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Hani J. Marcus, Fahid T. Rasul, Ziad Hussein, Stephanie E. Baldeweg, Helen A. Spoudeas, Richard Hayward, Noor ul Owase Jeelani, Dominic Thompson, Joan P. Grieve, Neil L. Dorward and Kristian Aquilina


The management of children with craniopharyngioma has evolved over time, with a trend toward less invasive neurosurgical approaches as surgeons have sought to balance oncological control and treatment-related morbidity. To this end, the aim of this study was to evaluate the safety and effectiveness of the current management of children with craniopharyngioma compared to the previous management methods used at the authors’ treatment center.


A prospectively maintained database was searched over a 14-year period between January 1, 2005, and December 31, 2018, to identify all children 17 years of age or younger with a new diagnosis of craniopharyngioma. A retrospective case note review was performed for each child to extract data on the presentation, investigation, treatment, and outcome of their illness. Morbidity was assessed in the same fashion as in previous cohorts, according to the following categories: visual loss, pituitary dysfunction, hypothalamic dysfunction, neurological deficits, and cognitive impairment.


In total, 59 children were identified with craniopharyngioma during the study period. A total of 92 operations were performed, including cyst drainage (35/92; 38.0%), craniotomy and resection (30/92; 32.6%), and transsphenoidal resection (16/92; 17.4%). Approximately two-thirds of all operations were performed using image guidance (66/92; 71.7%) and one-third were performed using endoscopy (27/92; 29.3%). The majority of children had adjuvant therapy comprising proton beam therapy (18/59; 30.5%) or conventional radiotherapy (16/59; 27.1%). The median follow-up duration was 44 months (range 1–142 months), and approximately one-half of the children had no evidence of residual disease on MRI studies (28/59; 47.5%). Of the remaining 31 children, there was a reduction in the volume of residual disease in 8 patients (8/59; 13.6%), stable residual disease in 18 (18/59; 30.5%), and tumor growth in 5 patients (5/59; 8.5%). There was significantly reduced morbidity (p < 0.05) in all categories in the current cohort compared with our last cohort (1996–2004).


The authors’ institutional experience of pediatric craniopharyngioma confirms a trend toward less invasive neurosurgical procedures, most of which are now performed with the benefit of image guidance or endoscopy. Moreover, the authors have identified an expanding role for more targeted radiotherapy for children with residual disease. These advances have allowed for tumor control comparable to that achieved in previous cohorts, but with significantly reduced morbidity and mortality.

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Richard Hayward, Jonathan A. Britto, David Dunaway, Robert Evans, Noor ul Owase Jeelani and Dominic Thompson

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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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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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Charles Kondageski, Dominic Thompson, Mandy Reynolds and Richard D. Hayward


The overdrainage of cerebrospinal fluid (CSF) in children with shunt-treated hydrocephalus may cause chronic disabling symptoms and require repeated surgery. Externally adjustable valves offer a noninvasive way of altering the valve opening pressure. The authors report on their experience with using the Strata valve in the management of symptomatic CSF overdrainage.


The authors treated 24 patients with symptomatic CSF overdrainage by inserting a Strata valve. The severity of symptoms was graded, and the frequency of hospital visits and shunt operations was recorded before and after insertion of the valve. Additionally, results of brain imaging and intracranial pressure monitoring were reviewed. Nineteen patients (79.2%) had severe symptoms at the time of the insertion; 1 year after Strata valve insertion only one patient (4.17%) still suffered severe symptoms. The number of hospital admissions was 3.38/patient/year before placement and 1.21 for the 1st year, 1 for the 2nd, and 0.4 for the 3rd postoperative year. The number of operations was 3.42/patient/year during the year before placement of the valve, and then 0.71 for the 1st, 0.56 for the 2nd, and 0.25 for the 3rd postoperative years. During the 1st year after placement of the Strata valve, the settings were changed 2.79 times/patient/year, 1.29 for the 2nd, and 1.33 times/patient/year for the 3rd year.


The Strata valve was effective in improving the symptoms of overdrainage in the majority of patients in this series. The number of hospital admissions and operations for valve malfunction was reduced.

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Ali Nader-Sepahi, Adrian T. H. Casey, Richard Hayward, H. Alan Crockard and Dominic Thompson


The aim of this study was to audit the treatment of symptomatic atlantoaxial instability in Down syndrome and to assess factors associated with fusion failure in this group of patients.


The authors reviewed the cases of 12 children with Down syndrome presenting with symptomatic atlantoaxial instability who underwent surgery for internal fixation and fusion. A review of clinical histories, radiological investigations, and details of operative interventions was also performed.

The mode of presentation was acute spinal cord injury (five cases), progressive myelopathy (four cases), and neck pain or stiffness (three cases). The atlantodental interval ranged from 5 to 13 mm. The posterior atlantodental interval at the C-1 level was 9.5 mm (range 6–11 mm). In 10 patients an os odontoideum was present. Translocation of the odontoid process occurred in one patient, and occipitoatlantal instability was also identified in two cases. Twenty-four operations were performed in the 12 patients. A transoral odontoidectomy was required in four children. Successful fusion was demonstrated in seven patients at the first operation. Three of the five patients with acute cord injury made significant functional recovery and were left with no or mild disability.


Additional bone abnormalities at the CVJ are common in the Down syndrome population. Young age at the time of fusion and multiple osseous anomalies pose a higher risk factor in fusion failure. The authors recommend an aggressive surgical approach for management of symptomatic cases of CVJ instability.

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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.

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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.