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INTRODUCTION: Velopharyngeal insufficiency (VPI) is the inability to close the velopharyngeal sphincter during phonation and/or feeding. VPI is clinically characterised by hypernasal speech and nasal regurgitation. In cases of severe VPI, pharyngoplasty is recommended. Cases of mild-to-moderate VPI can be treated with fat grafting of the posterior pharyngeal wall in addition to speech therapy. The lipofilling can also be useful after pharyngoplasty to improve the outcomes. MATERIALS AND METHODS: Twenty-one patients (14 males and 7 females), ages 4-23 affected by mild-to-moderate VPI and treated with lipofilling were included in this retrospective study. The mean injected fat volume was 7.95 cc (median 6 cc, min 4 cc, max 20 cc and range 16 cc). The follow-up ranged from 6 to 60 months. The pre- and post-operative Borel-Maisonny scores were compared using Wilcoxon test. Moreover, we performed a telephone survey with the aim to assess the parental perception on child's speech and quality of life after the surgical treatment. RESULTS: Despite the small sample size, in this case series, we observed a statistically significant Borel-Maisonny score improvement and a parental satisfaction rate of about 85%. CONCLUSIONS: The augmentation of the posterior pharyngeal wall in addition to speech therapy improved the Borel-Maisonny score and the intelligibility of this case series of patients affected by mild-to-moderate VPI. In these patients, evaluated in a multidisciplinary approach, this technique allowed us to avoid major surgical procedures that would modify the anatomy of the velopharyngeal port. However, prospective comparative studies or randomised controlled trials could be useful to compare fat grafting with velopharyngoplasty techniques, with the aim to clarify indications and to define a specific treatment protocol.
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BACKGROUND: Hypertension (HT) is rarely reported in patients affected by Neuroblastoma (NB), and management guidelines are lacking. Clinical features and perioperative medical treatment in such patients were reviewed to 1) ascertain whether a shared treatment strategy exists among centers and 2) if possible, propose some recommendations for the perioperative management of HT in NB patients. METHODS: A retrospective multicenter survey was conducted on patients affected by NB who presented HT symptoms. RESULTS: From 2006 to 2014, 1126 children were registered in the Italian Registry of Neuroblastoma (RINB). Of these, 21 with HT (1.8%) were included in our analysis. Pre- and intraoperative HT management was somewhat dissimilar among the participating centers, apart from a certain consistency in the intraoperative use of the alpha-1 blocker urapidil. Six of the 21 patients (28%) needed persistent antihypertensive treatment at a median follow-up of 36months (range 4-96months) despite tumor removal. Involvement of the renal pedicle was the only risk factor constantly associated to HT persistency following surgery. A correlation between the presence of HT and the secretion of specific catecholamines and/or compression of the renal vascular pedicle could not be demonstrated. CONCLUSION: Based on this retrospective review of NB patients with HT, no definite therapeutic protocol can be recommended owing to heterogeneity of adopted treatments in different centers. A proposal of perioperative HT management in NB patients is however presented. LEVEL OF EVIDENCE: IV.