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Biomedical subjects

A Hanieh

Publications and source records attributed to A Hanieh.

At least 19 recordsLinked to original sources
PubMed2006

Childhood glioblastoma multiforme of the spinal cord.

Astrocytoma accounts for more than 50% of all central nervous system tumours diagnosed, with particular prevalence in the 15- to 34-year-old age bracket, rarely arising in younger children. In 1995, a 7-year-old boy presented in Emergency with a 3-day history of severe radicular back pain and associated muscle spasms, exacerbated by lying on his back. Both bone scan and plain X-rays were unremarkable; however, MRI showed a 3-cm space-occupying lesion at the level of T5-T6. The patient proceeded to biopsy and partial excision of the tumour through laminectomy, histology confirming an anaplastic astrocytoma (glioblastoma multiforme), St Anne Mayo grade 4. Treatment consisted of a radical course of radiotherapy alone, delivering a total dose of 44.8 Gy at 1.6 Gy per fraction. The treatment comprised of three phases using two oblique wedged fields on a 6 MV linear accelerator. The patient remains disease free 7 years post treatment, with the only effect noted being a slight kyphoscoliosis at the site of the laminectomy and radiation. This report highlights the efficacy of combined surgery and radiation therapy in the management of spinal cord glioblastoma multiforme in preventing tumour recurrence, with acceptable morbidity. Further evaluation of the treatment efficacy would be difficult because of the scarcity of such cases.

Child↗
PubMed2001

Multiple cystic brain lesions in a patient with pilocytic astrocytoma.

Pilocytic astrocytomas are usually present as solitary posterior cranial fossa tumours. An unusual case of pilocytic astrocytoma in a 3 year and 8 month old boy is presented. The patient presented over the course of 10 months with intermittent headaches, vomiting, gait ataxia and drowsiness. After extensive investigations magnetic resonance imaging (MRI) revealed widespread lesions throughout the central nervous system, including multiple cystic cerebral grey matter lesions. A brain biopsy was performed and pathological studies revealed pilocytic astrocytoma. The literature pertaining to neuraxis dissemination of pilocytic astrocytomas in the paediatric population is reviewed. There are only a very limited number of reports of pilocytic astrocytoma ca using multiple brain lesions, with no publications of multiple cystic brain lesions. We believe this to be a unique case of pilocytic astrocytoma presenting with widespread cystic lesions throughout the brain.

Astrocytoma↗
PubMed2000

Syringomyelia in children with primary scoliosis.

The clinical notes of 35 children presenting with scoliosis were reviewed; all 35 had been investigated with MRI. Seven were found to have syringomyelia, and six of these had Chiari malformation. Correction of the syrinx resulted in improvement or stabilisation of the spinal curvature. We recommend that all cases presenting with primary scoliosis should have MRI and should be treated if a syrinx is found.

Arnold-Chiari Malformation↗
PubMed2000

CT-determined intracranial volume for a normal population.

Intracranial volume comparisons of patients with craniosynostosis and normal have been contrary to expectations, leading to questioning of the validity of the current normal reference material. Computed tomography-determined intracranial volume is presented for a white normal population. Specifically, intracranial volumes for 157 subjects (82 female and 75 male) were measured from computed tomography data using the Cavalieri estimator: volume determination was based on measuring the area in each computed tomography section. Monomolecular and Gompertz models were applied to find curves of best fit to the intracranial volume as a function of the age. The best fit was obtained using the monomolecular model when the response variable was the logarithmically transformed intracranial volume, and the independent variable was the logarithm of the age from conception. For example, the mean (standard deviation) for male subjects at 1 year and 20 years were 1,125.6 (89.6) ml and 1,472.9 (117.2) ml, respectively, and for female subjects 1,024.9 (84.0) ml and 1,321.7 (108.3) ml, respectively. Although the shape and rate of increase of the female and male curves is similar, the female mean is 1.3 standard deviations below the male mean at 20 years. These curves were compared with the commonly referenced curves of Blinkov (1941), Lichtenberg (1960), and Dekaban (1977). Our male curve is substantially higher than these curves in the age range 8 months to 4 years. Our female curve, however, is approximately 1 standard deviation below Lichtenberg's curve from birth to 7 months. There are then only minor differences between our female curve and Lichtenberg's curve until his curve crosses ours at 41 months, where they significantly diverge from approximately 4.5 years. Our curves indicate that 95% of the final intracranial volume has been attained by 42 months for girls and 46 months for boys.

Adolescent↗
PubMed1998

Growing skull fracture after cranial vault reshaping in infancy.

Growing skull fractures are reported in three infants undergoing cranial vault surgery for craniosynostosis. Although primary dural injury was overt in only one patient, an extensive dural defect with spatially coincident bony absence was identified in all patients at late exploration. The coincidence of dural injury, deficient overlying bone, and a pulsatile growing brain are necessary conditions for the development of growing skull fractures. With the advent of increasingly radical cranial vault procedures during infancy, the potential for this complication to arise may increase. Definitive correction requires autogenous tissue reconstruction of the dural and bony defect.

Child, Preschool↗
PubMed1998

Bilambdoid and posterior sagittal synostosis: the Mercedes Benz syndrome.

A consistent pattern of craniosynostosis in the sagittal and bilateral lambdoid sutures is described in three patients. The external cranial ridging associated with fusion of these sutures produces a characteristic triradiate, or "Mercedes Benz," appearance to the posterior skull. Locally marked growth restriction is evident in the posterior fossa with compensatory secondary expansion of the anterior fossa manifesting a degree of frontal bossing which mimics bicoronal synostosis. Although this appearance could lead to inadvertent surgery in the frontal region, attention to the occipital region with wide early suture excision and vault shaping is indicated.

Craniosynostoses↗
PubMed1998

Computer tomography determined intracranial volume of infants with deformational plagiocephaly: a useful "normal"?

Over the last 10 years, children with deformational (nonsynostotic) plagiocephaly have undergone computed tomography scans using the same protocols as children with lambdoid craniosynostosis because of their similarly distorted head shapes. These children are believed to have normal intracranial volume. Given the recent questioning of what is the normal range of intracranial volume in human populations, the authors have undertaken a comparison of the intracranial volume of children with deformational plagiocephaly and Lichtenberg's normal population (Lichtenberg R. Radiographie du crane de 226 enfants normaux de la naissance a 8 ans: Impressions digitiformes, capacite, angles et indices [thesis]. Paris: University of Paris, 1960). The intracranial volume was determined for 20 females and 46 males with deformational plagiocepahly ranging in age from 2.5 to 20.7 months using computed tomography scan data. Although no significant differences were found for the females, the authors found that the intracranial volume of the males with deformational plagiocephaly were significantly larger than Lichtenberg's population. The authors conclude that this is because Lichtenberg's data do not adequately reflect the normal range of intracranial volume for males ranging in age from 2.5 to 20.7 months, rather than gender differences associated with deformational plagiocephaly. Further, until a more suitable normal becomes available, the deformational (nonsynostotic) plagiocephaly data could be used as substitute normal reference material in the measured age range for assessment of the intracranial volumes of children with craniosynostosis.

Analysis of Variance↗
PubMed1998

Transcranial correction of orbital neurofibromatosis.

Neurofibromatosis is a systemic disease that often produces striking disfigurement. Orbital manifestations are common and include sphenoid dysplasia with or without infiltration of the periorbital soft tissues. The resultant deficiency of the posterolateral orbital wall may lead to protrusion of the temporal lobe into the orbit, displacement of the globe, and pulsatile exophthalmos. Treatment at our unit has consisted of transcranial orbital reconstruction with bone grafts and periorbital soft-tissue correction. Observation of complete bone graft resorption in one patient prompted an assessment of the Australian Craniofacial Unit's experience with particular attention paid to the stability of operative correction. Of 36 patients with head and neck neurofibromatosis treated during the period from 1981 to 1995, 14 patients underwent transcranial correction of orbital deformities secondary to sphenoid dysplasia. The treatment and outcomes of this transcranial group are reviewed. The most notable finding was that of recurrent globe pulsation in four patients following initial resolution. Computed tomography scans have documented partial to complete bone graft resorption in three of these patients. Titanium mesh is now being utilized to provide a more durable reconstruction.

Adolescent↗
PubMed1997

Trochlear nerve schwannomas occurring in patients without neurofibromatosis: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Despite their predilection for sensory nerves, intracranial schwannomas have been reported in a number of mixed and purely motor cranial nerves, especially in association with Type 2 neurofibromatosis. We report the rare occurrence of a trochlear nerve schwannoma in a patient without neurofibromatosis and review 17 other case reports from the literature. CLINICAL PRESENTATION: A 35-year-old woman presented with an 8-week history of evolving left hemiparesis, bilateral bulbar paresis, and out-of-character emotional lability. INTERVENTION: She underwent a left temporal craniotomy and a subtemporal, transtentorial approach to the tentorial hiatus, with complete excision of a cisternal trochlear nerve schwannoma. CONCLUSION: Postoperative complications included temporary oculomotor and abducens nerve palsies and temporary right hemiparesis and mild expressive dysphasia, which were resolved at 23-month follow-up. Preoperative symptoms and signs completely resolved, but a postoperative complete trochlear nerve palsy required inferior oblique myectomy for correction of diplopia. A review of the literature showed no preoperative trochlear nerve involvement in at least 45% of cases. The tumor is isointense on T1- and T2-weighted magnetic resonance images and enhances brightly with gadolinium. The most frequently used approach for surgical excision is the subtemporal approach, and the tumor is almost always totally excised. Long-term follow-up suggests recovery of preoperative deficit, and persisting or new trochlear nerve palsy is the rule.

Adult↗
PubMed1996

Cerebrospinal fluid spaces before and after infant fronto-orbital advancement in unilateral coronal craniosynostosis.

Computed tomographic scan-based examination of the cerebrospinal fluid-filled spaces before and after fronto-orbital advancement in infants with unilateral coronal synostosis was performed. In infants who had not undergone surgery, pronounced compression of the ipsilateral frontal brain substance, frontal subarachnoid space, and frontal horn of the lateral ventricle were evident, with deviation of the interhemisphere fissure. After infant fronto-orbital advancement, frontal dead-space is obliterated early by expansion of the frontal subarachnoid space. Asymmetries interhemispheric fissure are slower to adapt and may be persistent some years later.

Brain↗
PubMed1993

Apert's syndrome.

This paper discusses 33 cases of Apert's syndrome which were treated in the Australian Craniofacial unit at the adelaide Children's Hospital. The main features were discussed. We found that mild ventricular dilatation is common in Apert's syndrome but without associated raised intracranial pressure. Severe ventricular dilatation was seen in only one case. No shunt procedures were performed. We also studied the changes in the ventricular size after transcranial corrective procedures. There was no significant change in the ventricular size, the increase in the skull volume was compensated by expansion of the brain tissue and to some extent by increase in the subarachnoid space. Two cases with unusual features are also described.

Acrocephalosyndactylia↗
PubMed1993

The cloverleaf skull anomaly: managing extreme cranio-orbitofaciostenosis.

The cloverleaf skull anomaly represents the most manifestly extreme form of cranio-orbitofaciostenosis with hydrocephalus that has to date been associated with a uniformly poor outcome and frequent death in infancy. Assessment of the primary deformity and the approach to treatment in a consecutive series of 10 patients with the cloverleaf skull anomaly in the one unit are presented. Early predictable surgical correction of the cranio-stenosis is possible by fronto-orbital advancement and lambdoid craniectomy. Less satisfactory correction of hydrocephalus and orbitostenosis is possible with the attendant increased morbidity. Early survival and the potential for a satisfactory long-term outcome depend on the management of the faciostenosis and an airway that may be anomalous at multiple levels. The initial approach was routine tracheostomy, which provided short-term relief but with the morbidity associated with such airway maintenance in any environment other than the most sophisticated health services. More recently, upper airway surgery (uvulopalatopharyngoplasty, adenoidectomy, and soft palatal split) has ensured airway control and avoided the progression to tracheostomy. When utilized later (1 to 10 years), such surgery may alleviate the symptoms of upper airway obstruction and sleep apnea and permit delay of midfacial advancement.

Child↗
PubMed1992

Gangliogliomas in childhood.

Ganglioglioma is a tumour of the central nervous system composed of an admixture of dysplastic nerve cells resembling pleomorphic ganglion cells, and glial elements, which may be astrocytic and/or oligodendroglial in appearance. A series of 12 patients aged between 9 months and 15 years 9 months, all of whom had suffered epilepsy refractory to medical treatment for up to 8 years, is presented. Computed tomographic and magnetic resonance scans were of prime use in localisation of the tumours. Calcification was noted preoperatively in 4 of 12 cases. The majority of patients obtained at least partial relief from symptoms after complete or partial resection. Histologically, 11 of the tumours included grade 1 astrocytic elements and the remaining one exhibited grade 2 areas. The diagnosis of ganglioglioma should be suspected in a child with refractory, long-standing epilepsy. Prognosis of these tumours is determined by the astrocytic component; if this is of low grade, surgical excision may result in marked symptomatic improvement or cure.

Adolescent↗
PubMed1991

Head injuries in infants and young children: the value of the Paediatric Coma Scale. Review of literature and report on a study.

The normal verbal and motor responses embodied in the standard Glasgow Coma Scale (GCS) are not achievable during the first few years of life. The recent literature contains numerous reports of attempts to devise scales of responses quantitating the conscious level in infants and young children, both for research purposes and as clinical guides; some of these scales incorporate items, e.g. brainstem reflexes, that are not included in the GCS. We have reported on a simple paediatric version of the GCS, which uses the standard scale with minor modifications in the verbal component, and sets realistic age-related normal responses. This has been tested prospectively in a series of 60 head-injured infants and children (age range 0-72 months). Of 6 cases recorded as comatose 6 h after injury, 4 have confirmed or suspected residual disabilities. Of 35 cases considered to be fully conscious at 6 h, 31 have made good recoveries and only 1 has suspected residual disabilities. The study suggests that the scale accords with the realities of neurological immaturity, and confirms that it can be used in routine paediatric practice. For comparative therapeutic trials, the conscious level in infants has limited value as an index of brain injury, and should be complemented by other indices, such as brainstem reflexes.

Brain↗