OBJECTIVES: This study aimed to explore the relationship between alveolar cleft and secondary nasal deformity post unilateral cleft lip repair in adults. METHODS: A total of 27 patients aged 16-30 years old with unilateral secondary nasal deformity and alveolar cleft were included, 13 of whom underwent bone grafting. Spiral CT data of all preoperative and postoperative patients who had alveolar bone grafting were collected. Then, Mimics software was used for three-dimensional reconstruction to evaluate the correlation between the width, height, and volume of the alveolar cleft and those of the nasal deformity. The difference in nasal deformity before and after alveolar bone grafting was also explored. RESULTS: The width of the alveolar cleft was positively correlated with the difference in bilateral nostril floor width (P<0.05). As the effective depth of the alveolar cleft increased, the sub-alare inclination angle largened (P<0.05). However, no significant difference was found in the nasal deformity between before and after alveolar bone grafting. CONCLUSIONS Alveolar cleft is closely related to secondary nasal deformities post unilateral cleft lip repair, especially nasal floor deformities. Alveolar bone grafting benefits adult patients for the improvement of secondary nasal deformities post unilateral cleft lip repair.
Humans ; Adult ; Adolescent ; Young Adult ; Nose/surgery* ; Cleft Lip/surgery* ; Rhinoplasty/methods* ; Imaging, Three-Dimensional ; Treatment Outcome ; Cleft Palate/complications*

Objective To investigate the multiple correspondence of genetic and environmental risk factors with abnormal birth history and provide a scientific basis for improving the birth defects surveillance system and reducing the incidence of birth defects. Methods Data were collected from all the perinatal infants from 28-week-old fetuses to 7-day-old infants born in all the hospitals with obstetrical department in Xi'an from 2003 to 2015. Results A total of 1 236 937 perinatal infants were surveyed,including 10 619 with birth defects.The average incidence rate of birth defects was 0.86% (0.70%-1.15%).Multiple correspondence analysis showed that the women who had had 1 or 2 children with birth defects were associated with the history of spontaneous abortion,family history of birth defects,and history of exposure to toxic and harmful substances.The women who had had 3 or more children with birth defects showed stronger association with family history of birth defects.The birth defects in women with history of spontaneous abortion (257/10 619) was ranked in the order of congenital heart disease,polydactyly,neural tube defects,congenital hydrocephalus,cleft lip with cleft palate,and simple cleft lip.The birth defects in women who had given birth to children with birth defects (135/10 619) followed the order of cleft lip with cleft palate,anencephaly,hydrocephalus,neural tube defects,cleft lip,and talipes equinovarus. Conclusions Abnormal birth history is associated with family history of birth defects and history of exposure to environmental risk factors.Giving birth to three or more children with birth defects is highly correlated with the family history of birth defects.
Child ; Pregnancy ; Female ; Humans ; Cleft Lip/etiology* ; Cleft Palate/complications* ; Reproductive History ; Abortion, Spontaneous ; Neural Tube Defects/epidemiology* ; Risk Factors

OBJECTIVE: To analyze clinical and genetic features of a family affected with Van der Woude syndrome. METHODS: The umbilical cord blood of the proband and the peripheral blood of the parents were used for the whole exon sequencing to find the candidate gene.Peripheral blood of 9 members of the family were collected for Sanger sequencing verification, bioinformatics analysis and genotype-phenotype correlation analysis. RESULTS: The proband was diagnosed with cleft lip and palate by ultrasound. His father and grandmother had hollow lower lip and all other family members did not have the similar phenotype. A missense c.263A>G (p.N88S) mutation was found in exon 4 of gene in the proband, his father and his grandmother.The mutation was not found in other family members. CONCLUSIONS A missense c.263A>G (p.N88S) mutation in gene probably underlies the pathogenesis of Van der Woude syndrome in the family and the mutation has been firstly discovered in China.
Abnormalities, Multiple ; genetics ; China ; Cleft Lip ; complications ; diagnostic imaging ; etiology ; genetics ; Cleft Palate ; complications ; diagnostic imaging ; etiology ; genetics ; Cysts ; complications ; genetics ; Female ; Humans ; Interferon Regulatory Factors ; genetics ; Lip ; abnormalities ; Male ; Mutation ; Pedigree ; Ultrasonography

BACKGROUND: The postoperative speech outcomes of submucous cleft palate (SMCP) surgery are known to be poorer than those of other types of cleft palate. We attempted to objectively characterize the postoperative complications and speech outcomes of the surgical treatment of SMCP through a comparison with the outcomes of incomplete cleft palate (ICP). METHODS: This study included 53 SMCP patients and 285 ICP patients who underwent surgical repair from 1998 to 2015. The average age of the patients at the time of surgery was 3.9±1.9 years for the SMCP patients and 1.3±0.9 years for the ICP patients. A retrospective analysis was performed of the complications, the frequency of subsequent surgical correction for velopharyngeal dysfunction (VPD), and speech outcomes. RESULTS: In both the SMCP and ICP patients, no cases of respiratory difficulty, bleeding, or wound disruption were noted. Delayed wound healing and fistula occurred in 18.9% and 5.7% of the SMCP patients and in 14% and 3.2% of the ICP patients, respectively. However, no statistically significant difference in either delayed wound healing or fistula occurrence was observed between the two groups. The rate of surgical correction for VPD in the SMCP group was higher than in the ICP group. In the subset of 26 SMCP patients and 62 ICP patients who underwent speech evaluation, the median speech score value was 58.8 in the SMCP group and 66 in the ICP group, which was a statistically significant difference. CONCLUSIONS: SMCP and ICP were found to have similar complication rates, but SMCP had significantly worse speech outcomes.
Cleft Palate* ; Fistula ; Hemorrhage ; Humans ; Postoperative Complications ; Retrospective Studies ; Velopharyngeal Insufficiency ; Wound Healing ; Wounds and Injuries

OBJECTIVETo classify the patients with cleft lip and palate who need orthognathic surgery and to propose the corresponding operations.

METHODSFrom January 2005 to May 2015, 121 patients with cleft lip and palate diagnosed as maxillary retrusion were treated by orthognathic surgery. Inclusion criteriar: (1) male aged over 16, female aged over 14; (2) diagnosed as non-syndromic cleft lip and palate without systemic disease and other genetic diseases; (3) without previous orthodontic and orthognathic treatment; (4) having no other craniofacial malformation. Maxillary features and repaired types were recorded.

RESULTS93 patients were included and divided into two categories depended on the dental crowding. Class I: the teeth quantity and bone quantity is coordinated, space analysis ≤ 4 mm (mild dental crowding). The forward distance of maxillary less than 6 mm was defined as Class I a (36 cases) more than 6 mm as Class I b (28 cases). Class II: the teeth quantity and bone quantity is not coordinated, space analysis > 4 mm ( moderate or severe dental crowding). After the simulation of distraction osteogenesis, the anterior crossbite was corrected defined as Class II a (23 cases), not corrected defined as Class II b (6 cases). Class I a were corrected by conventional orthognathic surgery. While Class I b were corrected by Le Fort I maxillary advancement using distraction osteogenesis. Class II a were repaired just by anterior maxillary distraction. While Class II b need to combine conventional orthognathic surgery with anterior maxillary distraction. All the patients were satisfied with the treatment effect.

CONCLUSIONSThe patients of cleft lip and palate with maxillary retrusion who need orthognathic surgery can be classified as the method mentioned above, and then choose the appropriate operations.

Adolescent ; Adult ; Cleft Lip ; complications ; Cleft Palate ; complications ; Female ; Humans ; Male ; Maxilla ; Osteogenesis, Distraction ; Osteotomy, Le Fort ; Retrognathia ; classification ; surgery

Cleft Lip ; complications ; Cleft Palate ; complications ; Humans ; Maxillofacial Abnormalities ; pathology ; Orthodontics, Corrective

Choristoma ; complications ; Cleft Palate ; complications ; Humans ; Salivary Glands ; Tongue ; abnormalities

INTRODUCTIONThis study aims to review the results of hearing screens in newborns with cleft deformities.

MATERIALS AND METHODSA retrospective audit of 123 newborns with cleft deformities, born between 1 April 2002 and 1 December 2008, was conducted. Data on the results of universal newborn hearing screens (UNHS) and high-risk hearing screens, age at diagnosis, severity/type of hearing loss and mode of intervention were obtained from a prospectively maintained hearing database.

RESULTSThirty-one of 123 newborns (25.2%) failed the first automated auditory brainstem response (AABR). Seventy percent of infants (56 out of 80) who passed the UNHS failed the high-risk hearing screens which was conducted at 3 to 6 months of age. Otolaryngology referral rate was 67.5% (83/123); 90.3% of 31 newborns who failed the first AABR eventually required otolaryngology referrals. Incidence of hearing loss was 24.4% (30/123; 25 conductive, 2 mixed and 3 sensorineural), significantly higher than the hospital incidence of 0.3% (OR: 124.9, 95% CI, 81.1 to 192.4, P <0.01). In terms of severity, 8 were mild, 15 moderate, 5 severe, 2 profound. Eighteen out of 30 infants (60%) were detected from the high-risk hearing screens after passing the first AABR.

CONCLUSIONThese newborns had a higher risk of failing the UNHS and high-risk hearing screen. There was a higher incidence of hearing loss which was mainly conductive. Failure of the first AABR was an accurate predictor of an eventual otolaryngology referral, suggesting that a second AABR may be unnecessary. High-risk hearing screens helped to identify hearing loss which might have been missed out early on in life or which might have evolved later in infancy.

Cleft Lip ; complications ; Cleft Palate ; complications ; Hearing Loss ; complications ; Humans ; Infant, Newborn ; Neonatal Screening ; Retrospective Studies

Bleeding, is one of the most common postoperative complications after palatoplasty in patients with cleft palate. The purpose of this article is to present our experience using a Foley catheter balloon for perioperative palatoplasty bleeding management. A retrospective data analysis was performed for all cases of palatoplasty performed by the author from 1999 to 2012 that experienced postoperative hemorrhage. These patients were managed by utilizing posterior nasal compression with a Foley catheter balloon for the period 2006 to 2012. We have compared two methods (we use before 2006 the reoperative method) with regards to the later development of palatal fistulae and cases with reoperative hemostasis. The study found no statistically significant differences between the conventional and proposed method in relation to the development of palatal fistulae (P=0.7). These findings suggest that nasal packing using a Foley balloon is a safe and effective method for postoperative bleeding control after palatoplasty in patients with cleft palate.
Catheters ; Cleft Palate ; Fistula ; Hemorrhage ; Hemostasis ; Humans ; Postoperative Complications ; Postoperative Hemorrhage ; Retrospective Studies ; Statistics as Topic ; Urinary Catheterization

Cleft Palate ; surgery ; Humans ; Incidence ; Oral Fistula ; epidemiology ; etiology ; Palate ; surgery ; Postoperative Complications ; epidemiology ; Reconstructive Surgical Procedures ; adverse effects ; Statistics as Topic