Basilar impression and osteogenesis imperfecta: a 21-year retrospective review of outcomes in 20 patients

2007 ◽  
Vol 7 (6) ◽  
pp. 594-600 ◽  
Author(s):  
Ahmed G. Ibrahim ◽  
H. Alan Crockard
Keyword(s):  
Osteogenesis Imperfecta ◽  
Retrospective Review ◽  
Senior Author ◽  
Karnofsky Performance Scale ◽  
Decompression Surgery ◽  
Basilar Impression ◽  
Occipitocervical Fixation ◽  
Dorsal Stabilization ◽  
Patient Series

Object Basilar impression (BI) secondary to osteogenesis imperfecta (OI) is a rare but debilitating condition that is often progressive unless it is halted. More recently, ventral decompression surgery has been advocated for this condition. This study is a retrospective review of the 21-year experience of ventral decompression surgery and dorsal occipitocervical fixation in patients with BI secondary to OI and is the largest patient series reported to date. Methods Twenty patients treated between 1982 and 2003 by the senior author at the authors' institution were included in this study. All patients underwent ventral decompression surgery followed by dorsal craniocervical stabilization. Patients were followed up for a median of 10 years. Results There were no intraoperative or perioperative deaths. Postoperatively, 16 of 20 (80%) patients showed objective improvement or maintained their good preoperative level of function. After surgery, of the 15 patients admitted with Karnofsky Performance Scale (KPS) scores of 70% or less, 11 improved, two remained unchanged, one patient's condition deteriorated, and one patient died of an unrelated cause. Of five patients admitted with a KPS score of 80% or greater, no patient's condition deteriorated in the short- and midterm period, but one patient had recurrence 15 years after surgery. At the end of follow-up, 25% of the patients had recurrence of brainstem compression symptoms or had died, and 15% showed no improvement after surgery. All of the remaining patients (60%) had sustained a long-term benefit from surgery. Conclusions Aggressive ventral decompression surgery and dorsal stabilization for patients with BI secondary to OI can not only halt disease progression but can also produce a good and sustainable long-term functional outcome, even in those patients who present as severely symptomatic. Patients who presented early with minor symptoms had good long-term outcomes.

Occipitocervical Fixation

Neurosurgery ◽  
2016 ◽  
Vol 79 (4) ◽  
pp. 549-560 ◽  
Author(s):  
Eduardo Martinez-del-Campo ◽  
Jay D. Turner ◽  
Samuel Kalb ◽  
Leonardo Rangel-Castilla ◽  
Luis Perez-Orribo ◽  
...  
Keyword(s):  
Case Series ◽  
Median Number ◽  
Senior Author ◽  
Neurological Deficits ◽  
Neurological Injury ◽  
Long Term Results ◽  
Occipitocervical Fixation ◽  
Surgical Fixation

Abstract BACKGROUND Occipitocervical junction instability can lead to serious neurological injury or death. Open surgical fixation is often necessary to provide definitive stabilization. However, long-term results are limited to small case series. OBJECTIVE To review the causes of occipitocervical instability, discuss the indications for surgical intervention, and evaluate long-term surgical outcomes after occipitocervical fixation. METHODS The charts of all patients undergoing posterior surgical fixation of the occipitocervical junction by the senior author were retrospectively reviewed. A total of 120 consecutive patients were identified for analysis. Patient demographic characteristics, occipitocervical junction pathology, surgical indications, and clinical and radiographic outcomes are reported. RESULTS The study population consisted of 64 male and 56 female patients with a mean age of 39.9 years (range, 7 months to 88 years). Trauma was the most common cause of instability, occurring in 56 patients (47%). Ninety patients (75%) were treated with screw/rod constructs; wiring was used in 30 patients (25%). The median number of fixated segments was 5 (O-C4). Structural bone grafts were implanted in all patients (100%). Preoperative neurological deficits were present in 83 patients (69%); 91% of those patients improved with surgery. Mean follow-up was 35.1 ± 27.4 months (range, 0-123 months). Two patients died, and 10 were lost to follow-up before the end of the 6-month follow-up period. Fusion was confirmed in 107 patients (89.1%). The overall complication rate was 10%, including 3 patients with vertebral artery injuries and 2 patients who required revision surgery. CONCLUSION Occipitocervical fixation is a durable treatment option with acceptable morbidity for patients with occipitocervical instability.

EFFECTS OF LONG-TERM ALENDRONATE TREATMENT ON A LARGE SAMPLE OF PEDIATRIC PATIENTS WITH OSTEOGENESIS IMPERFECTA

2016 ◽  
Vol 22 (12) ◽  
pp. 1369-1376 ◽  
Author(s):  
Fang Lv ◽  
Yi Liu ◽  
Xiaojie Xu ◽  
Jianyi Wang ◽  
Doudou Ma ◽  
...  
Keyword(s):  
Osteogenesis Imperfecta ◽  
Pediatric Patients ◽  
Large Sample ◽  
Alendronate Treatment

The operative management of basilar impression in osteogenesis imperfecta

Neurosurgery ◽  
1990 ◽  
pp. 782 ◽  
Author(s):  
H L Harkey ◽  
H A Crockard ◽  
J M Stevens ◽  
R Smith ◽  
A O Ransford
Keyword(s):  
Osteogenesis Imperfecta ◽  
Operative Management ◽  
Basilar Impression

Low Creatininuria due to Hyponatremia Is Reversible in Many Patients

Nephron ◽  
2021 ◽  
pp. 1-5
Author(s):  
Guy Decaux
Keyword(s):  
Retrospective Review ◽  
Sodium Excretion ◽  
Biochemical Data ◽  
Creatinine Excretion ◽  
Urine Creatinine ◽  
Chronic Hyponatremia ◽  
Before And After ◽  
Solute Excretion

<b><i>Background:</i></b> Chronic hyponatremia has been reported to be associated with low solute intake and low creatinine excretion (reflecting likely sarcopenia). We wanted to study the effect, on the long term, of correction of hyponatremia on solute and creatinine excretion in chronic SIADH. <b><i>Methods:</i></b> We made a retrospective review of clinical and biochemical data of patients with euvolemic hyponatremia. We analyzed 24-h urine solute and creatinine excretion in volunteers with hyponatremia induced by dDAVP over 4 days, in 12 patients with chronic SIADH (&#x3e;1 month) before and after a few days of SNa correction and in 12 patients (6 women and 6 men) before and after 3 months of SNa correction by a vaptan or urea. <b><i>Results:</i></b> We confirm a low urine creatinine and solute excretion only in patients with chronic hyponatremia (&#x3e;1 month). Correction of SNa (from 127 ± 2.3 mEq/L to 139 ± 2.8 mEq/L) for &#x3e;3 months, in the 12 patients (mean age 58 ± 18), was associated with an increase in 24-h creatinine excretion (from 986 ± 239 to 1,238 ± 220 mg; <i>p</i> &#x3c; 0.02) and in patients treated with a vaptan (<i>n</i> = 5) solute excretion increased from 656 ± 207 mmol/24 h to 960 ± 193 mmol/24 h (<i>p</i> &#x3c; 0.02). Sodium excretion increased also in the 12 patients (from 100 ± 53 mEq/24 h to 169 ± 38 mEq/24 h; <i>p</i> &#x3c; 0.01). <b><i>Conclusion:</i></b> Chronic hyponatremia (&#x3e;1 month) is associated with a decrease in solute output (or intake) and in creatinine excretion. In many patients, these abnormalities are reversible in the long term.

Long-term Effects of Neridronate in Adults with Osteogenesis Imperfecta: An Observational Three-Year Italian Study

2017 ◽  
Vol 100 (4) ◽  
pp. 341-347 ◽  
Author(s):  
O. Viapiana ◽  
L. Idolazzi ◽  
A. Fassio ◽  
G. Orsolini ◽  
M. Rossini ◽  
...  
Keyword(s):  
Osteogenesis Imperfecta ◽  
Long Term Effects ◽  
Italian Study
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