Lhermitte-Duclos Disease (Dysplastic Gangliocytoma of the Cerebellum) and Cowden Syndrome: Clinical Experience From a Single Institution with Long-Term Follow-Up

Abstract Thyroid acropachy is an extreme manifestation of autoimmune thyroid disease. It presents with digital clubbing, swelling of digits and toes, and periosteal reaction of extremity bones. It is almost always associated with ophthalmopathy and thyroid dermopathy. During a 26-yr period at our institution, of 178 patients with thyroid dermopathy, 40 had acropachy. Clubbing associated with thyroid dermopathy (pretibial myxedema) was seen in 35 patients. Clubbing usually was not a patient complaint and was noted only by clinical observers. Four of eight patients with hand and extremity radiographs had periosteal reaction. Seven had associated extremity and joint pain; this pain was absent at long-term follow-up. Half of the patients required systemic corticosteroid therapy, 53% required transantral or transfrontal orbital decompression for severe ophthalmopathy, and 18% had the elephantiasic form of dermopathy. Cigarette-smoking rates were 81% for women and 75% for men (mean, 28 pack-years). All 13 patients who had thyroid-stimulating Ig measurement had high titers. Long-term follow-up (median, 12.5 yr) revealed that acropachy was not a complaint in follow-up visits or questionnaires. The data suggest that thyroid acropachy is an indicator of severity of ophthalmopathy and dermopathy. It is a source of clinical concern only if dermopathy is persistent and severe.

Download Full-text

Long-Term Outcomes of Scaphoid Malunion

Hand ◽

10.1177/1558944716643295 ◽

2016 ◽

Vol 12 (1) ◽

pp. 26-30 ◽

Cited By ~ 8

Author(s):

Blake P. Gillette ◽

Peter C. Amadio ◽

Sanjeev Kakar

Keyword(s):

Nonoperative Management ◽

Corrective Osteotomy ◽

Nonoperative Treatment ◽

Long Term Outcomes ◽

Single Institution ◽

Surgical Evaluation ◽

Long Term Follow Up ◽

The Mean

Background: The optimal treatment of patients with a scaphoid malunion remains controversial. The long-term outcomes of operative and nonoperative management have not been established. Methods: We conducted a retrospective review of the outcomes of all scaphoid malunions treated at single institution over a 30-year period. This included patients who underwent corrective osteotomy, salvage procedures (ie, dorsal cheilectomy, radial styloidectomy, and scaphoidectomy with midcarpal fusion), and those who refused operative intervention. The Mayo Wrist Score was determined at the time of surgical evaluation. Patient-Rated Wrist Evaluation (PRWE) and Disabilities of the Arm, Shoulder and Hand (QuickDASH) surveys were sent to all patients for long-term follow-up. Results: Seventeen patients had follow-up at a mean 21.4 years (range, 12-30 years). The mean initial lateral intrascaphoid angle was 58°. Of the 17 patients, 11 proceeded with surgery and 6 opted for nonoperative management. A corrective osteotomy was performed in 4 patients. Of the remaining 7 surgical patients, 5 patients underwent procedures such as cheilectomy and radial styloidectomy, whereas 2 patients had a scaphoidectomy with midcarpal fusion. The final mean PRWE and QuickDASH scores for corrective osteotomy, salvage procedures, and nonoperative treatment were 23 and 6, 18 and 10, and 33 and 22, respectively. Conclusion: Long-term outcomes were similar between operative and nonoperative management.

Download Full-text