Prevalence of Tympanostomy Tube Placement in Relation to Cleft Width and Type.

OBJECTIVES/HYPOTHESIS: The prevalence of tympanostomy tube surgery (TTS) in patients with a cleft deformity was investigated, in relation to cleft width and cleft type. STUDY DESIGN: Retrospective review of medical health records. METHODS: Retrospective review of medical health records. Seventy-eight patients with non-syndromic cleft deformity of the palate and/or alveolus and lip between 2003 and 2017 were investigated. All available medical documents were analyzed. The study group was divided into subgroups: 1) patients with isolated cleft palate (CP) and patients with a cleft palate with cleft lip and alveolus (CLP). 2) According to Veau's classification (I-IV), further subgroups were defined. Cleft width was measured using plaster cast models. RESULTS: TTS was performed in 55% of the patients (n = 43). Considering Veau's classification, TTS was conducted as follows: Veau I 65.2% (n = 15/23), Veau II 55.0% (n = 11/20), Veau III 47.6% (n = 10/21), and Veau IV 50.0% (n = 7/14). Cleft classifications, maxillary arch width, and absolute/relative cleft width had no statistical impact on TTS occurrence. Although no significant correlation could be found, patients in our study group with CP (Veau I and II) underwent TTS more often (60.5%, n = 26/43) than patients with CPL (Veau III and IV; 48.6%, n = 17/35) during a three-year follow-up. CONCLUSION: None of the cleft characteristics examined had a significant impact on the proportion of patients who received TTS. Nevertheless, patients with lower Veau classification and CP received tympanostomy tubes more often. Therefore, otolaryngologists and pediatricians treating children with cleft palate should maintain a high level of suspicion for chronic middle ear effusion, even in patients with small clefts. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:E2764-E2769, 2021.

Post-operative intravenous fluid administration for infant cleft surgery: An observational study.

OBJECTIVE: To investigate post-operative intravenous fluid administration and length of stay in a single site cleft centre. Previous publications have linked increased length of stay following primary cleft surgery to the administration of intravenous fluids post-operatively. MATERIALS AND METHODS: One hundred and ten primary cleft operations were conducted from May 2015 to April 2016 on non-syndromic infants. At West Midlands Cleft Centre, there are three cleft surgeons and 20 paediatric anaesthetists. This observational study compares classification of cleft type and surgical procedure with intravenous fluid administration, time taken to tolerate oral feeding, and length of stay. RESULTS: Cleft lip repair infants had the shortest length of stay in hospital, 25 h 8 min (median) and 33% had intravenous fluids. The palate repair only and lip and palate repair children had a median length of stay of 29 h 20 min and 29 h 0 min respectively, A total of 79% and 76% of these groups had intravenous fluids administered. Cleft lip repair infants fed in significantly less time than palate alone or lip and palate operations (p values 0.00 and 0.03, respectively). CONCLUSION: Cleft lip repair only infants feed well post-operatively and rarely require intravenous fluids. Infants having cleft repair involving the palate are slower to feed orally, and may require fluids due to poor oral intake. Intravenous fluids following lip repair is associated with longer hospital stay. We suggest intravenous fluids may not be needed routinely following cleft lip repair, but should always be considered following repair involving the palate.

Strategic management of the minor-form and microform cleft lip: A long-term outcome assessment.

OBJECTIVE: The treatment of the lesser form cleft lip deformity (i.e., minor-form, microform, and mini-microform) is challenging to achieve patient satisfaction. There are no studies investigating how treatment outcomes balance patients' expectations and satisfaction with surgical or non-surgical care relative to the initial nasolabial findings. METHODS: Based on Mulliken's classification, consecutive records of patients with the lesser-form cleft lip from 1990 to 2015 were selected for analysis. Demographics, management, and revisions were reviewed. A panel analysis was performed based on the ratings from both professionals and non-professionals using patients' photographs. FACE-Q questionnaires were used to compare patient/parent-reported satisfaction to the normal controls. RESULTS: A total of 135 patients were included. All of the minor-form (23/23), 89% (58/65) of the microform, and 62% (29/47) of the mini-microform patients underwent surgery. Fifty-two percent (15/29) of the mini-microform patients, who underwent surgery, showed no significant improvement. Post-operative upper lip scars and remaining nasal asymmetry were the most common concerns in the treatment group. However, the FACE-Q results showed that patients experienced a statistically significant improvement in their overall facial appearance and social function in their respective groups. CONCLUSIONS: This study showed that differences exist in the perception of facial esthetics between the surgeon and the patient/parent. To achieve optimal individual treatment outcome and patient satisfaction, an awareness of the differences and treatment selection for patients are critical in surgical planning. This understanding and an open detailed discussion of overall long-term outcomes help in the management of patient expectations.

Correlation between Type of Clefting and the Incidence of Otitis Media among Children with Lip and/or Palate Clefts.

INTRODUCTION: Otitis media is a frequent condition among children with high morbidity. Orofacial clefts are undoubtedly one of the most well-established risk factors for otitis media during childhood. The clinical spectrum of disease in orofacial clefting is broad according to the subtype of malformation. This study aims to correlate the occurrence of otitis media among lip and/or palate cleft children with clinical and epidemiological parameters, in particular with the subtypes of malformation diagnosed. METHODS: This is a clinical, retrospective, case-control type of study. Epidemiological and clinical data were obtained from medical records of children born between 2005 and 2008 and attending a multidisciplinary center for cleft patients. RESULTS: 53% of the patients had registers of middle ear disorder during follow-up, and secretory otitis media was the most frequently diagnosed condition. Five children (1.39%) had chronic otitis media during the study period. Those patients with malformations including involvement of structures located posteriorly to the incisive foramen were more frequently diagnosed with otitis media than those with isolated pre-foramen cleft (p value < 0.001, odds ratio: 5.33). Gender and bilateral malformations did not correlate with increased occurrence of middle ear disease (p value > 0.05). CONCLUSION: Otitis media is frequent among lip and/or palate cleft children, although the grade of middle ear involvement seems to vary widely within this population. Post-foraminal malformations are clearly associated with increased incidence of otitis media, as well as with more severe diseases.

[Application of mathematical function model in morphological description and classification of bone defect in cleft palate cases].

Objective: To establish the functional models based on various shapes of bone defects in isolated cleft palate patients and to classify the bone defects of the cleft palate cases using different functional curves. Methods: Tracking back from January 2018 to December 2018, 143 patients with cleft palate (Veau Ⅰ & Ⅱ) treated in Department of Oral and Maxillofacial Surgery, Peking University School and Hospital of Stomatology, were included (age of 7 months to 25 years, average age 1.6 years, median age 1.0 year, the male to female ratio was 0.57∶1).The pre-operative (CT) data sets were reconstructed into a three dimensional model to produce a direct image of the cleft palate. According to the shapes of the bone defect, cleft palate cases were divided into three types, i.e."" shape, inverted "V" shape and inverted "U" shape, and then the cases were assessed and reviewed by five examiners independently. Using Microsoft Excel software, the curves of the bone defect were placed in the coordinate system for mathematical modeling, including exponential function (y=ae(bx)), linear function (y=ax+b) and logarithm function (y=alnx+b). The function of the maximum fit R(2) value was used as the final fit curve. Statistical analysis was performed in four aspects: ① The reliability and feasibility of the curve fitting of the functions; ② The calculation of the composition ratio of the types of bone defect; ③Analyzing the correspondence between the subjective judgment results and fitting function curves; ④ The R(2) values of three types of functional curves homologous to different morphological types, and the data were tested by variance analysis and P values were shown. Results: Among the 143 patients with cleft palate, the "" shaped defect accounted for 18% (26/143), the inverted "V" shaped defect accounted for 31% (44/143), and the inverted "U" shaped defect accounted for 51% (73/143). The coincidence rate of the "" shaped defect with the exponential function (y=ae(bx)) was 96%, the coincidence rate of the inverted "V" shaped defect with the linear function (y=ax+b) was 82%, and the coincidence rate of the inverted "U" shaped defect with the logarithmic function (y=alnx+b) was 93%. The differences in R(2) values amongst the three groups were statistically significant (P<0.05). Conclusions: The shapes of bone defects of the incomplete cleft palate can be described by functional curve models which include exponential, linear and logarithmic functions and can be used to classify and lay the foundation for digital classification of cleft lip and palate cases.

A systematic genetic analysis and visualization of phenotypic heterogeneity among orofacial cleft GWAS signals.

Phenotypic heterogeneity is a hallmark of complex traits, and genetic studies of such traits may focus on them as a single diagnostic entity or by analyzing specific components. For example, in orofacial clefting (OFC), three subtypes-cleft lip (CL), cleft lip and palate (CLP), and cleft palate (CP) have been studied separately and in combination. To further dissect the genetic architecture of OFCs and how a given associated locus may be contributing to distinct subtypes of a trait we developed a framework for quantifying and interpreting evidence of subtype-specific or shared genetic effects in complex traits. We applied this technique to create a "cleft map" of the association of 30 genetic loci with three OFC subtypes. In addition to new associations, we found loci with subtype-specific effects (e.g., GRHL3 [CP], WNT5A [CLP]), as well as loci associated with two or all three subtypes. We cross-referenced these results with mouse craniofacial gene expression datasets, which identified additional promising candidate genes. However, we found no strong correlation between OFC subtypes and expression patterns. In aggregate, the cleft map revealed that neither subtype-specific nor shared genetic effects operate in isolation in OFC architecture. Our approach can be easily applied to any complex trait with distinct phenotypic subgroups.

Craniofacial cephalometric morphologies in different cleft types: a retrospective cross-sectional study of 212 patients.

OBJECTIVE: The aim of this study was to evaluate and compare the craniofacial cephalometric morphologies among different cleft types in a Spanish population. METHODS: A retrospective cross-sectional study was carried out on 212 patients. The patients were subdivided into four groups according to their cleft types: unilateral cleft lip and palate; bilateral cleft lip and palate; cleft lip; and cleft palate. Angular and linear cephalometric measurements were taken on lateral radiographs. RESULTS: Unilateral cleft lip and palate was associated with a dolichofacial growth pattern, skeletal Class III with correct maxillary position, and lingual incisor inclination. Bilateral cleft lip and palate was associated with a mesofacial growth pattern, skeletal Class I with protruded maxillary position, and lingual incisor inclination. Cleft palate was associated with a mesofacial growth pattern, skeletal Class III with correct maxillary position, and lingual incisor inclination. Cleft lip was associated with a brachyfacial growth pattern, skeletal Class I with protruded maxillary position, lingual upper incisor inclination, and corrects lower incisor inclination. Significant correlations were observed between cleft types and their craniofacial cephalometric measurements. CONCLUSIONS: The present information can be used for the determination of orthodontic treatment and even future orthognathic surgery planning, a requirement in most cleft patients.

Increased Risk for Neurodevelopmental Disorders in Children With Orofacial Clefts.

OBJECTIVE: Children with orofacial clefts (OFC) may have an increased risk of poor mental health. This study aimed to investigate the risk of psychiatric diagnoses in individuals with OFC, stratified by cleft type. METHOD: A nationwide register-based cohort of all individuals born with nonsyndromic OFC in Sweden between 1973 and 2012 (n = 7,842) was compared to a matched cohort (n = 78,409) as well as to their unaffected siblings (n = 9,637). The risk of psychiatric diagnoses, suicide attempts, and suicides was examined by crude and adjusted Cox regression models. Effect modification by sex was investigated with interaction terms in the models. RESULTS: Children with cleft lip (CL) had a significantly higher risk of any psychiatric disorder, intellectual disability, and language disorders; children with cleft lip and palate (CLP) had, in addition, an increased risk of autism spectrum disorder (ASD). Children with cleft palate only (CPO) had risk increases for the same diagnoses as children with CL and CLP, but with higher hazard ratios, and also for psychotic disorders, attention-deficit/hyperactivity disorder (ADHD), and other behavioral or emotional disorders in childhood. Sex stratification indicated higher risk increases among females in CL and CLP but not in CPO. Siblings without OFC were less likely to be diagnosed with any psychiatric disorder, intellectual disability, language disorder, ASD, or ADHD compared to their siblings with OFC. CONCLUSION: Children with nonsyndromic clefts had a significantly higher risk of neurodevelopmental disorders. This risk is unlikely to be explained by familial influences such as inherited genetic or shared environmental factors.

Microforma de fisura labial superior incompleta unilateral: reporte de un caso / Microform of unilateral incomplete upper cleft lip: report of a case

La microforma de fisura labial es una expresión de la fisura labial superior incompleta que se caracteriza por un surco o depresión cutáneomuscular a lo largo del margen del filtrum, asimetría o discontinuidad del margen mucocutáneo a nivel del pico del arco de Cupido, discontinuidad de la porción superior del músculo orbicular de la boca y deformidad menor del ala nasal. Es una condición que por su aparente poca gravedad y aparente fácil reparación, así como por su inusual presentación clínica, los reportes literarios sobre ella son escasos. Se presenta una revisión bibliográfica al respecto y se comunica un caso clínico como aporte a la literatura (AU)

The microform cleft lip is an expression of incomplete superior cleft lip is characterized by a groove or depression cutaneous and muscular along the margins of the philtrum, asymmetry or discontinuity mucocutaneous margin at the peak of Cupid's bow, discontinuity in the upper portion of the orbicularis oris muscle and minor deformity of the nasal ala. It is a condition for its apparent low gravity and apparent easy to repair as well as for its unusual clinical presentation, literature reports are scarce. We present a literature review and communicate about a case as a contribution to literature (AU)

SPINA classification of cleft lip and palate: A suggestion for a complement.

Spina's classification uses the incisive foramen as an anatomic reference to define groups I, II, and III. In some cases, the morphological manifestation of the cleft arises simultaneously pre- and postforamen, but without communicating. Considering that group I refers to isolated clefts of the primary palate and group III includes isolated clefts of the secondary palate, the authors suggest the classification group IIa for the association of these two occurrences in the same patient, thus associating two classifications. The original structure proposed by Spina is maintained and simply complemented and updated to Spina-A classification.

Integrated assessment of differentially expressed plasma microRNAs in subtypes of nonsyndromic orofacial clefts.

BACKGROUND: Orofacial clefts include cleft lip only (CLO), cleft palate only (CPO), and cleft lip with palate (CLP). Previously, we reported the expression profile of plasma microRNAs in CLO, CPO, and CLP, respectively. However, the interaction of each subtype remains poorly investigated. METHODS: In this study, we integrated the expression profiles of plasma miRNAs in these 3 subtypes, and assessed the distinct and overlapping dysregulated miRNAs using Venn diagrams. Their respective target genes reported in the literature were further analyzed using pathway analysis. RESULTS AND CONCLUSION: The results showed that distinct or overlapping signaling pathways were involved in CLO, CPO, and CLP. The common key gene targets reflected functional relationships to the Wnt, Notch, TGF-beta, and Hedgehog signaling pathways. Further studies should examine the mechanism of the potential target genes, which may provide new avenues for future clinical prevention and therapy.

Dentoskeletal effects of facemask therapy in skeletal Class III cleft patients with or without bone graft.

INTRODUCTION: The association between maxillary protraction and bone graft in patients with cleft lip and palate remains unclear. The purpose of this study was to investigate whether a secondary alveolar bone graft influences dentoskeletal effects of facemask therapy in unilateral cleft lip and palate patients with a skeletal Class III relationship. METHODS: In this prospective nonrandomized clinical trial, 61 consecutive boys with unilateral cleft lip and palate and skeletal Class III malocclusion were divided into 3 groups: grafted facemask group (n = 21), ungrafted facemask group (n = 20), and untreated control group (n = 20). Sixteen dentoskeletal measurements on lateral cephalometric radiographs were compared before and after therapy or observation with 1-way analysis of variance or the Mann-Whitney U test. RESULTS: After facemask therapy, the grafted group showed a statistically significantly greater advancement of Point A (S-Vert-A, 4.18 ± 1.94 mm; SNA, 3.51° ± 2.21°) than did the ungrafted group (S-Vert-A, 2.64 ± 1.58 mm; SNA, 1.92° ± 1.05°). Furthermore, significant SNB changes were found in the grafted group when compared with those in the ungrafted group (-0.38° ± 1.77° vs -1.69° ± 1.34°; P <0.05). The changes in the mandibular plane angle (MP-SN, MP-FH) in the grafted group were less pronounced than in the ungrafted group by approximately 2° (P <0.05). Flaring of the maxillary incisors was more pronounced in treated subjects than in untreated subjects. The mandibular incisors proclined in both grafted (1.54° ± 4.21°) and control (0.97° ± 3.71°) patients, and were retroclined in the ungrafted group (-2.13° ± 3.68°). CONCLUSIONS: Facemask therapy performed after an alveolar bone graft produced more anterior maxillary migration (90%) and less pronounced mandibular clockwise rotation (10%) than those in the ungrafted group (50%, 50%, respectively).

Subphenotyping and Classification of Cleft Lip and Alveolus in Adult Unoperated Patients: A New Embryological Approach.

OBJECTIVE: Previously, a new embryological classification was introduced subdividing oral clefts into fusion and/or differentiation defects. This subdivision was used to classify all subphenotypes of cleft lip with or without alveolus (CL±A). Subsequently, it was investigated whether further morphological grading of incomplete CLs is clinically relevant, and which alveolar part is deficient in fusion/differentiation defects. DESIGN: Observational cohort study. SETTING: Three hundred fifty adult unoperated Indonesian cleft patients presented themselves for operation. Cephalograms, dental casts, and intraoral and extraoral photographs-eligible for the present study-were used to determine morphological severity of CL±A. PATIENTS: Patients with unilateral or bilateral clefts of the primary palate only were included. MAIN OUTCOME MEASURES: Clefts were classified-according to developmental mechanisms and timing in embryogenesis-as fusion and/or differentiation defects. Grades of incomplete CLs were related to the severity of alveolar clefts (CAs) and hypoplasia, and permanent dentition was used to investigate which alveolar part is deficient in fusion/differentiation defects. RESULTS: One hundred eight adult patients were included. All subphenotypes-96 unilateral and 12 bilateral clefts-could be classified into differentiation (79%), fusion (17%), fusion-differentiation (2%), or fusion and differentiation (2%) defects. The various grades of incomplete CLs were related to associated CAs and hypoplasia, and all alveolar deformities were located in the premaxillae. CONCLUSIONS: This study showed that all CL±A including the Simonart bands can be classified, that further morphological grading of incomplete CLs is clinically relevant, and that the premaxilla forms the deficient part in alveolar deformities.

Prevalence of orofacial clefts and risks for nonsyndromic cleft lip with or without cleft palate in newborns at a university hospital from West Mexico.

We determined the overall prevalence of typical orofacial clefts and the potential risks for nonsyndromic cleft lip with or without cleft palate in a university hospital from West México. For the prevalence, 227 liveborn infants with typical orofacial clefts were included from a total of 81,193 births occurred during the period 2009-2016 at the "Dr. Juan I. Menchaca" Civil Hospital of Guadalajara (Guadalajara, Jalisco, Mexico). To evaluate potential risks, a case-control study was conducted among 420 newborns, including only those 105 patients with nonsyndromic cleft lip with or without cleft palate (cases), and 315 infants without birth defects (controls). Data were analyzed using multivariable logistic regression analysis expressed as adjusted odds ratio with 95% confidence intervals . The overall prevalence for typical orofacial clefts was 28 per 10,000 (95% confidence interval: 24.3-31.6), or 1 per 358 live births. The mean values for the prepregnancy weight, antepartum weight, and pre-pregnancy body mass index were statistically higher among the mothers of cases. Infants with nonsyndromic cleft lip with or without cleft palate had a significantly higher risk for previous history of any type of congenital anomaly (adjusted odds ratio: 2.7; 95% confidence interval: 1.4-5.1), history of a relative with cleft lip with or without cleft palate (adjusted odds ratio: 19.6; 95% confidence interval: 8.2-47.1), and first-trimester exposures to progestogens (adjusted odds ratio: 6.8; 95% CI 1.8-25.3), hyperthermia (adjusted odds ratio: 3.4; 95% confidence interval: 1.1-10.6), and common cold (adjusted odds ratio: 3.6; 95% confidence interval: 1.1-11.9). These risks could have contributed to explain the high prevalence of orofacial clefts in our region of Mexico, emphasizing that except for history of relatives with cleft lip with or without cleft palate, most are susceptible of modification.

Cleft Skeletal Asymmetry: Asymmetry Index, Classification and Application.

OBJECTIVE: To quantitatively measure the extent of 3D asymmetry of the facial skeleton in patients with unilateral cleft lip and palate (UCLP) using an asymmetry index (AI) approach, and to illustrate the applicability of the index in guiding and measuring treatment outcome. METHOD: Two groups of subjects between the ages of 15 and 20 who had archived CBCT scan were included in this study. Twenty-five patients with complete UCLP were compared with 50 age-matched noncleft subjects. The CBCT scans were segmented and landmarked for 3D anthropometric analysis. An AI was calculated as a quantitative measure of the extent of facial skeletal asymmetry. RESULTS: For the control group, the AI ranged from 0.72 ± 0.47 at A point to 4.77 ± 1.59 at Gonion. The degree of asymmetry increased with the increasing laterality of the landmark from the midsagittal plane. In the UCLP group, the values of AI significantly increased compared to the control group at nearly all measured landmarks. The extent of the asymmetry to involve the upper, middle, and lower facial skeleton varied widely with the individual patient with UCLP. CONCLUSION: The asymmetry index is capable of capturing the 3D facial asymmetry of subjects with UCLP and as a basis for classification of the extent of the asymmetry. We found the index to be applicable in surgical planning and in measuring the outcome in improving the symmetry in patients who have undergone orthognathic surgery.

Secondary Cleft Nasolabial Deformities: A New Classification System for Evaluation and Surgical Revision.

INTRODUCTION: Secondary or residual cleft lip and nasal deformities following primary unilateral or bilateral cleft lip repair are common. Many classification systems have been proposed to describe congenital cleft lip and palate deformities before repair. This article proposes a one-of-a-kind classification system for residual cleft deformities and describes its application to 136 cleft lip revision cases from cleft outreach missions worldwide. METHODS: Patients' demographics and deformities were classified preoperatively, and a database of the classification was created. Postoperatively, the type of surgery performed was added to the database and comparison was done using an independent t test. RESULTS: Kappa coefficient was 0.92 and showed excellent agreement between the type assigned preoperatively to the patient and the type of procedure done. CONCLUSIONS: This system proves to provide good descriptions of the deformities, is user friendly, facilitates the planning of the corrective surgical procedure, and enhances the communicative lingo between surgeons and members of cleft multidisciplinary care teams. It is broadly applicable in outreach missions with limited resources and cleft referral centers with considerable load.

A numerical classification system for cleft lip and palate in the dog.

An easy-to-use classification that enables an accurate record of canine cleft lip and palate is fundamental for effective communication between professionals and researchers and optimal use of published information. Here we present how a classification system for human cleft lip and palate can be used to register spontaneous cases in dogs, highlighting its advantages. This system is based on four topographic areas with a numerical representation of the severity of the cleft in each area. The use of spontaneous cases has the advantage of providing clefts that are naturally similar to their human counterparts for surgical, genetic and genomic studies and, furthermore, will reduce the need for experimental models of this condition.

Clinical Finding and Management of 12 Orofacial Clefts Cases With Nevoid Basal Cell Carcinoma Syndrome.

Objective To study the phenotype and overview the clinical management on Cleft Lip and/or Palate (CL/P) with Nevoid Basal Cell Carcinoma Syndrome (NBCCS) patients in Peking University School and Hospital of Stomatology. Design Case series study. Main Outcome Measures To describe the clinical phenotype of 12 CL/P with NBCCS patients who fulfilled the diagnostic criteria as well as to explore clinical management. Results Seven cases (7/12, 58.33%) were classified as bilateral complete cleft lip and palate (BCCLP). Two cases (2/12, 16.67%) were classified as unilateral complete cleft lip and palate (UCCLP). Three cases (1/12, 8.33%) were classified as unilateral complete cleft lip (UCCL), submucosa cleft uvula (SCU), and bifid uvula (BU), respectively. The ratio of male/female was 9/:3. Keratocystic odontogenic tumors (KCOTs) were presented in all 12 cases. The most common site was the mandible region (12/12, 100%) followed by the maxilla region (7/12, 58.33%). The diagnostic age of 12 NBCCS with CL/P ranged from 11 to 42 years old (usually postponed to the occurring of KCOTs). The delayed diagnosis of NBCCS can be attributed to its complicated clinical manifestations. In some cases, the mutual effect between the surgical therapy of removing KCOTs and alveolar bone grafting made the team approach (TA) of CL/P more complicated. Conclusion CL/P may become important clinical phenotype in NBCCS. The type of cleft varied, with bilateral cleft lip and palate comprising above 50%. Larger sample sizes are needed to study and confirm this result. KCOTs, as one of the most common clinical feature of NBCCS, make the diagnosis delayed and the TA more difficult because of the occurring time and site. This compels us to improve the diagnostic criteria to make an early diagnosis and explore a better therapeutic protocol for CL/P.

Single stage correction of bilateral tessier 4 cleft.

Tessier facial clefts are among the rarest facial clefts reported in literature and many contradicting issues have always been rising over the management and surgical approaches involved during the craniofacial cleft repair. Among the craniofacial clefts Tessier no. 4 is an extremely rare facial anomaly, and there are very few evidence which clearly describe the surgical approaches and techniques. Often these type of craniofacial clefts yield very poor surgical results, and they require multidisciplinary sequential corrective surgeries. This article presents a rare case of an 18-month-old baby with bilateral Tessier no. 4 clefts and its successful rehabilitation.

Comprehensive and reliable classification system for primary diagnosis of cleft lip and palate.

OBJECTIVE: Due to the lack of a universally accepted classification system, we are aiming to introduce a modified comprehensive, precise and relatively simple classification system for primary diagnosis of cleft lip and palate. METHODS: The proposed classification is based on the Kernahan's striped Y diagram with more details in cleft extent and with the addition of severity scores to each cleft component. Clear definitions of cleft extents and severity degrees were described based on 400 consecutive primary cases. Two medical students were taught the classification then diagnosed photographs of 100 cases twice to test its reliability. RESULTS: The students' results were 11% and 13% wrong diagnoses for student 1 and 2 in the first time, 8% and 10% in the second time, respectively. The inter-rater reliability for the two students in the first and second time was 0.716 and 0.878, respectively. The intra-rater reliability for student 1 and 2 were 0.826 and 0.755 respectively. The average duration to diagnose a case was less than a minute. CONCLUSION: This classification is comprehensive and records many diagnostic variables with high reliability and precision.