Kidney Involvement in Systemic Calcitonin Amyloidosis Associated With Medullary Thyroid Carcinoma.

A 52-year-old woman with widely disseminated medullary thyroid carcinoma developed nephrotic syndrome and slowly decreasing kidney function. A kidney biopsy was performed to differentiate between malignancy-associated membranous glomerulopathy and tyrosine kinase inhibitor-induced focal segmental glomerulosclerosis. Surprisingly, the biopsy specimen revealed diffuse glomerular deposition of amyloid that was proved to be derived from the calcitonin hormone (Acal), produced by the medullary thyroid carcinoma. This amyloid was also present in an abdominal fat pad biopsy. Although local ACal deposition is a characteristic feature of medullary thyroid carcinoma, the systemic amyloidosis involving the kidney that is presented in this case report has not to our knowledge been described previously and may be the result of long-term high plasma calcitonin levels. Our case illustrates that systemic calcitonin amyloidosis should be considered in the differential diagnosis of proteinuria in patients with medullary thyroid carcinoma.

Pediatric rhinoplasty in an academic setting.

Nasal septal surgery and rhinoplasty are controversial in children. Traditionally, an attitude of restraint has been employed by most surgeons till an empirical age of 16 to 18 years. This is to avoid the possible adverse effects that the growth spurts may have on the nose and midface region. Some authors, however, have claimed a paucity of evidence that such untoward effects of surgery are frequent. Research has shown that surgical intervention limited to certain areas of the bony and cartilaginous nasal framework is less likely to affect natural growth patterns. There is a growing consensus toward early intervention, especially in a select group of patients, where deferring the surgery may turn out to be the poorer option in the short and the long term, and suggestions have been made that conservative guidelines may be employed to minimize the unwarranted results. This article presents our experience with septorhinoplasty in children over the last two decades in an academic setting. We have retrospectively studied the pediatric patients who underwent septorhinoplasty at the Academic Medical Centre, Amsterdam, Netherlands. There were 106 children aged between 3 and 19 years who underwent nasal surgery between February 1994 and August of 2007. Sixty-six of these were boys and 40 were girls. Their follow-up ranged from 12 to 157 months with a mean follow-up period of 53 months. Eighteen patients underwent revision surgery. The clinical circumstances, indications for surgery, extent of surgical interference, and outcome in 106 patients are discussed. Importantly, the patients in this series have been followed for variable periods after puberty and adolescence, allowing for assessment beyond the nasal and midfacial growth spurts. Based on our experience, we have outlined the clinical guidelines that we follow for surgery in this age group of patients.

Open rhinoplasty in children.

Septorhinoplasty in children carries the risk of growth disturbance of the nose and premaxilla. The open or external approach has the advantage that the cartilaginous nasal skeleton remains intact. This open approach enables the surgeon to excise dermoid cysts and to realign lower lateral cartilages (unilateral cleft) without disturbing the integrity of the cartilaginous skeleton. In our experience, absolute indications for open rhinoplasty in children include dermoid cyst, cleft lip nose, and septal abscess. Relative indications include septal deviations causing severe nasal airway obstruction and or progressive distortion of the nose. In this article, we discuss the indications, advantages, disadvantages, and operative technique of open rhinoplasty in children. Our indications for open rhinoplasty in children and some selected cases are illustrated.

Acceptance and long-term compliance of nCPAP in obstructive sleep apnea.

In a retrospective cohort study, we evaluated whether improvements in nasal continuous positive airway pressure (nCPAP) technology, particularly the introduction of automatic adjustment of the nCPAP pressure (auto-CPAP), have led to better acceptance and (long-term) compliance in patients with obstructive sleep apnea syndrome (OSAS) as compared to earlier reported data. Questionnaires were sent to 256 patients, who were referred to our clinic for an overnight polysomnography from January 1997 to July 2005 and received nCPAP therapy for OSAS. Of the 256 patients, 24 patients were unavailable for follow-up. Of the remaining 232 patients, 58 patients (25%) had discontinued therapy, while 174 patients (75%) were still using nCPAP after 2 months to 8 years of follow-up. One Hundred and thirty eight (79%) of these 174 patients used nCPAP for at least 4 h/night during >or= 5 nights/week, 82,1% of the conventional nCPAP (fixed pressure CPAP) group (n = 78) and 77,1% of the auto-CPAP group (n = 96). Therefore, including the 58 failures, only 59.5% of patients can be seen as compliant. There were no statistical differences between the fixed pressure CPAP and auto-CPAP users, and between the compliant and non-compliant users according to age, BMI, AHI and Epworth sleepiness scale (ESS). Auto-CPAP patients used significantly more cm H2O. The long-term compliance of nCPAP therapy has have increased only slightly since the introduction of the fixed pressure CPAP 25 years ago, in spite of many efforts to improve it. It seems that a plateau has been reached and that it is unrealistic to aim at a substantially higher compliance rate.

One stage multilevel surgery (uvulopalatopharyngoplasty, hyoid suspension, radiofrequent ablation of the tongue base with/without genioglossus advancement), in obstructive sleep apnea syndrome.

We evaluated the surgical results of a one tempo multilevel surgical approach of the upper airway to treat patients with obstructive sleep apnea syndrome (OSAS) in a prospective case series. Twenty-two patients with OSAS and obstruction at both palatinal and tongue base level, as assessed by sleep endoscopy, underwent UPPP, RFTB, HS with or without GA in one operative session. The mean apnea hypopnea index (AHI) decreased from 48.7 (range 17.4-100.9) to 28.8 (P < 0.0001). The success rate (AHI <20 and >50% reduction in AHI) was 45%, the response rate (reduction in AHI of 20-50%) was 27%. The overall response rate was 72%. The success rates of patients with an AHI <55 and >55 were 56 and 0%, respectively. The overall response rate of patients with an AHI <55 was 78% and >55 was 50%. Improvement of desaturation index was significant from 31.9 to 17.6 (P < 0.0001). Visual analogue scales for snoring and hypersomnolence and the Epworth Sleepiness Scores showed significant improvements too (all P < 0.0001). There was no difference in objective and subjective outcomes between the group with and without GA. This study demonstrates that one stage multilevel surgery, in which genioglossus advancement is not of additional value, is a valuable addition to the therapeutic armentarium and can be considered a viable alternative, objective as well as subjective, to NCPAP or as primary treatment in well selected patients with moderate to severe OSAS with an AHI <55.

Bipolar radiofrequency induced thermotherapy of the tongue base: Its complications, acceptance and effectiveness under local anesthesia.

We assessed adverse events and complications of bipolar radiofrequency induced thermotherapy of the tongue base (RFTB) in patients with socially unacceptable snoring (SUS) or obstructive sleep apnea syndrome (OSAS) and determine its acceptance and effectiveness when conducted under local anesthesia. This investigation consisted of (1) a prospective, open-enrollment study of 24 consecutive patients with snoring and OSAS at the tongue base level only (Fujita III), assessed by sleep endoscopy. Polysomnography, questionnaires, and visual analog scales (VAS) were used to assess outcome. (2) In addition, a retrospective review of 83 patients, who underwent RFTB (in 59 cases as part of a multilevel treatment), was performed to evaluate adverse events and complications. Twenty-two of the 24 patients completed postoperative questionnaires and VAS, and ten patients had postoperative polysomnography. Reduction in snoring (P = 0.0003), hypersomnolence (P = 0.002), and globus (P = 0.031) was significant. A positive trend in AHI (P = 0.001, n = 3) is shown in patients with moderate to severe OSAS. Concerning postoperative adverse events and complications, only two patients had a mild and transient tongue deviation directly after the procedure, which resolved within an hour postoperatively (adverse event rate 1.8%). No postoperative complications such as infections, abscesses, hematomas, or ulcerations of the tongue base occurred. This study demonstrates that bipolar RFTB in patients with obstruction at the tongue base only (Fujita type III) as visualized by sleep endoscopy is a safe and simple procedure under local anesthesia and can be effective in patients with SUS. No complications during this study were observed. Its effect on OSAS has been shown by other authors, although long-term effects are not stable. The RFTB can be considered as first choice treatment in case of snoring and mild OSAS in Fujita type III obstruction. In the case of moderate to severe sleep apnea, RFTB can be considered as an additional treatment.

The role of sleep position in obstructive sleep apnea syndrome.

We analyzed the role of sleep position in obstructive sleep apnea syndrome (OSAS). The polysomnograms of 120 patients with sleep apnea syndrome were analyzed. We associated the apnea hypopnea index (AHI) of the supine position with the AHI of the other positions. Patients were stratified in a group of positional patients (PP) (AHI supine >or= 2 x AHI other positions) and a group of non-positional patients (NPP). In 55.8% of our patients, OSAS was position dependent. PP patients were significantly (6.7 years) younger. BMI and AHI were higher in the NPP group, but the difference was not significant. Level of obstruction in the upper airway (retropalatinal vs retrolingual vs both levels) as assessed by sleep endoscopy was not significantly different between the two groups. Total sleep time (TST) was equal in both groups, but the average time in supine position was 37 min longer in the PP group. This study confirms the finding that in more than 50% of patients, OSAS is position dependent. Apart from age, no patient characteristics were found indicating the position dependency. Overall AHI does not identify positional OSAS.

Sleep endoscopy versus modified Mallampati score in sleep apnea and snoring.

OBJECTIVES/HYPOTHESIS: The aim of this study is to quantify the correlation between a large tongue classified by the modified Mallampati score (MMS) and obstruction at the tongue base observed during sleep endoscopy in patients with socially unacceptable snoring (SUS) and obstructive sleep apnea syndrome (OSAS). STUDY DESIGN: Prospective. METHODS: One hundred twenty-seven patients with obstructive sleep apnea and SUS underwent sleep endoscopy. Patients were classified into four specific groups according to their MMS. After intravenous administration of midazolam, obstructive events in the upper airway were classified. The level of obstruction was expressed as palatal (2), palatal and to a lesser degree base of the tongue/laryngeal level (2), tongue base/laryngeal level and to a lesser degree palatal (3), and tongue base/laryngeal level (3). The degree of correlation between the level of obstruction and the MMS was analyzed by means of Spearman's rank-order correlation. RESULTS: No linear association between the level of obstruction and MMS could be found. A large tongue (MMS 3 or 4) does not predict obstruction at the base of the tongue. Patients classified as MMS 1 or 2 did not frequently show obstruction at the base of the tongue (9 of 38, 23,6%). The majority of the population showed retropalatal obstruction. A large tongue (MMS 3 or 4) was often accompanied by retropalatal obstruction, 76% of the cases (64 of 89). CONCLUSIONS: No positive correlation exists between a large tongue and obstruction at tongue base level. In both groups, retropalatal obstruction occurred more often than retrolingual obstruction. Sleep endoscopy provides qualitative insight into important changes during sleep in patients with SUS and OSAS. In addition, anatomic aberrations are clearly visualized in a dynamic setting. Classification of the size of the tongue, as assessed by the MMS, may be of great importance as well.

Hyoidthyroidpexia: a surgical treatment for sleep apnea syndrome.

OBJECTIVES/HYPOTHESIS: The aim of this study is to evaluate the results of primary hyoidthyroidpexia (HTP) and HTP after previous uvulopalatopharyngoplasty (UPPP) in patients with obstructive sleep apnea syndrome (OSAS). STUDY DESIGN: Prospective case series. METHODS: Thirty-one patients with obstruction at tongue base level and moderate to severe sleep apnea syndrome underwent HTP. Seventeen patients underwent surgery after an unsuccessful UPPP (secondary HTP), and in 14 patients, primary HTP was performed. RESULTS: Patients who underwent primary HTP showed a significant decrease in apnea hypopnea index (AHI) (P = .007), whereas those patients who had secondary HTP did not (P = .06). Overall, the AHI significantly changed (P = .0005). Visual analogue scales for snoring and hypersomnolence and the Epworth sleepiness scores showed significant improvement for both groups, without any difference between them. HTP was considerably less painful when compared with UPPP. CONCLUSION: This study demonstrates that HTP, in particular as primary treatment in cases of obstruction at tongue base level, is a valuable addition to the therapeutic armamentarium of moderate to severe OSAS. Selection criteria are moderate to severe OSAS with preferably a body mass index less than 27, multilevel obstruction with emphasis on the base of tongue, small tonsils, and normal uvula, without a floppy epiglottis or a palatal stenosis after UPPP.