Endoscopic transnasal decompression for management of basilar invagination in osteogenesis imperfecta

2008 ◽  
Vol 9 (4) ◽  
pp. 354-357 ◽  
Author(s):  
Mitchell A. Hansen ◽  
Melville J. da Cruz ◽  
Brian K. Owler
Keyword(s):  
Osteogenesis Imperfecta ◽  
Collagen Synthesis ◽  
Bone Development ◽  
Basilar Invagination ◽  
Technical Note ◽  
Occipitocervical Fusion ◽  
Transnasal Approach ◽  
Endoscopic Transnasal Approach

Osteogenesis imperfecta (OI) is a disorder of bone development caused by a genetic dysfunction of collagen synthesis. Basilar invagination (BI) is an uncommon but serious complication of OI. Brainstem decompression in OI is undertaken in certain circumstances. Transoral-transpalatopharyngeal ventral decompression with posterior occipitocervical fusion has become the treatment of choice when required. This technical note outlines a novel endoscopic transnasal approach for ventral decompression. The literature is reviewed and a strategy for the management of BI in patients with OI is outlined.

Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management

1997 ◽  
Vol 86 (6) ◽  
pp. 950-960 ◽  
Author(s):  
Paul D. Sawin ◽  
Arnold H. Menezes
Keyword(s):  
Osteogenesis Imperfecta ◽  
Posterior Fossa ◽  
Basilar Invagination ◽  
Posterior Fossa Decompression ◽  
Occipitocervical Fusion ◽  
Female Ratio ◽  
Content Type ◽  
Brainstem Compression ◽  
The Mean ◽  
Fine Print

✓ Osteogenesis imperfecta (OI) is a heritable disorder of bone development caused by defective collagen synthesis. Basilar invagination is an uncommon but devastating complication of this disease. The authors present a comprehensive strategy for management of craniovertebral anomalies associated with OI and related osteochondrodysplasias. Twenty-five patients with congenital osteochondrodysplasias (18 OI, four Hajdu—Cheney syndrome, and three spondyloepiphyseal dysplasia) and basilar invagination were evaluated between 1985 and 1995. The male/female ratio in this cohort was 1:1. The mean age at presentation was 11.9 years (range 13 months–20 years). Fourteen patients (56%) presented during adolescence (11–15 years of age). Symptoms and signs included headache (76%), lower cranial nerve dysfunction (68%), hyperreflexia (56%), quadriparesis (48%), ataxia (32%), nystagmus (28%), and scoliosis (20%). Four patients (16%) were asymptomatic. Seven (28%) had undergone previous posterior fossa decompression; one had also undergone ventral decompression. Imaging findings included basilar invagination (100%), ventral brainstem compression (84%), hydrocephalus (32%), hindbrain herniation (28%), and syringomyelia/syringobulbia (16%). Patients with hydrocephalus underwent ventricular shunt placement. Reducible basilar invagination (40%) was treated with posterior fossa decompression and occipitocervical fusion. Those with irreducible ventral compression (60%) underwent transoral—transpalatopharyngeal decompression followed by occipitocervical fusion. All patients improved initially. However, basilar invagination progressed radiographically in 80% (symptomatic in 24%) despite successful fusion. Prolonged external orthotic immobilization with the modified Minerva brace afforded symptomatic improvement and arrested progression of the deformity. The mean follow-up period was 5.9 years (range 1.1–10.5 years). Ventral brainstem compression in OI should be treated with ventral decompression, followed by occipitocervical fusion with contoured loop instrumentation to prevent further squamooccipital infolding. Despite fusion, however, basilar invagination tends to progress. Prolonged immobilization (particularly during adolescence) may stabilize symptoms and halt further invagination. This study represents the largest series to date addressing craniovertebral anomalies in OI and related congenital bone softening disorders.

The Expanded Endonasal Approach: A Fully Endoscopic Transnasal Approach and Resection of the Odontoid Process

Skull Base ◽  
2005 ◽  
Vol 15 (S 2) ◽  
Author(s):  
Carl Snyderman ◽  
Amin Kassam ◽  
Paul Gardner ◽  
Ricardo Carrau ◽  
Richard Spiro
Keyword(s):  
Odontoid Process ◽  
Endonasal Approach ◽  
Transnasal Approach ◽  
Expanded Endonasal Approach ◽  
Endoscopic Transnasal Approach

The Expanded Endoscopic Transnasal Approach for the Treatment of Clival Chordomas

Skull Base ◽  
2007 ◽  
Vol 17 (S 1) ◽  
Author(s):  
Amir Dehdashti ◽  
Fred Gentili ◽  
Ian Witterick ◽  
Ahmed Ganna
Keyword(s):  
Transnasal Approach ◽  
Endoscopic Transnasal Approach

Endoscopic Transnasal Approach to the Parapharyngeal Space

Skull Base ◽  
2009 ◽  
Vol 19 (S 02) ◽  
Author(s):  
D.H. Wang ◽  
H.P. Yu ◽  
X.C. Sun ◽  
L. Hu
Keyword(s):  
Parapharyngeal Space ◽  
Transnasal Approach ◽  
Endoscopic Transnasal Approach

Ossifying fibromyxoid tumour of the sphenoid sinus

2009 ◽  
Vol 124 (4) ◽  
pp. 437-440
Author(s):  
S D Shetty ◽  
R J Salib ◽  
S B Nair ◽  
N Mathad ◽  
J Theaker
Keyword(s):  
Skull Base ◽  
Sphenoid Sinus ◽  
Neoplastic Disease ◽  
Clinical Effects ◽  
Soft Tissue Neoplasm ◽  
Transnasal Approach ◽  
Fibrous Stroma ◽  
Neuroectodermal Origin ◽  
Endoscopic Transnasal Approach

AbstractIntroduction:Ossifying fibromyxoid tumour is a recently described, rare but morphologically distinctive soft tissue neoplasm characterised by a combination of myxoid and/or fibrous stroma with areas of ossification. Although most authors postulate a neuroectodermal origin for this peculiar tumour, there is no agreement in the literature regarding its histopathogenesis. To our knowledge, this is the first reported case of ossifying fibromyxoid tumour involving the sphenoid sinus.Histological findings:Tumour of low cell density, composed of small, spindle-shaped or stellate cells with small, irregular nuclei set in a fibromyxoid stroma.Management:Following discussion at the skull base multidisciplinary team meeting, a combined surgical team including an otorhinolaryngologist and a neurosurgeon carried out resection of the lesion, using an endoscopic transnasal approach, followed by reconstruction of the defect.Conclusions:An awareness of the distinctive histopathological features of ossifying fibromyxoid tumour, and of its clinical effects, is crucial to establishing a definitive diagnosis and thereby instituting appropriate management. This case report also reinforces the evolving role of the endoscopic transnasal approach in the management of inflammatory and neoplastic disease involving the skull base. This is increasingly being made possible by close collaboration between multiple surgical specialties, including otorhinolaryngology and neurosurgery.

Aggressive verrucous carcinoma of the paranasal sinuses invading the orbit: case report and literature review

2009 ◽  
Vol 123 (11) ◽  
Author(s):  
J Yiotakis ◽  
T Vamvakidis ◽  
E Iakovou ◽  
L Manolopoulos
Keyword(s):  
Case Report ◽  
Paranasal Sinuses ◽  
Verrucous Carcinoma ◽  
Transnasal Approach ◽  
Early Management ◽  
Combined Radiochemotherapy ◽  
Endoscopic Transnasal Approach ◽  
Well Differentiated ◽  
Orbital Invasion ◽  
Histological Result

AbstractIntroduction:Verrucous carcinoma is a variant of well differentiated squamous cell carcinoma characterised by an exophytic, warty growth which is slowly but locally invasive and which can cause extensive local destruction if left untreated.Case report:We report the first known case of veruccous carcinoma of the paranasal sinuses with rapid orbital invasion. A 58-year-old man presented with right-sided nasal obstruction, a huge right intranasal lesion, intractable dacryocystitis and cutaneous involvement of the nasolacrimal sac region. The tumour was partially removed using an endoscopic transnasal approach. Post-operatively, and while waiting for the histological result, the patient developed signs of rapid invasion of the orbit, with uniocular visual acuity deficit. He underwent combined radiochemotherapy, with a moderate response.Conclusion:Knowledge of the potentially aggressive nature of verrucous carcinoma may be helpful in early management of intraorbital invasion and salvaging of the eye.

scholarly journals The influence of a high-protein, low-carbohydrate diet on bone development in the fetuses of rat dams with streptozotocin-induced diabetes

1988 ◽  
Vol 59 (1) ◽  
pp. 57-62 ◽  
Author(s):  
W. Keith Harvey ◽  
Tetsuo Nakamoto
Keyword(s):  
G Protein ◽  
Collagen Synthesis ◽  
Bone Development ◽  
High Protein ◽  
Diabetic Group ◽  
Long Bones ◽  
Carbohydrate Diet ◽  
Low Carbohydrate Diet ◽  
Low Carbohydrate ◽  
Ca Uptake

1. The purpose of the present study was to determine the effects of diet on the mandibles and growth centres of the long bones in the fetuses of diabetic rat dams given a normal diet compared with those given a high-protein, low-carbohydrate diet.2. On the 9th day of gestation, the controls, groups 1 and 3, were injected with citrate buffer and given 200 and 600 g protein/kg diets respectively. Groups 2 and 4 were injected with 40 mg streptozotocin/kg body-weight and pair-fed with groups I and 3 respectively on the 200 and 600 g protein/kg diets.3. On day 22, some dams were injected with either 45Ca or [14C]proline. Mandibles and long bones were removed and weighed and analysed for Ca content, 45Ca uptake, collagen and collagen synthesis.4. The body-weights, and mandibular and long-bone weights of the fetuses in the diabetic 200 g protein/kg group were smaller than those of the non-diabetic 200 g protein/kg group, whereas those of the diabetic 600 g protein/kg group showed no difference from the non-diabetic 600 g protein/kg group.5. The rate of collagen synthesis was higher in the fetuses of the diabetic 600 g protein/kg group than those of the non-diabetic group. Bones of the diabetic 200 g protein/kg group were lower in collagen content when compared with the non-diabetic group, whereas there was no difference between the diabetic and non-diabetic 600 g protein/kg groups.6. Ca uptake and total Ca contents in the mandibles and long bones showed no difference between diabetic and non-diabetic groups fed on both diets.7. A high-protein, low-carbohydrate diet appeared to have a certain beneficial effect on bone development of the growing fetuses from diabetic dams.

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