✓ Selective dorsal rhizotomy (SDR) is an evidence-based treatment for cerebral palsy (CP) spasticity. During their lifetime, patients with CP spasticity may require orthopedic surgery for muscles and joints to correct physical deformities and provide a better quality of life. In this review, the authors discuss the timing of such orthopedic surgery, its necessity, and whether it is influenced by the performance of SDR. A review of findings from the authors' 19 years of experience yields the following conclusions: 1) that SDR reduces orthopedic surgery requirements when compared with historical controls; 2) that SDR performed in patients at a young age (2–4 years) can reduce future orthopedic surgery requirements; 3) that independent walkers and diplegic patients will have the smallest amount of orthopedic surgery post-SDR; and 4) that patients who need assistance walking and those with quadriplegia will have the greatest amount, although the frequency of orthopedic surgery for quadriplegic patients is not as high as popularly believed.
Donncha F. O'Brien and Tae Sung Park
Michael S. Turner
Donncha F. O'Brien, Tae Sung Park, Joan A. Puglisi, David R. Collins, and Eric C. Leuthardt
Object. The authors performed a long-term evaluation of gait status to determine the frequency with which orthopedic operations for cerebral palsy are conducted before and after selective dorsal rhizotomy (SDR) and the relation between pre- and post-SDR orthopedic surgery and age.
Methods. Fifty-two patients with spastic quadriplegia were prospectively followed for 5 to 9 years. All children were evaluated and underwent SDR at St. Louis Children's Hospital. Preoperative scores for gait function and details of previous orthopedic procedures were recorded for two age groups: those 2 to 5 (Group 1) and those 6 to 14 years of age (Group 2). Data were collected from parents who completed a questionnaire a mean of 7.5 years after SDR. Relations between gait status and the number/type of pre- and post-SDR orthopedic procedures, rate of improvement after SDR, benefit of operation according to parents, and return of spasticity were analyzed.
Forty-nine percent of patients in Group 1 and 25% of those in Group 2 had improved gait scores. The interaction between pre- or post-SDR time frame and walking mode was statistically significant (p = 0.004). Among those children who had not undergone orthopedic surgery before SDR, the incidence of surgery post-SDR was higher in the older children (Group 2) than the younger children (Group 1 [70% compared with 34%]). Parents of 75% of the Group 1 patients and 88% of the Group 2 patients felt that their children benefited from SDR.
Conclusions. The results of this study highlight the effect of SDR on gait status in children with spastic quadriplegic cerebral palsy. The percentage of patients needing orthopedic operations was not as high as reported previously. Parents indicated that SDR was beneficial to their children.
Case report and review of the literature
Kristian Aquilina, Donncha F. O’Brien, Michael A. Farrell, and Ciaran Bolger
✓The authors report on the case of a craniopharyngioma arising in the cerebellopontine angle (CPA) in a patient with Gardner syndrome. Although familial adenomatous polyposis (FAP) is associated with intracranial neoplasms, the current case is only the third reported craniopharyngioma in a patient with Gardner syndrome. Two of these tumors, including that of the current case, originated in the CPA, an unusual location for craniopharyngiomas. The literature concerning FAP and its associations with intracranial neoplasia, as well as the pathogenesis of craniopharyngiomas in the posterior fossa, is discussed.
Donncha F. O’Brien, Caroline Hayhurst, Barry Pizer, and Conor L. Mallucci
The primary aim of this study was to evaluate the success of endoscopic third ventriculostomy (ETV) as a treatment for obstructive hydrocephalus secondary to midline tumors (midbrain, pontine, pineal, tectal plate, thalamic, and third ventricular regions). In addition, the study examined the role and value of endoscopic tumor biopsy (ETB) in the management of such cases. All surgical procedures were performed through a single-trajectory approach.
A retrospective analysis of clinical notes, operation records, and pre- and postventriculostomy neuroimaging data was performed to determine the success or failure and complications of ETV and ETB in 42 patients presenting with tumor-induced obstructive hydrocephalus. Patient data were derived from an endoscopy database initiated in 1998. The study population included 21 female and 21 male patients (mean age 37 years, range 5–77 years). All 42 patients underwent an ETV; 33 of the 42 underwent an ETV and an ETB (single-trajectory). One patient was excluded from the follow-up analysis due to rapid deterioration of his condition from tumor progression. The duration of follow up ranged from 3 to 84 months (mean 32 months).
At the last follow up, 11 patients with ETVs had undergone shunt placement and two patients had undergone repeated ETVs, giving a long-term success rate of 68% (28 of 41 cases) for single ETV as a treatment for hydrocephalus at presentation. Statistical analysis revealed no significant relationship (p > 0.92) between tumor location and ETV success or failure. The mean time to ETV failure was 32 weeks. Histological examination of biopsy specimens was nondiagnostic in eight (24%) of the 33 cases in which ETB was performed. Seven of these cases involved pineal region tumors and one involved a tectal plate tumor. There was no death or major morbidity associated with ETV and ETB in this series.
Endoscopic third ventriculostomy is a safe and durable means of controlling hydrocephalus in tumor cases. Its success rate is high—comparable to that reported in aqueduct stenosis cases. Although ETB is probably not as accurate for diagnosis as biopsy with frame-based stereotactic guidance, it is associated with a lower mortality rate and, in the correct clinical setting, may be justifiably attempted as an initial biopsy procedure at the same time as ETV via a single-trajectory approach.
Donncha F. O'Brien, Mohsen Javadpour, David R. Collins, Pietro Spennato, and Conor L. Mallucci
The authors analyzed the role of endoscopic third ventriculostomy (ETV) as a primary treatment for hydrocephalus and also as an alternative to shunt revision for malfunctioning and infected ventriculoperitoneal (VP) shunts.
A retrospective analysis of clinical notes, operation records, and magnetic resonance imaging procedures before and after ventriculostomy was performed to determine the success or failure of ETVs in 170 patients who underwent a primary ETV and in 63 patients who underwent an ETV for shunt malfunction (secondary ETV). The patients' data were derived from an endoscopy database inaugurated in 1998. Of the 63 patients with shunt malfunctions, 49 patients (78%) had mechanical malfunction only and 14 patients (22%) had both infection and malfunction.
Seventy-four percent (126 of 170) of patients in the primary ETV group and 70% (44 of 63) of patients in the secondary ETV had a successful outcome at the time of analysis. The success rate for ETVs in cases involving a mechanical shunt malfunction alone was 67% (33 of 49) compared with 79% (11 of 14) in those cases involving an infected shunt.
The origin of the hydrocephalus in the primary and shunt malfunction groups was evaluated as a factor contributing to the success of the ETV. In the primary group, patients with a history of intraventricular hemorrhage (IVH) and meningitis as a cause for hydrocephalus had a poor rate of success after the ETV—27% (four of 15) and 0% (none of two), respectively. This pattern was not seen in the series involving shunt malfunction after the ETV, with 71% (five of seven) and 75% (three of four) of cases having a hydrocephalus origin of IVH and meningitis, respectively, in which a successful outcome was attained. A two-way mixed-model analysis of variance yielded a significant effect for origin (p = 0.011), a significant interaction between group and origin (p = 0.028), and a marginally nonsignificant effect of group (p = 0.0686). More than 95% of failures were evident within 1 month of the ETV in both groups. Complications were minimal in both groups, and there were no procedure-related deaths.
An ETV is a safe procedure with few complications and a high success rate in both primary and secondary groups. An ETV to address shunt malfunction, unlike a primary ETV, is not particularly origin specific. A bonus is its success in dealing with infected shunts. Most failures will be evident early, but long-term follow up is vital.
Donncha F. O'Brien, Tae-Sung Park, Joan A. Puglisi, David R. Collins, Eric C. Leuthardt, and Jeffrey R. Leonard
A retrospective study was performed to determine the following: 1) whether children who walk independently after selective dorsal rhizotomy (SDR) undergo fewer subsequent orthopedic operations than those who walk with assistance; and 2) the effect of age at SDR on the rate of orthopedic operations.
The cases of 158 children with spastic diplegia who were 2 to 14 years of age when they underwent SDR were followed over a 5- to 9-year period. Patients were grouped by age at the time of SDR as follows: 2 to 3 years (Group 1), 4 to 7 years (Group 2), and 8 to 14 years (Group 3). Follow-up data showed that children in all age groups who walked independently after SDR underwent fewer orthopedic operations than did children who walked with assistance. Overall rates of orthopedic surgery 5 to 9 years after SDR at last follow up were 24% for independent walkers and 51% for assisted walkers. Two-way categorical analysis (age group by ambulation) yielded a highly significant effect of ambulation (p = 0.0003). Children in Group 1 needed the fewest orthopedic operations at follow-up evaluation. In the older age groups (Groups 2 and 3), those who walked independently at the time of SDR underwent fewer orthopedic operations after SDR than did walkers who required assistance (p = 0.01).
These data are of value in advising parents about the likelihood of orthopedic surgery based on the child's gait status both at the time of SDR and at follow-up evaluation. Orthopedic surgery is more likely in patients destined to be nonambulators.
Konstantina Karabatsou, Caroline Hayhurst, Neil Buxton, Donncha F. O'Brien, and Conor L. Mallucci
Neuroendoscopy is increasingly used in neurosurgery. The authors report their evolving experience in the management of arachnoid cysts using endoscopic techniques and, more recently, the use of these techniques in combination with neuronavigation systems. The aim of this study was to assess the efficacy of this approach and the factors influencing the final outcome of treatment.
The authors reviewed 39 cases in which patients were treated endoscopically for intracranial arachnoid cysts over a period of 8 years. The indications and techniques used were reviewed and the surgical outcomes assessed. There was no death or significant morbidity associated with the procedure. Thirty-six patients had resolution or improvement of symptoms and only three required insertion of a shunt.
The treatment of arachnoid cysts has been revolutionized by the introduction of endoscopic techniques. The authors conclude on the basis of their experience that in most cases the combination of neuroendoscopy and frameless navigation represents a safe and reliable modality for treating this benign intracranial entity with minimal surgical trauma. The specific approach should be based on the individual characteristics of each cyst and the surgeon's experience.
Jecko V. Thachil, Mark Caswell, Russell Keenan, Caroline Hayhurst, Daniel A. Crooks, Paul L. May, and Donncha F. O’Brien
✓The authors report their experience in successfully treating a 15-week-old child who became comatose following a spontaneous intracerebral hemorrhage. It was initially believed that a tumor in the right frontal lobe caused the hemorrhage. Coagulation studies revealed abnormal results on presentation, and the problem was only partially corrected after an infusion of fresh frozen plasma. The child underwent an emergency craniotomy in which the hematoma was evacuated, and a biopsy specimen was obtained from a firm mass at the base of the hematoma cavity. Postoperatively, the child recovered completely, and an analysis of detailed coagulation studies revealed that the child had a factor X deficiency. Histological analysis of the biopsy specimen revealed normal brain tissue with hemorrhagic infiltration. Subsequently, the child achieved normal developmental milestones. A diagnosis of congenital bleeding disorder should be considered in children with spontaneous intracerebral hemorrhage, even in those with no prior episode of extracerebral spontaneous hemorrhage.
Chetan D. Parmar, Ajay K. Sinha, Caroline Hayhurst, Paul L. May, and Donncha F. O'Brien
✓ Osteogenesis imperfecta (OI) represents a rare heterogeneous group of inherited disorders characterized by low bone mass, increased bone fragility, and other connective tissue manifestations. This condition can contribute to dramatic complications after a seemingly insignificant injury. A large epidural hematoma that developed in a child with OI after a trivial fall highlights the importance of close monitoring in these patients. After an injury that occurred several months prior to the head trauma the authors describe, this child had been placed in foster care because it was believed that his skeletal injuries were caused by nonaccidental injury. Subsequent genetic analysis confirmed that the child was heterozygous for the missense mutation c767G>T,pG256V at exon 16 of COL1A2, consistent with OI, and the foster care order was overturned. The authors review the literature concerning OI, its relationship to head injury, and the importance of genetic analysis in its diagnosis.