Biomedical subjects

Donncha F O'Brien

Publications and source records attributed to Donncha F O'Brien.

15 recordsLinked to original sources

PubMed2007

Epidural hematoma formation following trivial head trauma in a child with osteogenesis imperfecta. Case report.

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 COLIA2, 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.

Child Abuse↗

PubMed2006

Is there an indication for ETV in young infants in aetiologies other than isolated aqueduct stenosis?

OBJECTIVES: The objective was to determine whether endoscopic third ventriculostomy (ETV) has a role in the management of obstructive hydrocephalus in aetiologies other than idiopathic aqueduct stenosis (AS) in infants. MATERIALS AND METHODS: In addition to reviewing the literature, we performed a retrospective analysis of our endoscopy database, which was established in 1998, and analysed the outcome results of all cases of ETV in those under 1 year of age which were performed between 1998 and 2003. We included cases of idiopathic AS in the outcome analysis as a benchmark of successful outcome. Successful outcome was assessed by resolution of the presenting clinical features and shunt freedom. RESULTS: Aetiologies that were identified included, in addition to idiopathic AS, suprasellar arachnoid cysts, AS in association with post-haemorrhagic hydrocephalus (PHH), post-infectious hydrocephalus (PIH), tumour-related hydrocephalus and a heterogenous group including Dandy-Walker malformation and other developmental anomalies. Suprasellar arachnoid cysts had a 100% outcome success. Idiopathic AS had a 50% outcome success and the successful outcome of PHH cases was poor at 18%. A statistical analysis revealed no effect of age at the time of ETV on the outcome in the aetiological groups. CONCLUSIONS: The definitive initial neurosurgical management of suprasellar arachnoid cysts causing significant hydrocephalus is ETV, whereas that for PHH and PIH is probably that of a ventriculo-peritoneal shunt placement. We speculate that there may be a sub-group of AS cases, termed pure or idiopathic AS, which have a higher rate of successful outcome that may be durable from a younger age. The role of repeat ETV is controversial.

Age Factors↗

PubMed2006

A review of orthopedic surgeries after selective dorsal rhizotomy.

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.

Cerebral Palsy↗

PubMed2006

Malignant disseminated chordoid meningioma in a 12-year-old child: a role for early cranial and spinal radiation treatment after subtotal resection.

CASE REPORT: We present a case of a chordoid meningioma, a tumour sub-type which comprises less than 0.5% of all meningiomas. The patient, a 12-year-old Somalian girl with a history of having had a craniotomy for a brain tumour in her native country 11 months previously, presented with seizures. Imaging revealed an isolated left frontal tumour, which at the time was felt to be a residual mass. She underwent a macroscopically complete resection of this tumour. Histology revealed it to be a grade 2 chordoid meningioma. Recurrence with additional local meningeal lesions was detected on follow-up magnetic resonance imaging (MRI) at 2 months post surgery. These were resected and she was treated with adjuvant cranial radiation treatment that resulted in non-progression of the cranial tumour on imaging 6 and 12 months post surgery. However, at 12 months post initial presentation, she re-presented with disseminated spinal disease refractory to salvage radiation treatment and succumbed to her illness. OUTCOME: This is the first reported case of cranial radiation treatment being used to treat sub-totally resected recurrent chordoid meningioma. Whilst intra-cranial control of the tumour was achieved with this management, it did not prevent spinal progression. The authors advise a high-surveillance management strategy when treating these lesions and to employ cranial and spinal radiation treatment at the first sign of disease progression.

Child↗

PubMed2006

Meningeal melanocytoma.

BACKGROUND: Meningeal melanocytoma was first described over 30 years ago as a benign tumour derived from melanocytes. Since then, data suggest that its mode of presentation is variable without a clear predilection for any particular site in the neuroaxis. Although classified as a benign tumour, this tumour shows a marked tendency towards reduced survival following subtotal resection and transformation over time in a limited number to malignant melanoma. Incomplete resection of these tumours without postoperative radiotherapy has only a 42% 5-year survival rate. Its classification as a benign tumour should be revised, given the published 5-year survival data. ILLUSTRATIVE CASE: We report a fatal case of meningeal melanocytoma in the cerebello-pontine angle in a 10-year-old child. This case exemplifies the vascular nature of these lesions even with minimal vascular blush on angiography. An updated literature search is presented, the results of which highlight the need for close follow-up and adjuvant treatment following subtotal resection.

Case-Control Studies↗

PubMed2006

Anatomical hemispherectomy for intractable seizures: excellent seizure control, low morbidity and no superficial cerebral haemosiderosis.

OBJECTIVE: This current study was performed to evaluate whether superficial cerebral haemosiderosis (SCH) is still a complication of modern day anatomical hemispherectomy. METHODS: We report a 13-year institutional experience with anatomical hemispherectomy for intractable epilepsy. Seizure control at a mean follow-up interval of 7 years was 83%. Though one patient died post-operatively from a non-neurosurgical complication, mortality was otherwise zero and morbidity minimal. The much-described complication of SCH following anatomical hemispherectomy was non-existent. We explain the history of SCH as a complication of anatomical hemispherectomy, and the measures that are presently taken to prevent it. CONCLUSIONS: We suggest that the importance of SCH in modern epilepsy surgery is probably over-emphasised.

Adolescent↗

PubMed2006

Primary cerebellopontine angle craniopharyngioma in a patient with gardner syndrome. Case report and review of the literature.

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.

Adult↗

PubMed2006

Factor X deficiency presenting as a pseudotumor. Case report.

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.

Brain Neoplasms↗

PubMed2006

Outcomes in patients undergoing single-trajectory endoscopic third ventriculostomy and endoscopic biopsy for midline tumors presenting with obstructive hydrocephalus.

OBJECT: 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. METHODS: 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 non-diagnostic 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. CONCLUSIONS: 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.

Adolescent↗

PubMed2005

Management of birth brachial plexus palsy.

INTRODUCTION: The indications for surgical repair of congenital brachial plexus palsy are controversial. Our objective was to determine the results of early brachial plexus surgery following obstetric-induced brachial plexus palsy. METHODS: We performed a retrospective analysis of the outcome of 58 cases of brachial plexus surgery. The indication for operation consisted of the presence of less than antigravity strength in the biceps, triceps, and deltoid muscle groups at 6 months of age. Data gathered prospectively, previously, showed the likelihood of improvement with less than antigravity strength in these cases to be poor. RESULTS: Follow-up data were obtained on 52 of the 58 cases. Overall mean follow-up was 2 years. Twelve patients had more than 3 years follow-up (mean 5.5 years, range 3-11.5 years). Significant improvement was seen in all injury patterns i.e., C5-C6, C5-C7, and C5-C8, T1. Greater than antigravity strength in the biceps, triceps, and deltoid muscle groups was seen in the majority of cases at follow-up. CONCLUSIONS: Repair of obstetrical brachial plexus palsy in children at 6 months of age that is based on less than antigravity strength in the biceps, triceps, and deltoid muscle groups produces improvement in functional capabilities. Children with obstetrical brachial plexus palsy should be referred soon after birth to a center that specializes in the treatment of this type of palsy.

Brachial Plexus Neuropathies↗

PubMed2005

Bilateral abducent and facial nerve palsies following fourth ventricle shunting: two case reports.

CASE REPORTS: Treatment of isolated fourth ventricle syndrome is difficult and there is no widely agreed method. Fourth ventriculo-peritoneal shunting is the most commonly utilized procedure for the management of this syndrome. Complications from shunting are common and are usually related to malfunction, infection, dislocation and overdrainage. We present two unusual cases in which both patients developed bilateral abducens and facial nerve palsies following shunting of an isolated fourth ventricle. Magnetic resonance imaging (MRI) in both cases revealed collapse of the fourth ventricles with downward displacement of the brain stem. In the first case the trans-tentorial pressure difference was equilibrated with the aid of a "Y" connector between the supratentorial and infratentorial shunts, with full recovery of the neurological deficits; in the second case this approach failed and following a complicated neurosurgical course successful endoscopic aqueductal stenting was performed. DISCUSSION: Pathogenesis of cranial nerve palsies following fourth ventricle shunting and the rationale of treatment are discussed and the literature is reviewed.

Abducens Nerve Diseases↗

PubMed2005

Orthopedic surgery after selective dorsal rhizotomy for spastic diplegia in relation to ambulatory status and age.

OBJECT: 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. METHODS: 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). CONCLUSIONS: 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.

Activities of Daily Living↗

PubMed2005

Endoscopic third ventriculostomy: an outcome analysis of primary cases and procedures performed after ventriculoperitoneal shunt malfunction.

OBJECT: 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. METHODS: 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. CONCLUSIONS: 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.

Adolescent↗

PubMed2004

Effect of selective dorsal rhizotomy on need for orthopedic surgery for spastic quadriplegic cerebral palsy: long-term outcome analysis in relation to age.

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.

Adolescent↗

PubMedDate not supplied

Distance of the motor cortex from the coronal suture as a function of age.

OBJECTIVE: This study was performed to investigate whether the distance from the motor cortex to the coronal suture changes with age. METHODS: We performed a retrospective review of the charts of pediatric patients who had undergone frontoparietal implantation of subdural grids for long-term video-EEG monitoring during an evaluation for seizure surgery from 1995 to 2001. Cortical mapping was performed in 17 patients to localize the motor cortex. On postimplantation lateral skull radiographs, the coronal suture was identified at its medial aspect, and the anterior-posterior distance was measured to the electrodes overlying motor cortex. RESULTS: Ages ranged from 10 months to 14.6 years with a mean of 8.6 years. The mean distance from the coronal suture to the motor cortex was 31.5 mm (+/-7 mm). The location of the motor cortex correlated significantly with age (r(s) = 0.84, p < 0.0005) and increased at a rate of 1.5 mm per year. All patients aged < 6 years had distances of < 3 cm from the coronal suture to the motor cortex. CONCLUSIONS: The distance from the coronal suture to the motor cortex increases with patient age. This variability has implications for determining the location of eloquent structures based on this anatomical landmark.

Adolescent↗