Objectives: This study aimed to determine the clinical profile of patients with orofacial clefts (OFC) who presented at a dental hospital in Johannesburg, South Africa, from January 1, 2013, to December 31, 2019. The aim of the study is to use the data collected to conduct an epidemiological survey and create an electronic database of all patients with OFC who presented to the hospital. Materials and Methods: A cross-sectional, retrospective record review of all cases of OFC treated at Johannesburg Cleft clinic from January 1, 2013, to December 31, 2019, was conducted. A structured, pretested record review form was used to obtain demographic and clinical information of patients with OFC. STATA version 15 was used to analyze the data and statistical tests were conducted at a 5% significance level. Results: One hundred and thirty-three records were analyzed. The majority were males (n = 68, 51.13%), while 48.87% (n = 65) were females. Most patients were South Africans 91.73% (n = 122,), and the majority were African blacks (67.67%). A statistically significant difference (P < 0.05) was found in gender and racial distribution of OFC. Majority of the sample presented with clefts of the lip and palate 55.64% (n = 74,), followed by isolated CP 24.81% (n = 33), CL 17.29% (n = 23), and 2.26% (n = 3) presented with a facial cleft. Majority of the clefts were described as unilateral (n = 50, 37.59%) and 30.83% (n = 41) of the clefts were described as bilateral. Isolated cleft of the palate accounted for 24.81% (n = 33), while 4.51% (n = 6) were midline clefts. Facial clefts accounted for 2.26% (n = 3). Of all unilateral clefts (n = 50), those who presented on the left side account for 68% (n = 34), while those who presented on the right side accounted for 32% (n = 16). Conclusions: The dental hospital is providing services to a large number of patients with OFC. The study findings should assist in planning services for these patients.

Background: Orofacial clefts (OFCs) are one of the most common congenital anomalies worldwide. The purpose of the present study is to identify the incidence of different types of OFCs and the presence of known risk factors among cleft cases in the Pakistani population. Patients and Methods: A retrospective study was conducted at a comprehensive cleft care center, and data were collected from patient files from December 2018 to July 2021. Only cases of cleft lip and/or cleft palate (CL/P) that presented to center were included. SPSS was used for data analysis, and risk factors associated with OFCs were identified. Results: The study included 1269 patients with CL/P. Of the total, 677 (53.3%) patients had cleft lip with cleft palate, 211 (16.6%) had cleft lip only, and 365 (28.7%) had cleft palate only. Bilateral complete cleft lip and palate (n = 276) was most common among the participants followed by midline incomplete cleft palate (n = 215). One hundred and six (8.3%) of the cases were syndromic, and among the remaining nonsyndromic cases, 240 (18.9%) had other associated anomalies. Chi-square tests revealed the following risk factors for CL/P: consanguinity among the parents (P < 0.001), a complication during pregnancy (P < 0.001), medication use during pregnancy (P < 0.001), maternal smoking or exposure to smoking (P = 0.002), history of miscarriage (P = 0.02), and positive family history (P < 0.001). Logistic regression analysis for these factors showed maternal smoking (odds ratio [OR]: 1.99), consanguineous marriage (OR: 1.89), complication during pregnancy (OR: 1.98), and positive history of cleft (OR: 1.9) to be increasing the odds of cleft development. Conclusion: While previous studies have shown many environmental factors to be associated with development of OFCs in the child, the present study provides a quantitative estimate of the risk posed by each individual factor in the Pakistani population.

Objectives: The aim of the study was to assess the influence of the experience of the surgeon on the occurrence of fistulas and breakdowns following palatoplasty. Materials and Methods: A retrospective review on the outcomes of palatoplasty done by a young surgeon in his initial 3 years of operating cleft palate was done. Cleft palate repair was performed using the Pinto's modification of Wardill–Kilner palatoplasty, Veau-Wardill-Kilner V-Y Pushback in both the techniques radical levator muscle dissection was carried out. Data were collected for age, sex, date of birth, syndrome, cleft palate type, type of repair, cleft width, length of soft palate, quality and quantity of muscle, fistula occurrence, and location of fistula. Results: Retrospective analysis was done on the outcomes of palatoplasty performed by a young surgeon in his initial 3 years at Charles pinto center for cleft lip palate and craniofacial on 220 cleft palate children which included all variants and dimensions of cleft palates. Postoperatively, the incidence of palatal fistulas occurred in 12 patients, three patients had bifid uvula, however, out of 12 patients who had fistulas only four needed fistula closure and one required a uvula re-repair; the rest healed well. Conclusion: We believe there is a learning curve in performing cleft palate repair. Our technique and principles followed in palatoplasty appear to have a low or zero fistula rate even in the initial period of learning. Furthermore, effective mentorship and guidance help in reducing errors and providing a better outcome.

Objective: To report the applicability of ambulatory cleft lip repair (CLR) and to review the existing literature. Materials and Methods: This is a retrospective clinical case series study of ambulatory CLR in North-western Nigeria. The study was carried out in Usmanu Danfodiyo University Teaching Hospital (UDUTH), Sokoto, between January 2012 and December 2018. Relevant clinical data of the subjects obtained include sociodemographics, type of cleft, and complications. Protocol employed includes premedication with diazepam (0.5 mg/kg, IV) slowly, followed by administration of glycopyrrolate (0.005 mg/kg, IV) and diclofenac potassium (1 mg/kg, IV). Then, ketamine (0.1 mg/kg, IV) was infused slowly over a 5-min period to achieve procedural sedation (Ramsey sedation score of 3). The data were analyzed using (SPSS; Chicago, IL, USA) program, version 20. The results were presented in simple descriptive statistics. Results: The age ranged from 2 to 21 years with male preponderance (M: F = 4:1). All participants had unilateral clefts lip with or without alveolus. The left side was twice involved as the right. There were no perioperative complications nor mortality recorded and no patient required conversion to general anesthesia. All patients were successfully discharged home on the same day of the operation. No case of readmission was recorded and subsequent follow-up was uneventful. Conclusions: Parenteral use of ketamine and diazepam for outpatient pediatric cleft lip procedures is safe and of immense benefits in reducing the surgical cleft burden without compromising patients safety.

Introduction: In India, approximately 35,000 new cleft patients are born every year. Many patients receive suboptimum, improper, little, or no treatment. The cumulative burden of cleft care is up to 1 million cases. The spectrum of problems is varied, the caseload is enormous, and the logistics of treatment delivery are complicated. The Indian Council of Medical Research (ICMR) task force project was launched in 2012 to evaluate the status of cleft care in India and develop strategies to provide comprehensive cleft care through a dynamic multidisciplinary and multidimensional tool. ICMR task force project (2012–14) was conducted in Delhi, and the National Capital Region reported that at least 50% of studied cases had complex and multiple treatment needs. The needs identified were related to surgical, orthodontic, dental, ENT and Speech, rehabilitation of mutilated dentition, and various psychological disturbances among patients with Cleft anomalies. Aim: The aim of this study was to develop and test web-based application to create a system for national data of patients with cleft anomalies and digitize the patient records in a standardized preagreed format. Methods and Results: Corresponding to contemporary digital technologies and evolutionary improvements in data collection, web-based data collection instrument, including text, photographs, X-rays, and audio files, was considered the most appropriate. The experts from varied domains in consultation with ICMR and National Informatics Centre evolved a web-based data collection instrument which is named the “IndiCleft tool.” The tool has been tested and used over the years and is presently being upgraded to dynamic version for a national data and patient care registry. Conclusion: The present article describes the process of the development of a “dynamic” web-based data collection instrument. The IndiCleft tool is the national resource on cleft data in India.

Aim of the Study: The aim of this study is to retrospectively analyze the surgical outcomes of patients who were treated for Tessier 7 cleft deformity, over a period of 10 years and compare the two cutaneous closure methods used for the treatment of the same in our cleft center. Patients and Methods: This is a retrospective audit of all the patients who had undergone Tessier 7 cleft repair from 2010 to 2020 in our center. Relevant details from recorded case histories were extracted and the surgical technique (Straight-line closure or Z-plasty technique) used for the closure of each case was analyzed. The benefits and drawbacks of linear closure and geometric closure methods were also studied. The preoperative, postoperative, and follow-up records and photographs were assessed for the results. Results: Of the 40 patients, 23 patients had skin closure done using Z-plasty technique while 17 patients had the closure done using the straight-line method. The average age at repair was 10.6 months and the follow-up period was unto 1.5 years. Visual Analog Scale and Scar assessment scales revealed Z-plasty skin closure scars were more conspicuous than straight-line closure ones. Lateral migration of the commissure was not an evident finding. Conclusion: Z-plasty or W-plasty can be avoided in repair of transverse facial clefts. Closure of the orbicularis oris muscle is the critical step to provide a counterforce to the contraction of the cutaneous scar and no lateral migration or hypertrophic scarring is present after straight-line cutaneous closure.

Roberts syndrome is a rare congenital anomaly which was described by John Roberts in 1919, also known as pseudothalidomidic or phocomelia. Characterized by skeletal deformities, in particular symmetric reduction of the extremities and craniofacial anomalies such as cleft lip and palate and cranio synostosis presenting more frequently, as well as bilateral symmetric short neck tetraphocomelia or hypomelia and brachydactyly. Its prevalence is unclear. As of now, 150 cases of different racial and ethnic origins have been reported in the literature. The diagnosis of Roberts Syndrome is established by cytogenetic and molecular analysis which show a phenomenon known as premature separation of the centromere or heterochromatin repulsion, constituting the main marker for Roberts syndrome. For any child with limb and craniofacial bone malformations, this syndrome should be considered as a differential diagnosis. The purpose of this study is to present a clinical case of a 2-year-old patient with Roberts Syndrome with a marked craniofacial anomaly.

Isolated deficiency of gonadotropin-releasing hormone is a heterogeneous disorder with wide genetic and clinical overlap. It mainly presents as hypogonadotropic hypogonadism (HH). HH associated with anosmia is known as Kallmann syndrome (KS), while its normosmic variant is called normosmic idiopathic HH. However, it is associated with several nonreproductive features including dental defects. Fibroblast growth factor receptor 1 gene mutation, which is seen in the autosomal dominant form of idiopathic HH (HH 2), has often been linked to the associated dental abnormalities and orofacial defects; however, no literature exists for its association with anosmin-1 (ANOS1) gene mutation which is found in the X-linked form of HH (KS). ANOS1 gene was earlier known as KAL1 (Kallmann syndrome 1) gene, and encodes for the extracellular matrix protein called anosmin. Hence, we report a case of idiopathic HH (KS) so as to delineate the possible role of ANOS1 gene in dental/orofacial development. This can help prioritize gene screening and also provide scope for further genetic studies required to prove such association.

Cleft palate is a developmental craniofacial anomaly with multiple aspects. With time, the understanding of its anatomy and its pathophysiology evolved leading to simultaneous improvement in surgical techniques. The timing of its surgical as well as nonsurgical management has great importance in determining the functional outcome. The priorities in its management and the techniques are quite well understood now though some difference of opinion still exists. In these series of articles, we aim to discuss its various aspects in detail, beginning with an emphasis on its history and anatomy.

Orofacial anomalies are common of which the most common orofacial deformity is cleft lip and palate (CLP), which affects one out of every 700–1000 infants globally. The management team should provide a thorough diagnosis, treatment plan, and follow-up. A cleft team often includes orthodontists, maxillofacial surgeons, plastic surgeons, pediatric dentists, prosthodontists, speech therapists, audiologists, psychologists, and pediatricians. Prosthetic rehabilitation of the CLP patients starts right from birth to adulthood. It varies from the naso alveolar molding of cleft segments before surgery and expansion of maxillary arch before the secondary alveolar bone grafting to the placement of fixed partial dentures or removable partial dentures for the establishment of better esthetics. A soft-tissue bulb prosthesis can also be given to children with velopharyngeal insufficiency to improve their speech. This article elucidates prosthetic rehabilitation techniques and modalities for patients with CLP.