Comparison of two-flap palatoplasty plus intravelar veloplasty technique with and without double-layer Z-plasty on the soft palate length in children with cleft palate.

PURPOSE: Velopharyngeal failure occurs as a result of a nasopharyngeal gap following inadequate velopharyngeal closure for structural or functional cause. We aimed to compare the soft palate length in two-flap palatoplasty with intravelar veloplasty (IVV) and two-flap palatoplasty with IVV plus double-layer Z-plasty combination in patients with cleft palate. METHODS: This clinical trial was conducted on infants aged 9 to 12 months with cleft palate in two groups. The method of two-flap palatoplasty with IVV technique and two-flap palatoplasty with IVV plus double-layer Z-plasty was compared in terms of soft palate length which was measured during operation and short-term complications. RESULTS: This study was conducted on 62 infants including 30 patients in two-flap palatoplasty with IVV group and 32 patients in two-flap palatoplasty with IVV plus double-layer Z-plasty group. The soft palate length in two-flap palatoplasty and IVV with and without Z-plasty groups before surgery was 17.56 ± 2.05 and 17.68 ± 1.88 mm, respectively (P = 0.561). After surgery, the soft palate length was significantly higher in two-flap palatoplasty with IVV plus Z-plasty group (22.43 ± 2.73 mm vs. 20.56 ± 2.42 mm) (P = 0.032). CONCLUSION: The two-flap palatoplasty with IVV plus Z-plasty technique is a suitable method for increasing the palatal length in infants with cleft palate. On the other hand, the addition of Z-plasty method could increase the length of the palate. Moreover, the complications are very low and further trials for development of this method on patients with cleft palate are recommended. TRIAL REGISTRATION NUMBER (TRN): IRCT2017032423559N11.

Effects of phenytoin spray in prevention of fistula formation following cleft palate repair.

BACKGROUND: The effectiveness of topical phenytoin has been reported for the treatment of oral biopsy ulcers, chemotherapy-induced oral mucositis, and chronic periodontitis. This study aimed to investigate the effects of topical phenytoin 2% on the prevention of fistula formation after cleft palate repair. METHOD: This randomized clinical trial studied patients with nonsyndromic cleft palate who were referred to a tertiary center and underwent cleft palate repair from March 2010 to February 2015. Patients in the phenytoin group received phenytoin spray 2% for 8 weeks and were compared with an age- and sex-matched control group. RESULTS: A total of 160 patients in two phenytoin and control groups (n = 80 for each group) were recruited to the study. The mean ages of patients in the phenytoin and control groups were 11.42 ± 1.30 and 11.08 ± 1.25 months, respectively. The results showed that six patients (7.5%) in the phenytoin group and 15 patients (18.8%) in the control group formed fistulas during the 6-month follow-up period. There was a significant difference in fistula formation between the phenytoin and control groups (p = 0.035). Furthermore, fistula size was significantly smaller in the phenytoin group compared with the control group (p < 0.001). CONCLUSION: More frequent use of phenytoin spray can be considered, although there is insufficient information on the long-term side-effects of the chosen drug.

Prevalence of Associated Anomalies in Cleft Lip and/or Palate Patients.

INTRODUCTION: Orofacial clefts are among the most common congenital anomalies. Patients presenting with orofacial clefts often require surgery or other complex procedures. A cleft lip or palate can be a single anomaly or a part of multiple congenital anomalies. The reported prevalence of cleft disease and associated anomalies varies widely across the literature, and is dependent on the diagnostic procedure used. In this study we determined the prevalence of associated anomalies in patients with a cleft lip and/or palate, with a specific focus on cardiac anomalies. MATERIALS AND METHODS: In this cross-sectional study, 526 patients with a cleft lip and /or palate admitted to the children's referral hospital between 2006 and 2011 were evaluated. All associated anomalies were detected and recorded. Patient information collected included age, gender, type and side of cleft, craniofacial anomalies and presence of other anomalies, including cardiac anomalies. Data were analyzed using SPSS version 16. RESULTS: Of the 526 patients enrolled in the study, 58% (305) were male and 42% (221) were female. In total, 75% of patients (396) were aged between 4 and 8 years and 25% (130) were aged less than 4 years. The most common cleft type in our study was bilateral cleft palate. The most commonly associated anomaly among cleft patients, in 12% of cleft patients, was a cardiac anomaly. The most common cardiac anomaly was atrial septal defect (ASD). CONCLUSION: The prevalence of associated anomalies among orofacial cleft patients is high. The most common associated anomaly is cardiac anomaly, with ASD being the most common cardiac anomaly. There are no significant relationships between type of cleft and associated cardiac anomalies.

Common Mutations of the Methylenetetrahydrofolate Reductase (MTHFR) Gene in Non-Syndromic Cleft Lips and Palates Children in North-West of Iran.

INTRODUCTION: Cleft lips and cleft palates are common congenital abnormalities in children. Various chromosomal loci have been suggested to be responsible the development of these abnormalities. The present study was carried out to investigate the association between the suspected genes (methylenetetrahydrofolate reductase [MTHFR] A1298C and C677T) that might contribute into the etiology of these disorders through application of molecular methods. MATERIALS AND METHODS: This cross-sectional and explanatory study was carried out on a study population of 65 affected children, 130 respective parents and 50 healthy individuals between 2009 and 2012 at Tabriz University of Medical Sciences, IR Iran. After DNA extraction, amplification refractory mutation system-polymerase chain reaction (ARMS-PCR) and restriction fragment length polymorphism (RFLP)-PCR were used respectively to investigate the C677T and A1298C mutations for the MTHFR gene. RESULTS: There was a significant difference in the rates of the C677T mutation when affected patients and their fathers were compared with the control group (odds ratio [OR]=0.44) (OR=0.64). However, there was no significant difference observed in the rate of this mutation between the patients' mothers and the control group (OR=1.35). In addition, the abnormality rate was higher in patients with the A1298C mutation and their parents, when compared with the control group. This abnormality rate was higher for the affected children and their fathers in comparison with their mothers (Fathers, OR=0.26; Mothers, OR=0.65; Children, OR=0.55). No significant difference was seen in the rate of the polymorphism C677T in its CC, when the affected children and their parents were compared with the control group. However, there was a significant difference in the A1298C mutation. CONCLUSION: An association was seen between the A1298C mutation and cleft lip and cleft palate abnormalities in Iran. However, there seems to be a stronger relationship between the C67TT mutation and these abnormalities in other countries, which could be explained by racial differences. Moreover, this association was more notable between the affected children and their fathers than their mothers. The findings in this study may be helpful in future studies and screening programs.

Medical treatment of allergy in children with recurrent or chronic sinusitis.

BACKGROUND: Diagnosis and treatment of chronic and recurrent sinusitis in children is of potential importance and many factors can havean influence on it. This study assessed the chronic and recurrent sinusitis and affecting factors, as well as the role of allergy in its course and treatment in children. MATERIALS AND METHODS: A total of 106 children with the diagnosis of chronic or recurrent sinusitis, referred to specialty clinics of otolaryngology and allergy of Tabriz Children Educational-Medical centres since 2010 to 2012, were enrolled. The history and physicalexamination findings were recorded for all patients and allergy Prick test was done for all. Response to treatment was evaluated during the follow-up visits. RESULTS: The mean age of studied patients was 6.5 ± 2.9 years. Of all the patients, 54 (50.9%) were male and 52 (49.1%) were female. Skin Prick test was positive in 69.8%. Response to treatment was seen in 86.8% of the patients while 7.5% did not have any favourable outcome. Anti-allergic treatment caused better outcomes in patients with positive Skin Prick test than those with negative results. CONCLUSION: The prevalence of allergic disease in children with chronic or recurrent sinusitis is considerable and anti-allergic treatments can result infavourable therapeutic outcomes in children with sinusitis, especially with positive skin prick test results.

First branchial cleft fistula associated with external auditory canal stenosis and middle ear cholesteatoma.

INTRODUCTION: First branchial cleft anomalies manifest with duplication of the external auditory canal. CASE REPORT: This report features a rare case of microtia and congenital middle ear and canal cholesteatoma with first branchial fistula. External auditory canal stenosis was complicated by middle ear and external canal cholesteatoma, but branchial fistula, opening in the zygomatic root and a sinus in the helical root, may explain this feature. A canal wall down mastoidectomy with canaloplasty and wide meatoplasty was performed. The branchial cleft was excised through parotidectomy and facial nerve dissection. CONCLUSION: It should be considered that canal stenosis in such cases can induce cholesteatoma formation in the auditory canal and middle ear.

Effect of peripheral edema on oscillometric blood pressure measurement.

INTRODUCTION: Blood pressure (BP) measurement is essential for epidemiological studies and clinical decisions. It seems that tissue characteristics can affect BP results and we try to find edema effect on BP results taken by different methods. METHODS: BP of 55 children before open heart surgery were measured and compared according to three methods: Arterial as standard and reference, oscillometric and auscultatory methods. Peripheral edema as a tissue characteristic was defined in higher than +2 as marked edema and in equal or lower than +2 as no edema. STATISTICAL ANALYSES: data was expressed as Mean and 95% of confidence interval (CI 95%). Comparison of two groups was performed by T independent test and of more than two groups by ANOVA test. Mann-Whitney U and paired T-test were used for serially comparisons of changes. P less than 0.05 was considered significant. RESULTS: Fifty five children aged 29.4±3.9 months were divided into two groups: 10 children with peripheral edema beyond +2 and 45 cases without edema. Oscillometric method overestimated systolic BP and the Mean (CI 95%) difference of oscillometric to arterial was 4.8 (8/-1, P=0.02) in edematous and 4.2 (7/1, p=0.004) in non edematous. Oscillometric method underestimated diastolic BP as -9 (-1.8/-16.5, P=0.03) in edematous group and 2.6 (-0.7/+5, P= 0.2) in non edematous compared to arterial method. CONCLUSION: Oscillometric device standards cannot cover all specific clinical conditions. It underestimates diastolic BP significantly in edematous children, which was 9.2 mmHg in average beyond the acceptable standards.

A case report: nager acrofacial dysostosis.

INTRODUCTION: Nager syndrome is a malformation resulting from problems in the development of the first and second branchial arches and limb buds. The cause of the abnormal development of the pharyngeal arches in Nager syndrome is unknown. It is also unclear why affected individuals have bone abnormalities in their arms and legs. Nager syndrome is thought to have an autosomal recessive inheritance pattern when unaffected parents have more than one affected child. The purpose of this report is to present a case of Nager syndrome where the patient exhibited upper limb shortening, an unusual feature that has been reported as coexisting in some individuals with Nager syndrome. CASE REPORT: A 3.5-year-old girl was referred to our Department of Pediatric Otorhinolaryngology due to a cleft palate. Her craniofacial anomalies included malar hypoplasia, severe mandibular hypoplasia with retrognathia, downward slanted palpebral fissures, a high narrow hard palate, absent soft palate, small retroplaced tongue, bilateral external auditory canal atresia, and dysplastic ears. There was no evidence of mental retardation. Based on the craniofacial characteristics and the coexisting upper limb preaxial anomalies, a diagnosis of Nager syndrome was confirmed. CONCLUSION: Nager syndrome is a rare disorder resulting from developmental abnormalities of the first and second branchial arches. It is linked to five other similar syndromes: Miller syndrome, Treacher-Collins, Pierre-Robin, Genee-Wiedemann, and Franceschetti-Zwahlen-Klein. Multidisciplinary management by a craniofacial team is needed. Early intervention, intensive education, new surgical techniques, and an emphasis on coordinated care have improved the quality of life in this patient with Nager syndrome.