Craniocervical instability in children with Down’s syndrome

Introduction. Pathology of the craniovertebral zone in children with Down’s syndrome is a very important topic, because of the high risk for developing neurological complications in these patients, after even a minor trauma.Material and methods. We performed a review of the literature highlighting the disorders of the cervical spine in children with Down’s syndrome.Results. We gathered data on the etiology, pathogenesis, and clinical presentation of craniocervical instability in children with Down’s syndrome. We reviewed the existing surgical treatment options, and presented our own clinical cases. We also developed a protocol for the management of these patients.Discussions. Understanding the several forms of craniocervical instability in children with Down’s syndrome is very important. As it is a very dangerous condition that can lead to devastating neurological deficits, all medical specialties working with these patients should be aware of them. There are clinical and radiological criteria for this condition that can help in the management of such patients. Surgical treatment is an effective option, but it has a high complication rate and rarely results in neurological improvement.

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Endovascular and Surgical Treatment of Internal Carotid Bifurcation Aneurysms

Neurosurgery ◽

10.1227/neu.0000000000000672 ◽

2015 ◽

Vol 76 (5) ◽

pp. 540-551 ◽

Cited By ~ 28

Author(s):

Juergen Konczalla ◽

Johannes Platz ◽

Nina Brawanski ◽

Erdem Güresir ◽

Stephanie Lescher ◽

...

Keyword(s):

Surgical Treatment ◽

Internal Carotid ◽

Treatment Options ◽

Carotid Bifurcation ◽

Neurological Deficits ◽

Bleeding Complications ◽

Treatment Rate ◽

Surgical Clipping ◽

Aneurysm Neck

Abstract BACKGROUND: Aneurysms of the internal carotid artery (ICA) bifurcation are rare, and no studies have compared patient outcomes after endovascular vs surgical treatment. OBJECTIVE: To report the safety, efficacy, and follow-up outcome of these 2 treatment options for patients with ICA bifurcation aneurysms. METHODS: Patient and aneurysm characteristics, treatment results, and follow-up outcomes (at 30 months) were analyzed from patient records and review of imaging findings. RESULTS: A total of 58 patients with ICA bifurcation aneurysms were treated. By interdisciplinary consensus, 30 aneurysms were assigned for coiling and 28 for clipping. Patients who underwent surgical clipping were younger and had larger aneurysms. More patients were assigned to coiling if their aneurysms originated only from the ICA bifurcation or projected superiorly. For the combined angiographic endpoint, complete and nearly complete occlusion (Raymond-Roy I + II), similar rates of 96% (coiling) or 100% (clipping) could be achieved. Raymond-Roy I occlusion occurred more often after clipping (79% vs 41% coiling). Follow-up of the endovascular group showed minor recanalization of the aneurysm neck (Raymond-Roy II) in 42%. One patient (4%) showed a major recanalization (Raymond-Roy III) and needed re-treatment. For incidental findings, no bleeding complications or new persistent neurological deficits occurred during follow-up. CONCLUSION: Treatment of ICA bifurcation aneurysms after interdisciplinary assignment to clipping or coiling is effective and safe. Despite significantly more minor recanalizations after coiling, the re-treatment rate was very low, and no bleeding was observed during follow-up. Multivariate analysis revealed that origin only from the ICA bifurcation was an independent predictor of aneurysm recanalization after endovascular treatment.

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Surgical treatment of symptomatic atlantoaxial subluxation in Down's syndrome - A case report.

Orthopedics & Traumatology ◽

10.5035/nishiseisai.37.544 ◽

1988 ◽

Vol 37 (2) ◽

pp. 544-546

Author(s):

Yukari Nishi ◽

Takashi Sakou ◽

Yoshiyuki Morizono ◽

Tatsuya Umezu ◽

Hidefumi Kawaida ◽

...

Keyword(s):

Case Report ◽

Surgical Treatment ◽

Down's Syndrome ◽

Down’S Syndrome ◽

Atlantoaxial Subluxation

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Cognitive deficits and associated neurological complications in individuals with Down's syndrome

The Lancet Neurology ◽

10.1016/s1474-4422(10)70112-5 ◽

2010 ◽

Vol 9 (6) ◽

pp. 623-633 ◽

Cited By ~ 216

Author(s):

Ira T Lott ◽

Mara Dierssen

Keyword(s):

Cognitive Deficits ◽

Down's Syndrome ◽

Down’S Syndrome ◽

Neurological Complications

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Graves' disease in children and adolescents. Late results of surgical treatment

Acta Endocrinologica ◽

10.1530/eje.0.1340710 ◽

1996 ◽

Vol 134 (6) ◽

pp. 710-715 ◽

Cited By ~ 33

Author(s):

Claes Rudberg ◽

Henry Johansson ◽

Göran Åkerström ◽

Torsten Tuvemo ◽

F Anders Karlsson

Keyword(s):

Surgical Treatment ◽

Medical Treatment ◽

Children And Adolescents ◽

Down's Syndrome ◽

Antithyroid Drug ◽

Down’S Syndrome ◽

Late Results ◽

Graves Disease

Rudberg C, Johansson H, ÅÅ G, Tuvemo T, Karlsson FA. Graves' disease in children and adolescents. Late results of surgical treatment. Eur J Endocrinol 1996;134:710–5. ISSN0804–4643 All children and adolescents with Graves' disease in the county of Uppsala (catchment area population 250000) treated between 1970 and 1994 were evaluated in a retrospective study. The material comprised 31 patients with a mean age of 11 years (range 4–16), 29 (94%) of whom were girls, and four (13%) of the patients had Down's syndrome. Treatment was primarily conservative and surgery was considered if prolonged medical treatment failed. Lasting remission after antithyroid drug therapy (median 6.5 years; range 4.5–8 years) was noted in 6/31 patients (19%), three (10%) of whom subsequently developed hypothyroidism. Twenty-four of the remaining patients (77%) ultimately underwent subtotal (N=20) or total thyroidectomy (N=4) after experiencing one or more episodes of recurrent hyperthyroidism during medical treatment (median 6 years; range 0.5–11 years). After surgery one patient developed permanent hypocalcemia requiring low-dose vitamin D supplementation. During a postoperative follow-up period of 12.2 years (median: range 1–17 years), there were two cases of recurrent thyrotoxicosis, 1 and 10 years after surgery. The results underline that gender and Down's syndrome are risk factors of juvenile Graves' disease and that the disorder often is difficult to control by long-term medical therapy. In such cases thyroid surgery offers a safe and prompt reversal of the thyrotoxicosis. A proportion of the patients may ultimately develop hypothyroidism, substantiating a need for long-term follow-up of persons afflicted with Graves' disease early in life. F Anders Karlsson, Department of Medicine, University Hospital, S-751 85 Uppsala. Sweden

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Craniocervical Stabilization Using Luque/Hartshill Rectangles

Neurosurgery ◽

10.1227/00006123-199001000-00004 ◽

1990 ◽

Vol 26 (1) ◽

pp. 32-36 ◽

Cited By ~ 34

Author(s):

Alasdair I. MacKenzie ◽

David Uttley ◽

Henry T. Marsh ◽

B. A. Bell

Keyword(s):

Early Mobilization ◽

Neurological Complications ◽

Cord Compression ◽

Atlantoaxial Instability ◽

Posterior Fixation ◽

High Morbidity ◽

Neurological Improvement ◽

Stable Part ◽

Craniocervical Instability ◽

Craniocervical Fusion

Abstract Unteated craniocervical instability is associated with a high morbidity and a significant mortality. Existing methods using bone grafts, interlaminar wires, or acrylic eventually produce stability but require prolonged periods of immobility and have a high failure rate. The ideal method of fixation should provide for permanent correction of deformity and relief of symptoms, with immediate stabilization, at a single procedure. Posterior fixation of the occiput to a stable part of the cervical spine with a molded metal rectangle held in place by interlaminar wires was used to accomplish this. We report 20 patients treated consecutively who have undergone craniocervical fusion by this method using Luque/Hartshill rectangles. Fourteen patients had preexisting atlantoaxial instability and 6 had cord compression, but would become unstable after decompression. All operations were performed under general anesthesia; 9 patients (40%) were awake for intubation/positioning, and 7 patients had a simultaneous decompression. Sixteen patients made an uncomplicated recovery and became mobile 3 days postoperatively. Symptomatic and neurological improvement occurred in 70% of all patients. Neurological complications occurred in 4 patients (20%), reflecting the serious nature of the condition; 2 patients (10%) showed no change. Scrutiny of their presentations and operations failed to identify avoidable risk factors, except faulty wiring techniques. In all patients, permanent stabilization was achieved immediately, facilitating early mobilization with a real chance of improvement, which indicates that the method merits wider application. (Neurosurgery 26:32-36, 1990)

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Neurological Complications after Surgical Treatment of Metastatic Tumours of the Spine

Ortopedia Traumatologia Rehabilitacja ◽

10.5604/15093492.1240792 ◽

2017 ◽

Vol 19 (3) ◽

pp. 227-237 ◽

Cited By ~ 3

Author(s):

Piotr Biega ◽

Grzegorz Guzik ◽

Tomasz Pitera

Keyword(s):

Spinal Cord ◽

Surgical Treatment ◽

Postoperative Period ◽

Neurological Complications ◽

Neurological Function ◽

Prognostic Scores ◽

Neurological Deficits ◽

Neoplastic Disease ◽

Spinal Cord Damage ◽

Function Impairment

Background. Postoperative impairment of neurological function is a rare but serious complication of surgical treatment of metastatic tumours located in the spine. This paper presents an analysis of the causes, symptoms and methods of treatment of spinal cord function impairment in the postoperative period. Materials and methods. The study retrospectively analysed the treatment of 525 patients diagnosed with compression of neural structures and neurological deficits in the course of metastatic disease who were operated on in 2012-2015. The baseline degree of spinal cord damage was assessed with the Frankel scale. Surgical treatment methods were selected based on the results obtained with the Tomita system as well as the Tokuhashi and Bauer scores. Results. A total of 8 (1.5%) cases of impaired neurological function were identified in the postoperative period. The spinal cord damage had no discernible cause in 6 cases. Symptoms of neurological function impairment occurred with a delay in 6 patients. In 2 cases, the symptoms were observed immediately after the end of the surgery. One patient demonstrated improved neurological status after revision surgery. Conclusions. 1. Surgical decompression of the spinal cord in the course of neoplastic disease is rarely complicated by neurological function impairment. 2. Neurological complications occurred mainly after treatment of tumours located in the thoracic section of the spine. 3. Prognostic scores used for qualifying patients for appropriate surgical treatment focus mainly on patient survival and do not account for potential complications.

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