In Search of the Optimal Surgical Treatment for Velopharyngeal Dysfunction in 22q11.2 Deletion Syndrome: A Systematic Review

<div><h3>Background</h3><p>Patients with the 22q11.2 deletion syndrome (22qDS) and velopharyngeal dysfunction (VPD) tend to have residual VPD following surgery. This systematic review seeks to determine whether a particular surgical procedure results in superior speech outcome or less morbidity.</p> <h3>Methodology/ Principal Findings</h3><p>A combined computerized and hand-search yielded 70 studies, of which 27 were deemed relevant for this review, reporting on a total of 525 patients with 22qDS and VPD undergoing surgery for VPD. All studies were levels 2c or 4 evidence. The methodological quality of these studies was assessed using criteria based on the Cochrane Collaboration's tool for assessing risk of bias. Heterogeneous groups of patients were reported on in the studies. The surgical procedure was often tailored to findings on preoperative imaging. Overall, 50% of patients attained normal resonance, 48% attained normal nasal emissions scores, and 83% had understandable speech postoperatively. However, 5% became hyponasal, 1% had obstructive sleep apnea (OSA), and 17% required further surgery. There were no significant differences in speech outcome between patients who underwent a fat injection, Furlow or intravelar veloplasty, pharyngeal flap pharyngoplasty, Honig pharyngoplasty, or sphincter pharyngoplasty or Hynes procedures. There was a trend that a lower percentage of patients attained normal resonance after a fat injection or palatoplasty than after the more obstructive pharyngoplasties (11–18% versus 44–62%, p = 0.08). Only patients who underwent pharyngeal flaps or sphincter pharyngoplasties incurred OSA, yet this was not statistically significantly more often than after other procedures (p = 0.25). More patients who underwent a palatoplasty needed further surgery than those who underwent a pharyngoplasty (50% versus 7–13%, p = 0.03).</p> <h3>Conclusions/ Significance</h3><p>In the heterogeneous group of patients with 22qDS and VPD, a grade C recommendation can be made to minimize the morbidity of further surgery by choosing to perform a pharyngoplasty directly instead of only a palatoplasty.</p> </div

Menstruation associated hypocalcemic symptoms and serum calcium in patients with idiopathic hypoparathyroidism

BACKGROUND: Some of the patients with idiopathic hypoparathyroidism (IHP) report symptoms of hypocalcemia during menstruation. There is limited data on this observation. METHODS: Twenty six menstruating women with IHP and 26 healthy controls were questioned regarding symptoms suggestive of hypocalcemia during menstruation. Twelve patients and eight controls were asked to prospectively monitor symptoms suggestive of hypocalcemia and premenstrual syndrome (PMS) if any, over two consecutive menstrual cycles. Serum ionized calcium (SiCa(++)), total and albumin adjusted calcium and intact paratharmone (iPTH) were measured at eight points covering menstrual, immediate post-menstrual, mid-cycle and premenstrual phase. RESULTS: Twelve of the 26 (46.2%) patients with IHP reported hypocalcemic symptoms during menstruation as compared to none of the controls. During prospective monitoring, there was no specific trend of hypocalcemic symptoms with respect to the phase of menstrual cycle. The mean SiCa(++), serum total and albumin-adjusted calcium, iPTH and inorganic-phosphorus measured over two menstrual cycles were not significantly different in either of the two study groups. None of the subjects had PMS. CONCLUSION: Women with IHP do not show any trend of hypocalcemic symptoms or fluctuations in serum calcium over different phases of menstrual cycles. Therefore, patients with hypoparathyroidism linking hypocalcemic symptoms with menstruation should be reassured regarding lack of this association

Can radiological results be surrogate markers of functional outcome in distal radial extra-articular fractures?

The purpose of this study was to evaluate the relationship between radiological and functional results in patients with extra-articular fractures of the distal radius. We conducted a prospective study of radiological and functional assessment in 95 consecutively selected extra-articular distal radius fractures. There were two patient groups: more than 60 and less than 60 years of age. The final fracture union radiographs were analysed for their functional outcome using the Michigan Hand Outcomes Questionnaire (MHQ) and the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire. In patients with acceptable radiological results, 62% (MHQ group) and 72% (DASH group) of patients had satisfactory functional outcome. Analysing patients with satisfactory functional results, 56% (MHQ group) and 59% (DASH group) had satisfactory radiological results. There was a higher proportion of patients with better functional results, despite poor radiological results, in both of the age groups. There was a statistically significant correlation between satisfactory radial tilt and functional outcome in the younger patients. In the older age group, patients with satisfactory radiological results had satisfactory functional outcome (p < 0.05). However, in the older age group, satisfactory functional results were achieved, despite unsatisfactory radiological results