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Martin M. Tisdall, Richard D. Hayward, and Dominic N. P. Thompson


A dermal sinus tract is a common form of occult spinal dysraphism. The presumed etiology relates to a focal failure of disjunction resulting in a persistent adhesion between the neural and cutaneous ectoderm. Clinical and radiological features can appear innocuous, leading to delayed diagnosis and failure to appreciate the implications or extent of the abnormality. If it is left untreated, complications can include meningitis, spinal abscess, and inclusion cyst formation. The authors present their experience in 74 pediatric cases of spinal dermal tract in an attempt to identify which clinical and radiological factors are associated with an infective presentation and to assess the reliability of MRI in evaluating this entity.


Consecutive cases of spinal dermal tract treated with resection between 1998 and 2010 were identified from the departmental surgical database. Demographics, clinical history, and radiological and operative findings were collected from the patient records. The presence or absence of active infection (abscess, meningitis) at the time of neurosurgical presentation and any history of local sinus discharge or infection was assessed. Magnetic resonance images were reviewed to evaluate the extent of the sinus tract and determine the presence of an inclusion cyst. Radiological and operative findings were compared.


The surgical course was uncomplicated in 90% of 74 cases eligible for analysis. Magnetic resonance imaging underreported the presence of both an intradural tract (MRI 46%, operative finding 86%) and an intraspinal inclusion cyst (MRI 15%, operative finding 24%). A history of sinus discharge (OR 12.8, p = 0.0003) and the intraoperative identification of intraspinal inclusion cysts (OR 5.6, p = 0.023) were associated with an infective presentation. There was no significant association between the presence of an intradural tract discovered at surgery and an infective presentation.


Surgery for the treatment of spinal dermal tract carries a low morbidity. While it seems intuitive that tracts without intradural extension carry a low risk of spinal cord tethering, it is not possible to reliably detect these cases using MRI. Similarly, intraspinal dermoid cannot be reliably excluded using MRI and carries an increased risk of infection. These points justify excision together with intradural exploration of all spinal dermal sinus tracts.

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Olivier Klein and Dominic Thompson

✓The authors present the case of a 3-month-old girl who presented with lipomyelomeningocele (LMM) associated with a low-lying conus and a terminal syrinx. At the time of presentation, the girl had a mild neurological, but congenital, deficit: weakness of the right foot and calf as well as absence of the right ankle reflex. Bladder assessment was normal, and it was decided to monitor this child closely, without performing surgery. Results of further magnetic resonance imaging performed 1 year after diagnosis and when the girl was 28 months old, showed a significant regression of LMM and associated syrinx. Interestingly, the child remained neurologically unchanged. Although described in the literature, spontaneous regression of LMM is a very unusual situation, and very few cases have been reported. This case represents the first report of concomitant regression of an associated syrinx. Close follow-up of nonsurgically treated children is mandatory, as the risk of late deterioration remains.

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Dora Steel, Matthew A. Kirkman, Dominic N. P. Thompson, and Kristian Aquilina

Open anterolateral cordotomy is an effective treatment option for adults with intractable pain, but it has seldom been attempted in children. In the 2 previously reported cases in children, the procedure was used within 10 days of death from neoplastic disease. In this paper the authors describe 2 cases in which open cordotomy was used successfully in children outside the immediate terminal phase of disease. Both patients experienced effective analgesia with minimal adverse effects. The authors propose that consideration of cordotomy as an option for the management of intractable pain in children does not need to be delayed until death is imminent.

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Juan F. Martínez-Lage

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Michael D. Cearns, Samantha Hettige, Paolo De Coppi, and Dominic N. P. Thompson


It is well established that Currarino syndrome (CS) may be associated with spinal dysraphism. Here, the authors report on 10 CS patients with dysraphic anomalies who had undergone a combined neurosurgical and general surgical approach to repair the dysraphic anomalies and resect the presacral mass in a single operation. They discuss the spectrum of spinal dysraphism that may coexist in CS in the context of its developmental etiology.


Children with a confirmed CS diagnosis who had undergone the combined operative approach were identified from a departmental database. Presenting features were recorded and preoperative imaging was analyzed to record features of the presacral mass and the dysraphic anomalies. The histopathological nature of the resected presacral mass and the outcomes postoperatively and at the last follow-up were reviewed.


Between 2008 and 2015, 10 patients presented with CS, 9 with constipation. Median age at the time of surgery was 1.3 years. Six of the 10 patients had anorectal malformation consisting of anal stenosis, rectal stenosis, or imperforate anus. Spinal anomalies included anterior meningocele (5 cases), low-lying conus (8), terminal syrinx (4), fatty filum (5), caudal lipoma (3), and intraspinal cyst (1). In all cases, the lumbosacral spinal canal was accessed via a midline approach with laminoplasty, allowing spinal cord untethering and repair of the dysraphic anomalies. Following dural closure, the incision was extended inferiorly to incorporate a posterior sagittal approach to resect the presacral mass. The histopathological nature of the mass was mature teratoma (8 cases), complex hamartomatous malformation (1), or neurenteric cyst (1). There were no new instances of neurological deterioration, with most instances of persisting morbidity related to constipation (6 cases) or neurogenic bladder dysfunction (8). There were no infective complications, no instances of cerebrospinal fluid fistula, no recurrences of the presacral mass, and no cases of retethering of the spinal cord.


Although not part of the original triad, spinal dysraphic anomalies are common in CS and in keeping with a disorder of secondary neurulation. Lumbosacral MRI is an essential investigation when CS is suspected. Children are at risk of sphincter impairment due to the anorectal malformation; however, both spinal cord tethering and compression from the presacral mass may further compromise long-term continence. A combined operative approach to repair the dysraphic anomalies and resect the presacral mass is described with good postoperative and long-term outcomes.

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Aabir Chakraborty, Darach Crimmins, Richard Hayward, and Dominic Thompson


The prevalence of shunt-dependent hydrocephalus in patients with myelomeningocele has been reported to be in the region of 85%, and shunt-related complications are a significant cause of morbidity and mortality in these patients. Since 1997 the authors have adopted a stringent policy with respect to shunt placement in patients with myelomeningocele, reserving this treatment for those with symptomatic hydrocephalus, severe ventricular dilation at the time of presentation, and/or unequivocal progressive ventriculomegaly after primary closure. They report their experience.


The authors reviewed all cases of myelomeningocele involving patients who presented to their institution over a 10-year period. They excluded cases in which the primary closure was carried out at another institution or in which there was not at least 12 months of clinical and imaging follow-up. Data regarding shunt insertion shunt-related complications, and clinical outcome were obtained from a review of the clinical records and analyzed.


Fifty-four cases satisfied the inclusion criteria for this study. Shunt insertion was performed in 28 of these cases (51.9%).


Applying more stringent guidelines for shunt placement, permitting moderate ventricular dilation, and accepting some mild increase in ventricular size after myelomeningocele closure has resulted in a reduced rate of shunt placement compared with previous series. The rate is comparable to that reported following in utero closure of myelomeningocele.

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Kanna K. Gnanalingham, Jesus Lafuente, Dominic Thompson, William Harkness, and Richard Hayward

Object. Traditionally, access to the posterior fossa involved a suboccipital craniectomy. More recently, posterior fossa craniotomies have been described, although the long-term benefits of this procedure are not clear. The authors compared the postoperative complications of craniectomies and craniotomies in children with posterior fossa tumors.

Methods. From a total of 110 children undergoing surgery for posterior fossa tumors, 56 underwent craniectomy and 54 had a craniotomy. The mean duration of the hospital stay was longer in the craniectomy group (17.5 compared with 14 days). At operation, similar numbers of patients in both groups had total macroscopic clearance of the tumor, complete dural closure, and duraplasty.

Postoperatively, more patients in the craniectomy group were noted to have cerebrospinal fluid (CSF) leakage (27 compared with 4%; p < 0.01) and pseudomeningoceles (23 compared with 9%; p < 0.05). There was no significant difference between the two groups in the numbers of patients with CSF infections, wound infections, or hydrocephalus requiring permanent CSF drainage. Patients with CSF leaks had a longer duration of hospital stay (20.7 compared with 14.9 days; p < 0.01), and were more likely to have CSF infections (35 compared with 12%; p < 0.01) and wound infections (24 compared with 1%; p < 0.01) than patients without CSF leaks. Postoperatively, wound exploration and reclosures for CSF leakage were more likely in the craniectomy group (11 compared with 0%; p < 0.01). Multivariate analysis revealed that the only predictor of CSF leakage postoperatively was the type of surgery (that is, craniotomy compared with craniectomy; odds ratio 10.8; p = 0.03).

Conclusions. Craniectomy was associated with postoperative CSF leaks, pseudomeningocele, increased wound reclosures, and thus prolonged hospital stays. In turn, CSF leakage was associated with infections of the CSF and wound. The authors propose mechanisms that may explain why CSF leakage is less likely if the bone flap is replaced.

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John E. Crossman, Dominic Thompson, Richard D. Hayward, Andrew O. Ransford, and H. Alan Crockard

✓ Atlantoaxial rotatory fixation (AARF) is an uncommon condition of childhood. Occasionally AARF may recur. The authors describe the cases of four patients with recurrent AARF (RAARF).

The probable cause of the RAARF and operative procedure selected are discussed. In three cases, attempts were made to stabilize the atlantoaxial complex rather than to perform fusion to preserve the function of the joint complex.

Joint stabilization is performed by incorporating a “check ligament” into the joint construct between the axial spinous process and the atlantal lateral mass. The authors believe this technique of joint stabilization augments the strength of the joint, allowing normal, but preventing excessive, rotation, until the joint reaches physiological maturity.