Isolated Cleft Palate Research Articles

Ten-year experience of more than 35,000 orofacial clefts in Africa.

BackgroundSurgical correction of orofacial clefts greatly mitigates negative outcomes. However, access to reconstructive surgery is limited in developing countries. The present study reviews epidemiological data from a single charitable organization, Smile Train, with a database of surgical cases from 33 African countries from 2001–2011.MethodsDemographic and clinical patient data were collected from questionnaires completed by the participating surgeons. These data were recorded in Excel, analyzed using SPSS and compared with previously reported data.ResultsQuestionnaires were completed for 36,384 patients by 389 African surgeons. The distribution of clefts was: 34.44% clefts of the lip (CL), 58.87% clefts of the lip and palate (CLP), and 6.69% clefts of the palate only (CP). The male to female ratio was 1.46:1, and the unilateral: bilateral ratio 2.93:1, with left-sided predominance 1.69:1. Associated anomalies were found in 4.18% of patients. The most frequent surgeries included primary lip/nose repairs, unilateral (68.36%) and bilateral (11.84%). There was seasonal variation in the frequency of oral cleft births with the highest in January and lowest by December. The average age at surgery was 9.34 years and increased in countries with lower gross domestic products. The average hospital stay was 4.5 days. The reported complication rate was 1.92%.ConclusionsWith the exception of cleft palates, results follow trends of worldwide epidemiologic reports of 25% CL, 50% CLP, and 25% CP, 2:1 unilateral:bilateral and left:right ratios, and male predominance. Fewer than expected patients, especially females, presented with isolated cleft palates, suggesting that limitations in economic resources and cultural aesthetics of the obvious lip deformity may outweigh functional concerns and access to treatment for females. A fewer than expected associated anomalies suggests either true ethnic variation, or that more severely-affected patients are not presenting for treatment. The epidemiology of orofacial clefting in Africa has been difficult to assess due to the diversity of the continent and the considerable variation among study designs. The large sample size of the data collected provides a basis for further study of the epidemiology of orofacial clefting in Africa.Electronic supplementary materialThe online version of this article (doi:10.1186/s12887-015-0328-5) contains supplementary material, which is available to authorized users.

Closure of huge palatal fistula in an adult patient with isolated cleft palate: a technical note.

Summary:Closure of huge palatal fistula surrounded by fully erupted permanent dentition in the adult patients with cleft is a challenge. Posteriorly based buccinator myomucosal flap is a neurovascular pedicled flap, with inherent nature of thin thickness, saliva secretion, and axial pattern blood supply. Vicinity of donor site to the palate and low donor-site morbidity are the other advantages. It is an ideal choice in such situation. In this article, the details of surgical technique and the effectiveness of this method are presented.

The Need for Orthognathic Surgery in Nonsyndromic Patients with Repaired Isolated Cleft Palate

To determine the frequency of need for orthognathic surgery among nonsyndromic patients with isolated cleft palate repaired during infancy at The Hospital for Sick Children in Toronto, Canada. Retrospective cohort study. PATIENTS with nonsyndromic isolated cleft palate born between 1970 and 1997 with available records including a lateral cephalometric radiograph taken at ≥15 years of age. PATIENTS who had undergone or were being prepared for orthognathic surgery were automatically counted as requiring surgery. For the remaining patients, lateral cephalometric radiographs were traced and analyzed. Arbitrarily set cephalometric criteria were used to identify the "objective" need for orthognathic surgery. Of the 189 patients identified with nonsyndromic isolated cleft palate and for whom records were available, 25 (13.2%) were deemed to require orthognathic surgery. Of the surgical cohort, 92% required surgical correction for a Class III malocclusion. Similar percentages of males and females required orthognathic surgery. An apparently greater proportion of patients of Asian background (18.5%) than of white background (10.6%) required surgery, but this difference was not significant (P = .205). The current results suggest that approximately one in eight patients at our institution with nonsyndromic isolated cleft palate requires orthognathic surgery. There is a tendency for this to be higher in patients of Asian descent and lower in patients of white descent. Variability in extent, severity, and phenotype of the cleft, which may be attributed largely to genetics, may play an important role in dictating the need for orthognathic surgery.

3D Facial Morphometry in Children with Oral Clefts

The aim of this study was to characterize three-dimensional (3D) facial morphological variation of children with cleft lip and palate compared to an age- and sex-matched control group. Cross-sectional, case-controlled observational morphometric study. Subjects were 103 children aged 8 to 12 years old with cleft lip and palate-40 with unilateral cleft lip and palate, 23 with unilateral cleft lip and alveolus, 19 with bilateral cleft lip and palate, and 21 with isolated cleft palate (ICP)-and 80 sex- and age-matched control subjects. Subjects were scanned using 3D stereophotogrammetry. Thirty-nine landmarks were digitized for each scan, and the x, y, and z coordinates for each landmark were extracted. Linear and angular facial measurements were computed. Multivariate analysis of covariance was undertaken to detect significant differences in facial morphometry between the groups. Statistically significant differences (P < .05) were observed between all groups. The greatest morphometrical impact was seen in those groups where both lip and palate were affected and repaired; the group with ICP was the most similar to the control subjects. Significant differences were seen in 3D soft-tissue measurements, mainly in the nasolabial region, between the cleft groups where the lip is affected and the ICP and control groups.

Isolated versus Pierre Robin Sequence Cleft Palates: Are They Different?

To determine if there are any differences in size between isolated cleft palates (CPs) and those associated with Pierre Robin (PR) sequence. Prospective case series. Tertiary care hospital. From 1993 to 2011, nonsyndromic isolated CP and PR sequence cases were classified as severe if the patients had respiratory or feeding difficulties. While patients were under general anesthesia, seven anatomical cleft parameters were prospectively measured in the operating room at the time of palatoplasty. Fifty-seven patients without the PR sequence and 36 patients with the PR sequence were enrolled. Within the PR group, 61% of cases were mild and 39% were severe. A larger soft palate width was found to be statistically significant in a comparison of the severe PR cases with the isolated clefts (P < .005) and mild PR (P < .05), respectively. For the hard palate width, a statistically significant difference was found in a comparison of the narrower isolated cleft cases with the mild PR (P < .05) and the severe PR cases (P < .05), respectively. A shorter cleft length was found to be statistically significant in isolated clefts versus both the clefts of the mild PR (P < .05) and the severe PR cases (P < .05). The patients with PR sequence presented statistically significant different cleft characteristics. The increased width of the cleft at the soft palate level showed the greatest correlation with increased airway and feeding problems.

Updating the Epidemiology of Isolated Cleft Palate

Updating the Epidemiology of Isolated Cleft Palate

Changing strategy and implementation of a new treatment protocol for cleft palate surgery in “Maria Sklodowska Curie” (MSC) Children's Hospital, Bucharest, Romania

In “Maria Sklodowska Curie” (MSC) Children's Hospital, Bucharest, Romania, cleft palate repair has been performed according to von Langenbeck since 1984. The speech was good in most patients but wide clefts had a high percentage of fistulas, abnormal speech due to short length and limited mobility of the soft palate. In 2009, the protocol was changed to Gothenburg Delayed Hard Palate Closure, (DHPC) technique. The present evaluation was performed to study the implementation of this technique. One hundred and sixty-eight patients with cleft palate were admitted, 89 isolated cleft palate (ICP), 53 unilateral (UCLP) and 26 bilateral (BCLP). In these, 228 surgical interventions were performed. Soft Palate Repair (SPR) and Hard Palate Repair (HPR) were performed with the DHPC procedure. The transfer to this technique was successfully performed in three steps: one team visit to Gothenburg by a surgeon from MSC and two visits by surgeons from Gothenburg to the MSC. Patients with SPR and HPR were operated on without major complications and there were no differences in results between Gothenburg surgeons and MSC surgeons. The interventions with SPR and HPR technique were proven to be easy to teach and learn and successfully performed without major complications. For cleft patients at MSC hospital it has meant earlier surgery, less re-operations and complications. This report shows a successful change of strategy for palatal repair with improved outcome regarding surgery. In future, speech and growth will be followed on a regular basis and will be compared with results from the Gothenburg Cleft Team.

Updating the Epidemiology of Isolated Cleft Palate

Sir: The report by Tanaka et al.1 on the epidemiology of isolated cleft palate raises several issues, the first one being the use of the term “isolated.” It would appear that the authors mean isolated with respect to the absence of a cleft lip, whereas to most workers in the field the term isolated means no other malformation, syndrome, sequence, or cytogenetic abnormality in association with cleft palate. Other issues include the fact that no sources are given for the data in their table, the lumping together of countries, and the short study term of 5 years. From the rates supplied, it would appear that the authors do not report truly isolated cleft palate but include cleft palate with multiple anomalies, syndromes, sequences, and cytogenetic abnormalities. It appears that the international data were taken from the 2008 Annual Report from the International Clearinghouse for Birth Defects, Surveillance and Research, which has a data column for 2002 to 2006, corresponding to Tanaka et al.’s study years. Many countries, including the United States, do not have a single national system. Those with highly reliable national systems are smaller countries with a long history of public health programs, particularly in Europe and especially the Nordic countries, whereas countries such as Canada, Australia, and England are better served by provincial, state, or regional surveillance registries. It is not clear how the authors derived rates for France or Italy, as each country has four or more surveillance registries. Finally, we suggest that a 5-year study is too short for inferring that the rates of cleft palate are increasing. In Alberta, using data for the 1997 to 2010 period, the cleft palate rate is 7.09 per 1000 total births (6.43 to 7.81) and is showing a decline not only for those without additional anomalies (true isolated) but also for those with multiple anomalies. The rates for those with syndromes remain stable (Fig. 1). The Alberta rate is very similar to two earlier long-term studies from British Columbia for 1952 to 19712 and 1952 to 1982.3 Other areas with long-term studies have shown no significant trend for cleft palate: The Netherlands from 1997 to 20064 and Victoria, Australia from 1983 to 2000.5Fig. 1: Cleft palate alone, Alberta 1997 to 2009. Total, isolated, multiple, and syndromes (including recognized syndromes and chromosomal abnormalities). (From Alberta Health and Wellness. Alberta Congenital Anomalies Surveillance System: Ninth Report 1997–2009. Edmonton, Alberta, Canada: Alberta Health and Wellness; 2012. Available at: http://www.health.alberta.ca/documents/Congenital-Anomalies-Report-8-2009.pdf.)ACKNOWLEDGMENTS The authors thank Judy Anderson and Tanya Bedard for manuscript assistance, and they are thankful for the support of Michael Sanderson and Larry Svenson from Alberta Health. The Alberta Congenital Anomalies Surveillance System is supported by the Surveillance Branch, Alberta Health and Wellness. DISCLOSURE The authors have no financial interest to declare in relation to the content of this communication. R. Brian Lowry, M.D., D.Sc. Alberta Congenital Anomalies Surveillance System Alberta Health, and Departments of Medical Genetics and Pediatrics University of Calgary and Alberta Children’s Hospital Research Institute Barbara Sibbald, B.N., M.Sc. Alberta Congenital Anomalies Surveillance System Alberta Health Calgary, Alberta, Canada

Alteration of Maxillary and Mandibular Growth of Adult Patients With Unoperated Isolated Cleft Palate

The objective of this study was to investigate the effects of cleft palate itself on the growth of maxilla and mandible. Fifty-two adult female patients with unoperated isolated cleft palate and 52 adult female individuals with normal occlusion were included in our study. Computer software was used for lateral cephalometry measurement. Manual measurement was performed for dental cast measurements, and sample t test analysis was applied to analyze the differences between the 2 groups using SPSS 17.0. The sella-nasion-subspinale point angle, subspinale-nasion-supramentale point angle, and maxillary arch length of the cleft group were significantly smaller than those of the control group (P < 0.01). Both maxillary and mandibular posterior dental arch widths of the cleft group were significantly larger compared with the control group (P < 0.01), whereas the sella-nasion-supramentale point angle, mandible arch length, palate height, and palate shelf inclination did not differ between the 2 groups. The measurements did not differ between the submucosal cleft and the overt cleft patients. Cleft palate itself has adverse effects on the maxilla growth with shorter maxillary arch length and wider posterior dental arch width.

The Craniofacial Morphology in Adult Patients with Unoperated Isolated Cleft Palate.

To address the effect of intrinsic factors on craniofacial growth by analyzing the craniofacial morphology of unoperated isolated cleft palate in Chinese adult. This study included 37 nonsyndromic isolated cleft palate and 39 age and gender matched non-clefts. Twenty-six cephalometric measurements were employed to evaluate the facial morphology. Independent samples T test and Mann-Whitney U were used for comparison. Significant difference was defined at 95% level. Data from this study showed patients with unoperated isolated cleft palate have a reduced maxillary sagittal length (ANS-PMP, A-PMP, P<0.05), a smaller ANB angle (ANB, P<0.05) and a retrusive ANS point (S-N-ANS, P<0.05; Ba-N-ANS, P<0.05). Measurements descripted position of maxilla (S-Ptm, P>0.05), depth of bony pharynx (Ba-PMP, P>0.05), anterior and posterior maxillary height (N-ANS, P>0.05; R-PMP, P>0.05) and mandible morphology (including linear measurements and angle measurements) did not show any significant difference between case and control groups. Patients with isolated cleft palate were characterized by maxillary retrusion. Mandible morphology and cranial basal morphology in isolated cleft palate showed no significant difference with nonclefts. Patients with isolated cleft palate are more vulnerable to cross bite than nonclefts. Intrinsic deficiencies did detrimental effect on maxilla sagittal length, but did no detrimental effect on maxilla position, mandible size and position.

Updating the Epidemiology of Isolated Cleft Palate

Updating the Epidemiology of Isolated Cleft Palate

3-Dimensional Computed Tomographic Analysis of the Pharynx in Adult Patients With Unrepaired Isolated Cleft Palate

This study investigated 3D differences of the pharynx in adult patients with unrepaired isolated cleft palate (ICP) versus normal adults using cone-beam computed tomography (CBCT). CBCT data of 32 adult patients with nonsyndromic unrepaired ICP and 30 normal controls were acquired. Image processing and analyses were performed using Mimics (Materialise NV, Leuven, Belgium). Linear, planar, and volumetric measurements and comparisons were performed between patients with ICP and controls. Interobserver and intraobserver reliabilities of 3D pharyngeal analysis were determined by the Pearson correlation coefficient. Statistical analyses comparing patients with ICP to normal adults were performed using independent-samples t test, with the significance threshold set at P = .05. Interobserver and intraobserver reliabilities were high. Pearson correlation coefficients ranged from 0.992 to 0.999 for interobserver measurements and from 0.994 to 0.999 for intraobserver measurements. Anterior height (P = .000), total depth (P = .003), and floor length (P = .034) of the bony nasopharynx; posteroanterior diameter of the pharyngeal airway at the palatal plane (P = .000); cross-sectional area of the pharyngeal airway at the palatal plane (P = .000); total volume (P = .031); volume above the palatal plane (P = .024); and the volume between the palatal plane and the plane of the most anterior point on the inferior margin of the outline of the body of the second cervical vertebra (P = .022) were larger in patients with ICP. This imaging study showed an enlarged nasopharynx in the sagittal plane and increased nasopharyngeal airway volume at the palatal plane in patients with ICP.

The nutrition status of mild form Pierre Robin sequence before cleft palate repair: an analysis of 34 cases

The objective of this study was to evaluate the nutrition status before cleft palate repair in patients with mild form isolated Pierre Robin Sequence (PRS). Detailed history and physical examination of 34 patients with mild form isolated PRS were compared with those of the control group with isolated cleft palate (ICP) at the age of 2 and 12 months. The height and weight of the 2 groups at 2 months were significantly different, whereas there was no significant difference at 12 months. The height and weight grades for PRS improved from 2 to 12 months. The feeding grades differed significantly at 2 months between the 2 groups, but showed no significant difference at 12 months. Patients with mild form isolated PRS had a nutrition status similar to patients with ICP at the age of 12 months. General conditions of mild form isolated PRS pose no problem with cleft palate repair at 12 months.

Craniofacial Morphology of Patients with Isolated Cleft Palate before Orthodontic Treatment

Craniofacial Morphology of Patients with Isolated Cleft Palate before Orthodontic Treatment

3D comparison of average faces in subjects with oral clefts

This prospective cross-sectional, case-controlled morphometric study assessed three dimensional (3D) facial morphological differences between average faces of 103 children aged 8-12 years; 40 with unilateral cleft lip and palate (UCLP), 23 with unilateral cleft lip and alveolus (UCLA), 19 with bilateral cleft lip and palate (BCLP), 21 with isolated cleft palate (ICP), and 80 gender and age-matched controls. 3D stereophotogrammetric facial scans were recorded for each participant at rest. Thirty-nine landmarks were digitized for each scan, and x-, y-, z-coordinates for each landmark were extracted. A 3D photorealistic average face was constructed for each participating group and subjective and objective comparisons were carried out between each cleft and control average faces. Marked differences were observed between all groups. The most severely affected were groups where the lip and palate were affected and repaired (UCLP and UCLA). The group with midsagittal palatal deformity and repair (ICP) was the most similar to the control group. The results revealed that 3D shape analysis allows morphometric discrimination between subjects with craniofacial anomalies and the control group, and underlines the potential value of statistical shape analysis in assessing the outcomes of cleft lip and palate surgery, and orthodontic treatment.

Highlights of Archives of Facial Plastic Surgery

Archives of Facial Plastic SurgeryVol. 14, No. 5 Free AccessHighlights of Archives of Facial Plastic SurgeryPublished Online:3 Sep 2012AboutSectionsPDF/EPUB Permissions & CitationsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail Split Hump Technique for Reduction of the Overprojected Nasal DorsumPeter J. F. M. Lohuis, MD, PhD, and colleagues describe their results using the split hump technique (SHT) in aesthetic rhinoplasty while emphasizing the additional advantage of this approach. The SHT allows for a more controlled reduction of the nasal dorsum with preservation of the entire upper lateral cartilage (ULC) during deprojection. This, in turn, enables more precise resection of the ULCs and less destabilizing of the key area. Ninety-seven patients who underwent rhinoplasty for aesthetic reasons using the SHT were evaluated for aesthetic contentment and functional satisfaction recorded by a questionnaire. Results demonstrate a significant subjective increase in both aesthetic and functional outcomes. The authors strongly recommend the use of the SHT to rhinoplasty surgeons.See page 346Reconstruction of Nasal Alar Defects in Asian PatientsDoo Hee Han, MD, PhD, and colleagues present a retrospective review regarding functional and aesthetic outcomes of methods used for nasal alar reconstruction in Asian patients while stating the criteria for selection of these methods. Although outcomes and criteria have been studied in Western populations, this is the first study in Asian people. The study consists of 17 patients, of whom 2 are described as representative cases. Objective and subjective aesthetic outcomes are evaluated along with subjective functional findings. Results show that the size and depth of the defect primarily affects the choice of reconstruction in Asian patients. The authors propose a working algorithm for reconstruction of nasal alar defects with respect to these 2 components.See page 312Impact of Cleft Width in Clefts of Secondary Palate on the Risk of Velopharyngeal InsufficiencyDerek J. Lam, MD, MPH, and colleagues question the relationship between cleft width and other risk factors with occurrence of velopharyngeal insufficiency (VPI). A total of 61 patients with isolated cleft palates (Veau type 2) are retrospectively surveyed for the presence of VPI, and the correlation with various parameters, including width and length of cleft, type of surgery, and age at which the operation was performed, are analyzed. Results show a significantly higher overall rate of VPI in subjects with a cleft width greater than 10 mm. The authors conclude that cleft width should be considered as a prognostic indicator of postoperative VPI, regardless of the Veau type.See page 360Role of Virtual Surgery in Preoperative Planning: Assessing the Individual Components of Functional Nasal Airway SurgeryJohn S. Rhee, MD, MPH, and colleagues demonstrate the use of computational fluid dynamics (CFD)-aided virtual surgery planning in evaluating the effects of individual components of functional nasal airway surgery. They accomplish this by evaluating nasal airway resistance and airflow allocation in preoperative and postoperative 3-dimensional digital nasal models created from computed tomographic images. Results prove that septoplasty and inferior turbinate reduction have the highest impact on reduction of nasal resistance, but the authors also state that contribution from nasal valve repair surgery is significant. They conclude with the proposition that CFD-aided surgery may be used for preoperative planning for a more successful outcome in patients with multifactorial anatomic nasal airway obstruction.See page 354Portrait of a Man Holding a Book, by Titian (Tiziano Vecellio) (about 1488 to 1576).See page 376This issue's Highlights were written by Ismet Emrah Emre, MD.FiguresReferencesRelatedDetails Volume 14Issue 5Sep 2012 InformationCopyright 2012 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.To cite this article:Highlights of Archives of Facial Plastic Surgery.Archives of Facial Plastic Surgery.Sep 2012.311-311.http://doi.org/10.1001/archfaci.2012.1014Published in Volume: 14 Issue 5: September 3, 2012PDF download

An Unusual Combination of Primordial Dwarfism with Isolated Cleft Palate

Primordial dwarfism is a rare anomaly with over 200 sub-types, which results in a relatively small body size in all stages of life, beginning at the intrauterine period. The life expectancy for these patients is around 30 years but very few survive their intra-uterine phase. Other features associated with primary dwarfism reported till date includes pigmentation of skin, cleft lip and palate, and semilobar holoprosencephaly. We report an unusual combination of primordial dwarfism associated with isolated cleft palate.

An Unusual Combination of Primordial Dwarfism with Isolated Cleft Palate

An Unusual Combination of Primordial Dwarfism with Isolated Cleft Palate

Prenatal ultrasound detection of cleft lip, or cleft palate, or both, in southern Sweden, 2006–2010

It has been possible to detect cleft lip (CL), with or without cleft palate (CLP), using ultrasound (US) since the beginning of the 1980s. The aim of this study was to assess the accuracy of prenatal diagnosis of cleft lip with or without cleft palate, and isolated cleft palate (ICP), in our catchment area. Screening protocols in the different US clinics in southern Sweden were also compared, as regards evaluation of the fetal face and prenatal diagnosis of CLP. Forty-four (31%) of the patients were diagnosed by prenatal US and 97/144 (67%) were diagnosed at birth. The detection rate was 44/102 (43%) if the ICP are excluded. The specificity was 100%. Among the prenatally diagnosed clefts, 25/44 (57%) were diagnosed before the gestational age of 20 weeks. In 19/44 (43%) of the cases the US diagnosis of cleft was accurate in the light of the postnatal outcome. All US departments in our catchment area follow the Swedish guidelines and offer one routine US examination during the second trimester between 18 and 20 weeks of pregnancy. In addition, many of the clinics offer an additional US examination during the third trimester. Our detection rate is similar to previous findings. The detection rates and the accuracy of the prenatal diagnosis can be improved. To achieve this, an increased focus on detecting clefts, standardising scanning plans, and rescans in case of incomplete facial views, are essential.

Diamond-Blackfan Anemia and Isolated Cleft Palate

Abstract Abstract Diamond Blackfan anaemia is associated with a variety of multiple congenital anomalies. The authors present a case of cleft palate occurring as the only anomaly in a child with Diamond Blackfan anaemia. Isolated cleft palate in association with Diamond Blackfan anaemia has not been previously reported in English literature. Therefore the authors believe this represents a new entity within the spectrum of associated congenital abnormalities in Diamond Blackfan anaemia. Keywords Diamond Blackfan anaemia, cleft palate, congenital anomalies.