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EDITORIAL |
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Why a need for a new journal on cleft and craniofacial? |
p. 1 |
Karoon Agrawal DOI:10.4103/2348-2125.126533 |
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FOREWORD BY THE PRESIDENT |
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Cleft care at cross road |
p. 3 |
Arun Kumar Singh DOI:10.4103/2348-2125.126535 |
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REVIEW ARTICLE |
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Diagnosis and management of velopharyngeal insufficiency following cleft palate repair |
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Michael S Gart, Arun K Gosain DOI:10.4103/2348-2125.126536 Background: Cleft lip and palate repair seeks to restore normal form, improve feeding and achieve normal speech, which requires velopharyngeal competence. The absence of this ability, termed velopharyngeal insufficiency (VPI), is seen in a wide range of patients following primary cleft palate repair. This review article focuses on patient assessment and the surgical management of VPI. Recent trends and future directions in management are also presented. After reading, one should be able to describe the various treatment approaches for a patient with suspected VPI. Materials and Methods: A PubMed search was conducted using the following search terms: VPI, velopharygneal incompetence, VPI, velopharynx, velopharyngeal port, velopharyngeal mechanism, veloplasty, intravelar veloplasty and hypernasal speech. Relevant manuscripts were identified by abstract review and additional articles selected based on bibliography review. Articles were restricted to those in the English language. A total of 88 articles were selected for further review. Conclusions: VPI is a common complication following primary palatoplasty. The decision to operate, as well as the selection of operative procedure, depends on a multimodal patient assessment, including speech evaluation and imaging studies of the pharyngeal mechanism. A thorough understanding of velopharyngeal anatomy and physiology is crucial to understanding the deficits in patients with VPI as well as the myriad methods of surgical correction. While many techniques are available, there are no conclusive data to guide procedure choice and newer techniques of imaging and treating patients with VPI continue to evolve. |
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ORIGINAL ARTICLE |
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Buccinator myomucosal flap in cleft palate repair: Revisited |
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Bhaumik Bhayani DOI:10.4103/2348-2125.126540 Objective: To present various ways of transfer and result of buccinator myomucosal flap (BMMF) in primary and secondary repair of palatal cleft and palate fistula. Design: This study has been designed on the basis of a clinical experience and literature search. Route of single or two BMMF(s) transfer was either lateral or posterior to the greater palatine neurovascular bundle. The flap has been used to repair defect in nasal layer, oral layer, or in combined nasal as well as oral layer of the palate. Materials and Methods: More than 160 palatoplasties have undergone palate repair with the use of BMMFs between 1999 and 2011. The analysis includes 98 palate repair. Unilateral flap was used most commonly in primary repair of nasal side of wide unilateral cleft palate (CP); whereas, two flaps were used mostly in primary repair of wide bilateral CP and in secondary palate repair for large fistula. Results: The fistula rate; in primary palatoplasty patients was 4.8% and in secondary palatoplasty patients, it was 8.3%. Good speech has been achieved in primary palatoplasty patients. After secondary palatoplasty also patients had good speech following therapy. Conclusion: The presented technique has been effective in anatomical and functional repair of wide palatal defects primary as well as secondary. The literature has been reviewed along with. |
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COMMENTARY |
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Buccinator myomucosal flap in cleft palate repair: Revisited |
p. 17 |
Richie Gupta |
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CME |
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Complications of cleft palate repair and how to avoid them  |
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Jyotsna Murthy DOI:10.4103/2348-2125.126546 Introduction: The cleft palate repair is commonly performed procedure in plastic surgery practices. In addition, this is also procedure done by trainees to the senior most surgeons. One of common procedure for trainee who are introduced to cleft lip and palate surgeries. Literature is flooded with articles on complication of cleft palate repair and probable factors influencing it till the latest one, which suggest that we are far from getting desirable results in cleft palate repair. Review: The common complications of cleft palate repair are fistulae, velopharyngeal insufficiency and detrimental effect on maxillary growth. Palatal fistula is commonly stated in literature with variable incidence ranging from 3-40% 1 , with an average of 7-10%. Other complications are poor speech outcome and poor growth potential of maxillary bone due to scars following palate repair. Every attempt needs to be made to avoid complications or reduce the rate of complications. This article reviews the factors and pitfalls that are likely to increase the chances of complication following palate repair. Conclusion: As surgeon we are duty bound to reduce the avoidable complication, specially related to judgment and techniques. However, the complication due to inherent deficiency of tissue like hypoplastic soft palate muscles and poor scarring tendencies leading to complications are not avoidable. |
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ORIGINAL ARTICLES |
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Clinical profile and treatment status of subjects with cleft lip and palate anomaly in India: Preliminary report of a three-center study |
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OP Kharbanda, Karoon Agrawal, Rakesh Khazanchi, Suresh C Sharma, Sushma Sagar, Manish Singhal, Neeraj N Mathur, Kumud Kumar Handa, Madhulika Kabra, Neerja Gupta, Neeraj Wadhwan DOI:10.4103/2348-2125.126550 Context: Treatment of patients with cleft lip and palate (CLP) anomaly requires a multidisciplinary approach from birth until adulthood. Many children with cleft anomaly are born in rural areas where resources for treatment and awareness on cleft care are limited. Consequently, many patients may receive limited or suboptimal care due to multitudes of reasons. Aims: The current study was aimed to record the baseline data on the spectrum of clinical profile of cleft patients, treatment protocols, quality of treatment and the residual treatment needs of patients with CLP anomaly visiting three major hospitals across Delhi and National Capital Region (NCR). The experience gained from the three-center study would be used to lay a framework to conduct a nationwide multicenter study in terms of logistics, feasibility and difficulties. Materials and Methods: The study titled "CLP anomaly in India: Clinical profile Risk factors and current status of treatment: A hospital based study" was started in 2010 as a Task Force project of Indian Council of Medical Research. The Pilot phase, which started in 2012, encompassed three cleft centers across Delhi and NCR, namely, All India Institute of Medical Sciences, Safdarjang Hospital and Medanta - The Medicity. Data for 126 non-syndromic CLP subjects was recorded on a specially designed performa. Each case was evaluated by a team of specialists comprising of a Plastic Surgeon, an Orthodontist, ENT Surgeon, Dental Surgeon, Speech therapist and an Audiologist. Clinical records included profile and intraoral photos, dental study models, audiometric and speech evaluation data. The current paper attempts to highlights a few of relevant observations of the pooled data from three centers. Results and Conclusions: The results indicate a lack of uniform protocol followed in providing care to cleft patients. A great variation was found in the quality of treatment received by many of the patients. |
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Clinico-epidemiological profile of orofacial clefts among children of coastal district of Southern India: A 5 year hospital based study |
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Supriya Nambiar, Prateek Singhal, Anand Menon, B Unnikrishnan DOI:10.4103/2348-2125.126554 Background: In India with a large multi ethnic population, most of the epidemiological studies on cleft lip/palate (CL/P) have been sporadic. Inequalities exist, both in access to and quality of cleft care with distinct differences in urban versus rural access and over the years the accumulation of unrepaired clefts of the lip and palate make this a significant health care problem in India. Aims and Objectives: The primary aims of this study was to assess the predominant pattern of cleft lip and palate (CLP) cases in Mangalore and secondary aim is to evaluate the role of possible risk factors like previous familial history, maternal diet and nutritional supplementation, consanguinity. Materials and Methods: This was a 5-year hospital based, retrospective study of the birth records, in which all the cases of CL/P up to the age group of 5 years were studied. The data was retrieved from the Medical Records Department of two main hospitals in Mangalore. The data was collected using a semi-structured, pre-tested proforma that was designed based on the review of literature. Results: The cleft occurrence in Mangalore was found to be 2.42/1000 births/year. Cleft lip (CL) along with cleft palate (CP) was found to be the highest in both Hospitals about (64.6%), followed by isolated CL (28.5%), followed by isolated CP (5.1%) and the least being isolated CL with cleft alveolus (1.9%). Conclusions: From the study, it can be concluded that recording of adequate ante-natal history in the birth records hasn't been given critical Importance. Furthermore, there is a lack of parental counseling provided in the government hospital. |
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Cleft data from surgical camps on rails: A doorstep health care delivery |
p. 38 |
Sameek Bhattacharya, Ashish Rai, Prabhat Shrivastava DOI:10.4103/2348-2125.126557 Aim: The aim of the following study is to investigate the epidemiology of cleft lip and palate (CL/P) patients in a given population. Patients and Methods: Data from cleft camps conducted at Life Line Express Hospital-on-train in Madhya Pradesh, India from year 2003 to 2007 was analyzed. A total of 839 patients of non-syndromic CL/P were included in the study. The data was analyzed for age of the patients, type of cleft, laterality of the defect, their relation to the gender of the patient and presence of cleft in first degree relatives. Result: Nearly 59.8% of the patients were in the age group of 5-18 years. There was an overall male predominance. Unilateral clefts were recorded in 83.3% and bilateral clefts in 14.7% of cases. Only 2% of the patients had isolated cleft palate (CPO) and females had 2.3 times higher risk for CPO than males. Of unilateral cleft lip, left side was more common (67.3%). Nearly 4.9% patients had history of familial inheritance among first degree relatives. Conclusion: In India, vast territories lack basic medical amenities and all modern medical facilities are limited to cities. "Cleft surgery at doorstep" is an extremely effective strategy in treating the "hidden" cleft population and collecting a meaningful epidemiological data from a "virgin" territory. We also stress upon the need to include facial clefts as a notifiable disease in our country to understand the gender, regional and ethnic variations of the disease. |
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Association of cervical vertrebra anomalies with cleft lip and palate |
p. 43 |
Mudita Srivastava, Anshul Aggarwal, Puneet Batra, Sangeev Datana, Prasanna Kumar, Klara Agneta Macrcusson DOI:10.4103/2348-2125.126561 Introduction: The aim of the present study was to evaluate the developmental relationship in the vertebral column in the cervical region and to relate its association in patient's with cleft lip and palate. Materials and Methods: The sample consisted of 228 cleft patients in the experimental group and 225 non cleft samples in control group. Cleft patients are subdivided into cleft palate only (CPO), Unilateral cleft lip and palate (UCLP) and bilateral cleft lip and palate (BCLP). Cervical vertebrae were traced on lateral cephalogram in children with cleft lip and palate to determine the prevalence of vertebral anomalies and their categorization. The cervical vertebral anomalies were classified into two types: posterior arch deficiency (PAD) and fusion anomalies (FUS). Results and Conclusion: The study showed 19 percent of cleft patients with cervical vertebral anomalies and 4 percent of control group with cervical vertebral anomalies. Posterior arch deficiency occurred more frequently in unilateral cleft lip and palate(UCLP) and cleft palate only (CPO), Fusion occurred significantly more often in bilateral cleft lip and palate (BCLP). |
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Reconstruction of the superior gingiva-labial sulcus in bilateral cleft lip palate patients: Our experience |
p. 48 |
Divya Narain Upadhyaya, Arun K Singh, Vijay Kumar, Brijesh Mishra, Veerendra Kumar DOI:10.4103/2348-2125.126564 Introduction: In many bilateral cleft lip palate patients the prolabium often remains adherent to the premaxilla and the upper alveolar-labial sulcus is absent. Cleft surgeons have struggled with this problem for many decades and a number of procedures have been described in the literature to correct this deformity. Materials and Methods: A retrospective review of the records of all patients who underwent upper gingivobuccal sulcus reconstruction between August 2003 and December 2012 was carried out. Results: A total of 97 patients were underwent upper gingivolabial sulcus reconstruction with full thickness skin graft from August 2003 to December 2012, a period of 9 years and 5 months. Discussion: An adequate sublabial or gingivolabial sulcus is crucial to both the function as well as the esthetics of the upper lip. Paucity of tissues in bilateral cleft lip and palate patients during lip repair often leads to a 'tight' repair with little or no sulcus at all. The full thickness skin graft has many benefits. It can be harvested in greater quantities than the mucosal graft, takes well, is hairless and does not contract or harden to any significant degree. One significant disadvantage of the skin graft in the upper labial sulcus is its pigmentation that can continue to irk the patients even after several years and probably forever. |
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MISSION IMPOSSIBLE MADE POSSIBLE |
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Parents' forum: A novel mission |
p. 52 |
Amik Garg, Deepa George DOI:10.4103/2348-2125.126568 Cleft management in India has come a long way in last decade or so as far as medical assistance to the children in terms of good quality surgery, orthodontic treatment, and so on is concerned. Presently, there are a number of organisations, both government and nongovernment taking remarkable initiatives in this direction with increasing number of quality surgeries being performed year after year. However, a gap still exists in the availability of ''parents groups'' working in this direction to address the anxiety and providing assistance to the new parents of cleft children. Recently, an initiative has been taken by the parents of the cleft children in India to address this gap that has led to the formation of a parent association with the sole objective of identifying/addressing various issues from the perspective of the new parents and disseminating required information and advice to them. This article discusses the need of such an association, its genesis, objectives, and the present status. |
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CASE REPORTS |
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Oropalatal dysplasia: A case report with an innovative sequence of reconstruction |
p. 55 |
Karoon Agrawal, Sanjay Kumar, Rakesh Kumar Srivastava, Mukund Gupta DOI:10.4103/2348-2125.126570 A rare syndromic cleft palate child with microstomia and ankyloglossia is presented. The child presented at 1-month of age with microstomia, complete cleft of the secondary palate, ankyloglossia with dorsal groove of the tongue. There was no family history of oropalatal anomaly. This is a case of Bettex Graf Syndrome. The karyotype was normal 46XY. The reconstructive surgery was delayed to facilitate anesthesia and surgery. The reconstruction was carried out in one stage with an innovative sequence. Microstomia was released using Y-V plasty; palate repair was carried out with Bardach's two flap palatoplasty and ankyloglossia was released with V-Y technique. The child has been followed-up for more than 6 months. Mouth opening is satisfactory, though there is recurrence of microstomia. Child is able to protrude the tongue well, however, it remains grooved. |
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Joubert syndrome with cleft palate |
p. 59 |
Annavarapu Gopalakrishna, Rajesh Jinka, TS Kumar, Basheer Ahmed Khan, Keyur Mevada DOI:10.4103/2348-2125.126573 Joubert syndrome is a rare autosomal recessive disorder with key finding of cerebellar vermis hypoplasia with a complex brainstem malformation that comprises the "molar tooth sign" on axial magnetic resonance images. Many congenital malformations such as polydactyly, retinal abnormalities, renal malformations, tongue and other orofacial malformations including midline cleft lip have been reported. In the review of literature we did not come across specific mention of a cleft palate. Until date approximately 200 such cases have been reported in the literature. We present a case of a 7-year-old female patient who has been under treatment since she was 1 year old. She had a microform midline cleft of the lip, a complete cleft of the secondary palate, polydactyly in both hands and feet, along with the features of Joubert syndrome including episodes of apnea and tachypnea, abnormal ocular movements, atonia and the classic "molar tooth sign" on axial magnetic resonance imaging. |
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A simple innovation for improving the donor site scar of the Abbe flap |
p. 62 |
Nitin J Mokal, Mahinoor F Desai DOI:10.4103/2348-2125.126576 The Abbe flap is commonly used for the correction of secondary deformities of cleft lip. The midline lower lip scar of the donor site can lead to a bridging contracture when it crosses the labiomental crease. By addition of a small Z-plasty we can ensure that the concavity of the crease is maintained with a superior aesthetic result. |
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Increased frontonasal angle and surface area of mandibular antegonial notch; reliable signs of Treacher Collins syndrome |
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Gyan P Singh, Sneh Lata Verma, Pradeep Tandon, Divya Mehrotra DOI:10.4103/2348-2125.126579 Background: Treacher Collin Syndrome is one of the most disfiguring congenital anomalies of the face, the visible part of the human body used for the identification of an individual. It is an inherited disorder in which there are bilateral symmetric anomalies of the structures within the first and second branchial arches. In general, there is complete penetrance and variable expressivity of the trait. Increased frontonasal angle and deep antegonial notch are syndrome specific characteristic distinguishing Treacher Collin Syndrome from other Syndromes. Main objectives of the presenting this case are to evaluate the validity of the aforementioned clinical signs for the diagnosis and to find out the significance of the surface area of antegonial notch in patient suffering from Treacher Collins syndrome. Methods: Persons of two generations of a family effected with Treacher Collins syndrome were examined for two cardinal signs traditionally associated with this disorder along with the normal siblings of the second generation for the above parameters. Conclusion: Significantly increased fronto nasal angle, frontal sinus area and deep antegonial notch was found in the father and son exhibiting characteristic signs of Treacher Collins-Franceschetti Syndrome in comparison to normal individuals of the family. |
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