IMPORTANCE Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis, and upper airway obstruction. Currently, no consensus exists regarding the diagnosis and evaluation of children with RS. An international, multidisciplinary consensus group was formed to begin to overcome this limitation. OBJECTIVE To report a consensus-derived set of best practices for the diagnosis and evaluation of infants with RS as a starting point for defining standards and management. EVIDENCE REVIEW Based on a literature review and expert opinion, a clinical consensus report was generated. FINDINGS Because RS can occur as an isolated condition or as part of a syndrome or multiple-anomaly disorder, the diagnostic process for each newborn may differ. Micrognathia is hypothesized as the initiating event, but the diagnosis of micrognathia is subjective. Glossoptosis and upper airway compromise complete the primary characteristics of RS. It can be difficult to judge the severity of tongue base airway obstruction, and the possibility of multilevel obstruction exists. The initial assessment of the clinical features and severity of respiratory distress is important and has practical implications. Signs of upper airway obstruction can be intermittent and are more likely to be present when the infant is asleep. Therefore, sleep studies are recommended. Feeding problems are common and may be exacerbated by the presence of a cleft palate. The clinical features and their severity can vary widely and ultimately dictate the required investigations and treatments. CONCLUSIONS AND RELEVANCE Agreed-on recommendations for the initial evaluation of RS and clinical descriptors are provided in this consensus report. Researchers and clinicians will ideally use uniform definitions and comparable assessments. Prospective studies and the standard application of validated assessments are needed to build an evidence base guiding standards of care for infants and children with RS.
Objective:Palatal fistula is a significant complication following cleft palate repair. The guidelines of management of the palatal fistula is dependent on the type of cleft, site of fistula, condition of surrounding tissue and associated problem. We studied the management and outcome of 194 cleft palate fistula in our institute.Design:We present the descriptive hospital-based study of management of palatal fistula in 194 cleft patients. We have excluded all the syndromic children and children whose anterior palate was not operated as per protocol.Settings:Of 194 cleft palate fistula, 37 had palate repair in our hospital and 157 were refereed with fistula following palate repair.The patients were evaluated by interdisciplinary team and plan of management was decided.Result:Various parameters like types of cleft, site of fistula and management of fistula were studied in all the patients. Fifty-two percent were in unilateral CLP and 30% in bilateral CLP because unilateral CLP is the commonest type of cleft. Postalveolar and hard palate region contributing to 67% of all fistulae, followed by junctional in (9%). Seventy-two percent of fistula were amenable for repair by local available tissue, 28% needed tongue flap due to shortage of tissue. Minor numbers have failure of procedure for fistula closure needing further management.Conclusions:This descriptive study present analysis of management of fistula in our institute. It also reinforces that patient with bilateral cleft lip and palate more likely to have shortage of local tissue needing the local flaps like tongue flap compare to other cleft types. The surgical management of fistula can be combined to tackle the associated problems.
Bilateral infraorbital block is superior to fentanyl in terms of analgesia, and time to awakening and feeding.
The birth prevalence of orofacial clefts, one of the most common congenital anomalies, is approximately one in 700 live births, but varies with geography, ethnicity, and socio-economic status. There is a variation in infant mortality and access to care both between and within countries, so some clefts remain unrepaired into adulthood. Quality of care also varies, and even among repaired clefts there is residual deformity and morbidity that significantly affects some children. The two major issues in attempts to address these inequalities are (a) etiology/possibilities for prevention and (b) management and quality of care. For prevention, collaborative research efforts are required in developing countries, in line with the WHO approach to implement the recommendations of the 2008 Millennium Development Goals (www.un.org/millenniumgoals). This includes the "common risk factor" approach, which analyzes biological and social determinants of health alongside other chronic health problems such as diabetes and obesity, as outlined in the Marmot Health inequalities review (2008) (www.ucl.ac.uk/gheg/marmotreview). Simultaneously, orofacial cleft research should involve clinical researchers to identify inequalities in access to treatment and identify the best interventions for minimizing mortality and residual deformity. The future research agenda also requires engagement with implementation science to get research findings into practice.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.