Clinical and epidemiologic studies of nonsyndromic cleft lip and palate in china: analysis of 4268 cases

The aim of our study was to review clinical and epidemiologic characteristic of the nonsyndromic cleft lip and cleft palate over a period of 10 years at West China Stomatological Hospital, Sichuan University. Four thousand two hundred sixty-eight nonsyndromic cleft lip and cleft palate cases were retrospectively analyzed according to the following variables: general information, cleft type, maternal age, familial history, as well as associated malformations, et al. In these cases, 1075 of which were cleft lip, 1985 were cleft lip with cleft palate; 1208 were isolated cleft palate. There were more men than women in the cleft lip and cleft lip with cleft palate group, while there were more women than men in the isolated cleft palate group. There were significantly more unilateral cleft lip cases than cleft lip on both sides; the cases of cleft lip on the left side were more than that of cleft lip on the right side. The cases with inherited history accounted for 6.68% of all the cases. Most infection cases occurred among the deleterious factors and the complications experienced during the first 3 months after the pregnancy. In all of the 14 twin cases, only 1 of the babies was affected. There were totally 152 cases with other associated malformations. Patients with cleft lip born in November to January were less than those born in the other three quarters of the year. Patients of A blood group composed a higher proportion than the control group. Our data may provide references for appropriate resource use, cleft lip and cleft palate prevention programs, and counseling programs with China-specific data.     

Frequency of homologous blood transfusion in patients undergoing cleft lip and palate surgery

Aim:

The study aims to determine the frequency of homologous blood transfusion in patientsundergoing cleft lip and palate surgery at the Lagos University Teaching Hospital, Nigeria.

Setting and Design:

A prospective study of transfusion rate in cleft surgery conducted at the Lagos University Teaching Hospital, Nigeria.

Material and Methods:

One hundred consecutive patients who required cleft lip and palate surgery were recruited into the study. Data collected included age, sex and weight of patients, type of cleft defects, type of surgery done, preoperative haematocrit, duration of surgery, amount of blood loss during surgery, the number of units of blood cross-matched and those used. Each patient was made to donate a unit of homologous blood prior to surgery.

Results:

There were 52 females and 48 males with a mean age of 64.4 ± 101.1 months (range, 3-420 months). The most common cleft defect was isolated cleft palate (45%) followed by unilateral cleft lip (28%). Cleft palate repair was the most common procedure (45%) followed by unilateral cleft lip repair (41%). The mean estimated blood loss was 95.8 ± 144.9 ml (range, 2-800ml). Ten (10%) patients (CL=2; CP=5, BCL=1; CLP=2) were transfused but only two of these were deemed appropriate based on percentage blood volume loss. The mean blood transfused was 131.5 ± 135.4ml (range, 35-500ml). Six (60%) of those transfused had a preoperative PCV of < 30%. Only 4.9% of patients who had unilateral cleft lip surgery were transfused as compared with 50% for CLP surgery, 11% for CP surgery, and 10% for bilateral cleft lip surgery.

Conclusions:

The frequency of blood transfusion in cleft lip and palate surgery was 10% with a cross-match: transfusion ratio of 10 and transfusion index of 0.1. A "type and screen" policy is advocated for cleft lip and palate surgery.     

Facial morphology and occlusion at the stage of early mixed dentition in cleft lip and palate patients treated with delayed closure of the hard palate

Cephalometric radiographs and dental study casts were analyzed in a group of 23 seven-year-old cleft lip and palate patients, 16 with unilateral and 7 with bilateral cleft. The patients' primary surgical procedures had been completed except for closure of the cleft in the hard palate. For comparison, similar records from another group of patients, 18 with unilateral and 8 with bilateral cleft lip and palate, were studied. In these cases the cleft of the hard palate had been repaired in infancy, using a vomer flap procedure. The results indicated that midfacial growth and dental occlusion of the unilateral cleft sample was significantly better in patients whose closure of the hard palatal cleft had been delayed to the stage of mixed dentition than where repair had been performed with a vomer flap in infancy. No differences were found, however, between similar subgroups with bilateral cleft lip and palate.     

Malnutrition in cleft lip and palate children in Uganda

The objective of this study is to investigate the nutritional status of patients with cleft lip and/or palate when compared to non-cleft lip or palate patients. A retrospective analysis was carried out of all patients aged less than 1 year who were operated on in the Comprehensive Rehabilitation Services in Uganda hospital since opening in April 2009 to November 2010. The data was divided into three groups: cleft lip patients; cleft lip and palate patients and non-cleft patients. The WHO anthropometric calculator was used to calculate weight-for-age Z scores on each patient for the initial outpatient appointment and the operation. The demographic data and Z scores were compared using independent T tests. Three hundred and twenty-one patients were identified, 131 patients had cleft lip alone, 112 patients had cleft lip and palate and 78 patients had no cleft. The cleft lip and palate group had significantly lower Z scores for both the outpatient appointment and operation (i.e. were more malnourished) than either the cleft lip group or the non-cleft group. Malnutrition is a well-documented problem associated with cleft lip and palate. Our research confirms this malnutrition but also highlights the severity of the malnutrition. The patients with cleft lip and palate are unable to feed adequately and therefore need intervention. We operate on these patients once they reach a target weight of 3 kg and repair both lip and palate in one operation to enable patients to feed and improve their nutritional status.     

Oral clefts in the Sultanate of Oman

This is a retrospective study of the epidemiology of congenital clefts of the lip and palate in babies of Omani nationals born in the Sultanate of Oman between 1989 and 1995. Babies of non-Omani immigrants were excluded from the study. The data were collected from two sources. The first was the Centralized Plastic Surgery Service of Khoula Hospital at Muscat, the capital city of Oman between 1989 and 1995, which registers all oral cleft cases in the country. These data revealed the overall incidence of oral clefts (OC) to be 1.5 per 1000 live births, with incidences of 0.62 per 1000 for combined lip and palate (CLP) cleft, 0.34 per 1000 for cleft lip (CL), and 0.54 per 1000 for isolated cleft palate (CP). The second data source was national delivery records from maternity sections of all hospitals in the country from 1989 to 1995, which include oral clefts as congenital anomalies identified at birth. These statistics were similar to those from the first source for CL/CLP but underestimated the number of isolated cleft palates (CP). The histories of patients attending the Plastic Surgery Clinic of Khoula Hospital during the last year of the study period could be examined in detail. A study of the 177 OC cases registered in 1995 alone revealed associated congenital anomalies in 38.4%. A higher inbreeding coefficient of 0.0294 was noted among oral cleft cases than in the general population, which was calculated at 0.0198. Familial clefts accounted for 23% of all cases. Greater parental age and birth order were found to be associated with increased risk of oral cleft. High temperatures during the tropical desert summers in Oman (when temperatures reach 48°C) do not seem related to the incidence of oral clefts.     

唇腭裂1386例发病特点回顾性分析

目的：通过对唇腭裂患者临床资料进行回顾性统计分析,研究该病的发病特点,为唇腭裂的预防及治疗提供临床参考。方法：统计分析2007年1月～2010年1月手术治疗的1386例唇腭裂患者,分别从患者诊断、性别、年龄、出生地,唇腭裂裂型分布情况等方面进行回顾性调查。结果：本组病例中,单纯唇裂356例（25．69％）,唇裂合并腭裂580例（41．84％）,单纯腭裂450例（32．47％）;男809例,女577例,男：女=1．40：1;单侧唇裂伴或不伴腭裂明显多于双侧者,两者之比为5．67：1,其中左侧多于右侧（1．82：1）。结论：唇腭裂发病以唇裂合并腭裂居多,男性发病多于女性,在腭裂患者中女性发病高于男性;单侧发病多于双侧,左侧多于右侧。     

Psychosocial effects of cleft lip and palate on Nigerians: the Ikeja-Lagos experience

We studied 116 patients who presented with cleft lip and palate (CLP) and were seen during a state-wide screening exercise for people with orofacial deformities in Ikeja-Lagos, Nigeria between May 2006 and July 2007. Detailed histories were taken and physical examinations made. The following psychometric instruments: General Health Questionnaire 28 (GHQ28), State-Trait Anxiety Inventory Y1 (STAI-Y1), STAI-Y2, and Self rating Depression Scale (SDS) were given on the respondents by a clinical psychologist. The respondents comprised 57 men and 59 women with a male:female ratio of 1:1.04. There were 42 (36%) adults and 74 (64%) children. Fifty-four (47%) patients had cleft lip (CL), 27 (23%) had cleft lip and palate (CLP), and 35 (30%) had cleft palate alone. Forty-five (39%) of the respondents were parents and guardians who responded to the presence of the deformity on behalf of children who were less than 6 years old, and assessed the reactions of those who were aged 6-12 years old. Older patients replied directly to the psychometric instruments. The outcome of the assessment of the Mental Health Profile of the participants showed high incidence of anxiety, depression, and impairment of general wellbeing. The effects were most pronounced in the 6-12 year age group. We recommend that the psychosocial impacts of CLP on the patients, parents, and relations should be managed together with the reconstructive procedures.     

Facial clefts involving the midline in combination with intracranial anomalies: Case studies illustrating surgical treatment and medical substitution

Abstract Malm? and Uppsala have been regional centres for the treatment of cleft lip and palate since the beginning of the 1950s. We have about 80 new cases every year and most patients have conventional oronasal clefts, either cleft lip and palate or isolated cleft palate. During a 10-year period we have come across four patients who have had varying degrees of midface dysplasia combined with intracranial anomalies. One child died at an early age, but the other three children were given medical substitution of hypopituitarism and have had their clefts reconstructed.     

Comparison of the rate of palatal fistulation after two-flap and four-flap palatoplasty.

We studied 73 repairs of cleft palate (48 cleft lip and palate and 25 isolated cleft palate) done during a 7-year period (January 1996-October 2002) by the same plastic reconstructive surgeon. Two-flap or four-flap palatoplasty techniques were used to provide tension-free, three-layer repairs for patients with cleft palate. Their ages ranged from 10-244 months (mean 27). The postoperative follow-up period ranged from 6 to 60 months (mean 21). There was a palatal fistulation rate of 7% (5/73). There were two fistulas after two-flap palatoplasty (5%, 2/39), and three fistulas after four-flap palatoplasty (9%, 3/34). The mean diameter was 7.8 mm (range 5.1 to 13). There was no significant difference between the two techniques.     

Facial clefts involving the midline in combination with intracranial anomalies: Case studies illustrating surgical treatment and medical substitution

Abstract

Malmö and Uppsala have been regional centres for the treatment of cleft lip and palate since the beginning of the 1950s. We have about 80 new cases every year and most patients have conventional oronasal clefts, either cleft lip and palate or isolated cleft palate. During a 10-year period we have come across four patients who have had varying degrees of midface dysplasia combined with intracranial anomalies. One child died at an early age, but the other three children were given medical substitution of hypopituitarism and have had their clefts reconstructed.     

Length of the cleft in relation to the incidence of hypodontia of the second premolar and to inheritance of cleft lip and palate in children with isolated cleft palate.

We studied severity of the isolated cleft palate expressed as the length of the cleft in relation to hypodontia in the second premolar regions and known inheritance of any type of cleft lip and palate. The material consisted of 47 children at 10 years of age born with non-syndromic isolated cleft palate of varying extent, who all had hypodontia of at least one second premolar, and had panoramic radiographs taken at 10 years of age. Information about length of cleft and inheritance of cleft lip and palate was collected from surgical files initiated at birth. The higher the number of missing second premolars, the more extended was the length of the cleft. More children had hypodontia of the second premolar in the mandible than in the maxilla. The family history had little influence on the length of the cleft.     

Use of Titanium Dental Implants as an Integrated Part of a CLP Protocol

The problem of edentulousness in the cleft area of patients with cleft lip and palate was formerly resolved with a conventional fixed bridge construction, but this approach did not always prove optimal. Nowadays, in these patients the bridge can be substituted by a crown on an osseointegrated titanium implant fixture. The concept of dental rehabilitation using titanium implants has gradually merged into our routine treatment for patients with cleft lip and palate. In this report we describe our surgical technique using osseointegrated titanium implants and evaluate our result in patients with cleft lip and palate. Sixteen patients with cleft lip and palate, 11 men and five women, were divided into two groups: group 1 consisted of six patients who did not need bone grafting prior to the fixture installation, and group 2 comprised 10 patients who had additional bone grafting three months before the fixture installation. Abutments were applied six months after fixture installation. Dental crowns and fixed bridges were then constructed. All patients were seen on regular follow-up visits. Routine roentgenograms were obtained preoperatively and when the abutments were applied. Photographs were taken at these occasions and also after the prosthodontic work was completed. Of a total of 31 fixtures, all except two were osseointegrated at the time of the abutment connection, and the remaining 29 have all been functional during the observation period, giving a success rate of 93%. In group 1 all fixtures (100%) were osseointegrated. The non-integrated fixtures were found in group 2 giving a success rate of 91% in this group. The mean follow-up time after fixture installation was six years and three months, and the mean observation time with loaded fixtures was five years and six months.     

22q11 Deletion in children with cleft lip and palate--is routine screening justified?

OBJECTIVE: To ascertain the prevalence of 22q11 deletion in children with a diagnosis of cleft lip and/or palate that had been referred to the Cleft Lip & Palate Service, Newcastle-upon-Tyne. DESIGN: Retrospective analysis of results of 22q11 FISH testing performed in all such referrals. PARTICIPANTS: 191 children, of whom 13 had a bilateral cleft lip and palate, two had a median cleft, 77 had a cleft palate only, 44 had a unilateral cleft lip, 47 had a unilateral cleft lip and palate and eight had a submucous cleft palate. RESULTS: nine patients had a positive 22q11 FISH test. CONCLUSION: This represents a higher percentage than has been previously reported. All children with cleft lip and/or palate should routinely have a 22q11 FISH test in view of the implications of a diagnosis of velocardiofacial syndrome.     

颌面部神经阻滞在唇腭裂修复术围手术期的应用进展

背景 唇腭裂在小儿先天性疾病中占很高比列,小儿唇腭裂修复术围手术期的疼痛对喂养和伤口愈合有很大影响. 内容 总结分析颌面部神经阻滞对唇腭裂患儿围手术期镇痛、术后苏醒、颌面部神经阻滞相关并发症以及对术后应用镇痛药物各方面的影响. 目的 通过对小儿唇腭裂修复术围手术期颌面部神经阻滞镇痛方式、应用药物研究现状的总结归纳,探讨颌面部神经阻滞用于唇腭裂修复术围手术期的镇痛效果和副作用,为唇腭裂修复术围手术期镇痛提供有效的依据,完善小儿镇痛. 趋向 颌面部神经阻滞是唇腭裂修复术围手术期较为理想的一种镇痛方式,超声的引进使小儿术后镇痛得到进一步完善.但目前仍缺乏一种绝对满意、安全的颌面部神经阻滞方法,相关内容仍需进一步临床研究.     

Multidisciplinary management of Opitz G BBB syndrome

Opitz G BBB syndrome is a rare condition characterized by the 3 major anomalies of hypertelorism, cleft lip and palate, and hypospadias, although there may be other associated anomalies. The underlying genetic causes are complex and consist of both X-linked recessive and autosomal dominant forms of the disorder. Previously, there have been publications on the underlying genetics and case reports, but there have been few reports regarding the long-term outcome. The aim in this study was to review the range of clinical presentation and evaluate outcomes of the multidisciplinary management of a cohort of patients with Opitz G BBB syndrome. In a 25-year period, 7 patients with Opitz G BBB syndrome were managed by the Australian Craniofacial Unit (ACFU), 5 male and 2 female. Most of the patients are now reaching skeletal maturity. Each one presented with a range of severity in the triad of hypertelorism, cleft lip and palate, and hypospadias anomalies. The males all exhibited the triad of anomalies, while the females both had hypertelorism, only 1 had isolated cleft palate, and neither had any genitourinary anomalies. Each patient underwent multidisciplinary assessment to make a treatment plan for staged management of different anomalies. Plan for surgical corrections of facial anomalies were performed according to the unit's protocol management of both hypertelorism and cleft lip and palate, but the presence of these coexisting anomalies required adjustment of the standard protocol of management of cleft lip and palate. In conclusion, we recommend that patients with Opitz G BBB syndrome require careful evaluation, and management of the anomalies should be in a coordinated manner by a multidisciplinary team.     

A comparison of dermabond tissue adhesive and sutures in the primary repair of the congenital cleft lip

OBJECTIVE: To compare the long-term cosmesis of Dermabond (octyl-2-cyanoacrylate) and traditional skin sutures among patients undergoing primary cleft lip +/- palate repair. MATERIALS AND METHODS: Eleven patients underwent photographic analysis following primary cleft lip +/- palate repair, including the use of Dermabond. Eleven age-matched controls who underwent cleft lip +/- palate repair with traditional suture closure served as controls. Cosmesis was assessed by 3 blinded plastic surgeons using a visual analogue scale (VAS) and the Hollander Wound Evaluation Scale (HWES). RESULTS: The overall mean VAS score for the patients treated with and without Dermabond was 70.0 (SD, 9.5) and 68.3 (SD, 13.4), respectively (P = 0.46). The overall mean HWES score for the patients treated with and without Dermabond was 1.7 (SD 1.7) (P = 0.92). CONCLUSIONS: Dermabond tissue adhesive offers equivalent mature wound cosmesis as traditional suture closure in the repair of the congenital cleft lip +/- palate.     

Cleft surgery in East timor: the first four years

BACKGROUND: Following the vote for independence in 1999, this team commenced the first coherent reconstructive surgical service in East Timor. The aim of this paper is to report the cleft lip and palate surgical experience during the 4 years since independence. METHODS: From June 2000, a record of all cleft surgical procedures carried out by our team in East Timor has been maintained. This has been reviewed to identify the clinical experience, procedures carried out and the outcomes of the cleft population in this, the poorest and newest, nation in Asia. RESULTS: From the 519 reconstructive surgical procedures carried out by this team in East Timor during the first 4 years, 267 were for cleft lip and palate deformity. Cleft lip and nose repairs were most commonly undertaken, although over the period of the study increasing numbers of cleft palate repairs were evident, reflecting the developing confidence in the service by the local population. CONCLUSION: The establishment of a regular, consistent cleft lip and palate surgical team in East Timor has not only seen the successful correction of a large number of untreated cleft patients, but has also contributed to a restoration of trust in the newly developing health system in East Timor.     

Comparison of the rate of palatal fistulation after two-flap and four-flap palatoplasty

We studied 73 repairs of cleft palate (48 cleft lip and palate and 25 isolated cleft palate) done during a 7-year period (January 1996–October 2002) by the same plastic reconstructive surgeon. Two-flap or four-flap palatoplasty techniques were used to provide tension-free, three-layer repairs for patients with cleft palate. Their ages ranged from 10–244 months (mean 27). The postoperative follow-up period ranged from 6 to 60 months (mean 21). There was a palatal fistulation rate of 7% (5/73). There were two fistulas after two-flap palatoplasty (5%, 2/39), and three fistulas after four-flap palatoplasty (9%, 3/34). The mean diameter was 7.8 mm (range 5.1 to 13). There was no significant difference between the two techniques.     

Cleft lip and palate patients prior to delayed closure of the hard palate: evaluation of maxillary morphology and the effect of early stimulation on pre-school speech

Speech and maxillary development were analysed in two groups of patients with unilateral cleft lip and palate; both groups had early jaw orthopaedic treatment and a surgical regimen that included two-stage lip surgery (mean ages of 2 and 19 months) and soft palate repair (8 months). Closure of the hard palate was postponed until the children were 8 to 10 years of age. The first group comprised 10 consecutive patients who were analysed at 5 and 7 years of age, and the second group seven patients who were studied at the age of 5. Both groups were thus investigated before the repair of the cleft in the hard palate. In addition to surgical and jaw orthopaedic treatment, the second group of patients received early stimulation of lip and tongue tip movements. Our results indicated that hypernasality was less a problem than was retracted palatal or velar articulation of dental consonants. These deviations tended to be reduced, however, after early stimulation. There seemed to be no clear association between the size of the residual cleft in the hard palate and the extent of speech development. The average size of the residual cleft in our patients was comparatively small, and decreased further during follow up. We conclude that preschool children with unilateral cleft lip and palate may develop good speech, in spite of the residual cleft, if they use an intraoral plate and are given extra lip and tongue tip stimulation, together with early speech therapy if necessary.     

A clinical and demographic profile of the cleft lip and palate in Sub-Himalayan India: A hospital-based study

Objectives:

To study the cleft lip and cleft palate in the poverty stricken Sub-Himalayan Garhwal region of India, being a commonly seen congenital abnormality and scarcity of studies about the demography of cleft in this region.

Design:

A prospective cohort observational case series was performed on 4657 cleft patients at a Tertiary care Hospital in Dehradun, India, over a period of 5 years.

Outcome measures:

The authors investigated the differences between age and sex with cleft status and family history of clefts, birth order, religion, socioeconomic status, parent literacy, source of information for treatment, haematological investigations showing the status of infection and coagulation in such children and satisfaction after treatment.

Results:

Seventy-two percent parents of cleft lip patients were illiterate, and only 8% were graduates, the majority of patients were from the low socioeconomic class. The siblings of 1.1% of the cleft patients had similar deformity. Anemia was seen in 83.16% cases which was commonly microcytic hypochromic type and eosinophilia was seen in 25.50% of cases. In the coagulation profile, International Nationalized Ratio was found to be raised in 52.12%. Almost 95% of the families were fully satisfied by the treatment and results.

Conclusion:

This study will provide baseline information on the status of these less privileged cleft patients in this mountainous region for future reference to health workers.KEY WORDS: Cleft lip, cleft palate, haematological, sub-Himalayan region, socioeconomic