recorded for operative details and complications. Cranial growth was measured by head circumference percentile (HCP) and cranial index (CI) Z-score. Aesthetic outcomes were compared using the Whitaker classification.

RESULTS

A total of 207 patients with nonsyndromic SC were treated with ES (n = 187) or CVR (n = 20). The ES group had a lower median operative duration (45 vs 195 minutes), length of stay (1 vs 3 days), and transfusion rate (2% vs 85%; p < 0.0001). Median age at follow-up was 3.0 years (interquartile range [IQR] 2.5–4.5, ES) and 3.9 years (IQR 2–5, CVR; p = 0.12). In both groups, HCP gradually decreased during a 3-year follow-up (p = 0.282). CI Z-scores were initially more favorable in the ES group (p < 0.05); 3 years following surgical intervention, Z-scores were equal between groups (p = 0.392). One nonsyndromic patient treated with ES (0.5%) demonstrated reossification and required secondary expansion. Most patients were Whitaker class I (99% of ES, 95% of CVR); 5% of CVR patients were class II and 1% of ES patients were class III. Four syndromic patients presented without clear evidence of a syndrome, were treated by ES, and subsequently required secondary expansion for raised intracranial pressure.

CONCLUSIONS

ES is an effective treatment for nonsyndromic SC with comparable head growth, aesthetic outcomes, and less morbidity relative to CVR. In the absence of a syndrome, secondary cranial expansion following ES is rarely required.

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Kathryn V. Isaac, John G. Meara and Mark R. Proctor

The authors compared the effectiveness of two main surgical techniques used for treating sagittal craniosynostosis (SC): endoscopic suturectomy (ES) and cranial vault remodeling (CVR). The safety, head growth, and aesthetic results following ES and CVR were compared by reviewing the charts of more than 200 patients. By comparing the effectiveness of these two treatments, this study will help guide selection of the optimal surgical treatment for patients with SC.

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Corinna C. Zygourakis, Kevin S. Cahill and Mark R. Proctor

A previously healthy 2-year-old girl sustained a C1–2 ligamentous injury after a motor vehicle accident and underwent successful halo immobilization, with postimmobilization images showing good cervical alignment. At the time, plain radiography, CT scanning, and MR imaging showed a normal odontoid. Four years later, however, the patient was found to have an os odontoideum, evident on plain radiography and CT imaging. At the 10-year follow-up, the os odontoideum had not been surgically repaired, and the child had mild hypermobility.

This is the first documented case in the modern imaging era of delayed os odontoideum formation after definitive CT scanning showed no fracture. As such, this suggests that os odontoideum may result from traumatic vascular interruption in the developing spine, with resulting osseous remodeling leading to an os odontoideum. This case argues against the congenital etiology of os odontoideum, as well as the strict posttraumatic theory whereby a trauma-induced odontoid fracture leads to osseous remodeling and subsequent development of an os odontoideum.

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Ning Lin, Clemens M. Schirmer and Mark R. Proctor

Disc cysts are rare intraspinal extradural lesions that communicate with the intervertebral disc and can mimic the symptoms of acute lumbar disc herniation. Initially reported in the Japanese-language literature as a new entity (discal cyst), there are few documented cases in North America, and only 1 prior case in the pediatric population. The authors present the case of a 16-year-old girl with an intervertebral disc cyst causing lumbar radiculopathy that progressed despite conservative treatment. All medical records, imaging studies, intraoperative findings, and pertinent literature were reviewed. Serial preoperative MR imaging revealed enlargement of the intraspinal cyst at the L4–5 level, resulting in compression of the right L-5 nerve root. Enlargement of the cyst occurred over a 4-month period despite conservative treatment with physical therapy and corticosteroid injections. Microsurgical discectomy and excision of the cyst resulted in complete resolution of the preoperative radiculopathy. An intervertebral disc cyst is a rare entity in the adult population and exceedingly rare in the pediatric population but should remain in the differential diagnosis of any intraspinal extradural mass. The authors hypothesize that there exists a spectrum of this entity that may not be responsive to conservative therapy. Cyst excision alone or in conjunction with microsurgical discectomy is safe and effective in treating radiculopathy caused by disc cysts.

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Subash Lohani, Richard L. Robertson and Mark R. Proctor

Arachnoid cyst is a common congenital anomaly in the pediatric population. The cysts are often asymptomatic, but they can cause headache and other symptoms. Occasionally a cyst may rupture after head trauma producing a subdural hematoma. The authors present the case of an 11-year-old boy who presented after a week of progressive and severe back pain radiating to the back of his thighs. Imaging revealed a spinal subdural blood collection at the L4–S1 level. This finding prompted further cephalad imaging of the spine and the brain, which revealed a sylvian fissure arachnoid cyst with intracystic hemorrhage and frontoparietal subdural hematoma. The child did not have headache at this time, although he had experienced chronic headaches since the age of 4 years. He was treated with a course of oral steroids, which immediately relieved his back and leg pain. Subsequent imaging showed resolution of the cranial and spinal subdural blood collections and diminished size of the arachnoid cyst. No surgical treatment was necessary.

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Kevin S. Cahill, Ian Dunn, Thorsteinn Gunnarsson and Mark R. Proctor

Object

Lumbar disc herniation is a rare but significant cause of pain and disability in the pediatric population. Lumbar microdiscectomy, although routinely performed in adults, has not been described in the pediatric population. The objective of this study was to determine the surgical results of lumbar microdiscectomy in the pediatric population by analyzing the experiences at Children's Hospital Boston over the past decade.

Methods

A series of 87 consecutive cases of lumbar microdiscectomy performed by the senior author (M.R.P.) from 1999 to 2008 were reviewed. Presenting symptoms, physical examination findings, and preoperative MR imaging findings were obtained from medical records. Immediate operative results were assessed including operative duration, blood loss, length of stay, and complications, along with long-term outcome and need for repeat surgery.

Results

This series represents the first surgical series of pediatric microdiscectomies. The mean patient age was 16.6 years (range 12–18 years) and 60% were female. The preoperative physical examination results were notable for motor deficits in 26% of patients, sensory changes in 41%, loss of deep tendon reflex in 22%, and a positive straight leg raise in 95%. Conservative management was the first line of treatment in all patients and the mean duration of symptoms until surgical treatment was 12.2 months. The mean operative time was 110 minutes and the mean postoperative length of stay was 1.3 days. Complications were rare: postoperative infection occurred in 1%, postoperative CSF leak in 1%, and new postoperative neurological deficits in 1%. Only 6% of patients needed repeat lumbar surgery and 1 patient ultimately required lumbar fusion.

Conclusions

The treatment of pediatric lumbar disc herniation with microdiscectomy is a safe procedure with low operative complications. Nuances of the presentation, treatment options, and surgery in the pediatric population are discussed.

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Ian F. Dunn, Mark R. Proctor and Arthur L. Day

✓Lumbar spine injuries in athletes are not uncommon and usually take the form of a mild muscle strain or sprain. More severe injuries sustained by athletes include disc herniations, spondylolistheses, and various types of fracture. The recognition and management of these injuries in athletes involve the additional consideration that to return to play, the lumbar spine must be able to withstand forces similar to those that were injurious. The authors consider common lumbar spine injuries in athletes and discuss management principles for neurosurgeons that are relevant to this population.

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Subash Lohani, Diana P. Rodriguez, Hart G. W. Lidov, R. Michael Scott and Mark R. Proctor

Object

Intrasacral meningoceles are rare cystic lesions that can cause focal compression within the bony sacral canal. Their mechanisms are poorly understood, but most intrasacral meningoceles appear to be intrasacral extradural cysts caused by arachnoid herniating through a small dural defect in the caudal end of the thecal sac. As opposed to perineural cysts, they are not associated with an exiting nerve root. When symptomatic, they can cause sacral pain or sacral nerve root dysfunction due to local compression.

Methods

This is a retrospective series from Boston Children's Hospital. All patients in whom symptomatic intrasacral meningocele that required surgical treatment was diagnosed between May 1994 and March 2011 were included in the study. Spine MRI was the diagnostic modality of choice. All patients underwent sacral exploration, with ligation and obliteration of the cyst. Resected cyst wall was subjected to pathological examination.

Results

There were 13 patients (11 boys and 2 girls) who underwent operation for intrasacral meningocele. The median age was 8 years (range 5 months–16 years). The most common presenting symptom was back pain (in 5) often described as deep tail bone pain, followed by urinary incontinence (3) and constipation (2). Three patients had evidence of associated tethered cord on MRI studies. Four patients were asymptomatic and their diagnosis was made following imaging for other reasons; they were surgically treated because of the increasing size of the lesion or association with other congenital lesions. Most patients had symptomatic improvement after surgery.

Conclusions

Intrasacral meningoceles are rare lesions that may result from a congenital dural weakness and a resultant arachnoid diverticulum. They present in childhood either incidentally or with symptoms secondary to nerve root compression. Identification of the point of herniation through the dura mater and ligation of the lesion provides cyst cure and resolution of symptoms in most patients.