The lack of isolated palatal clefts in Czech Gypsies

Orofacial clefts are usually divided into three basic types: isolated cleft lip (CL), cleft lip and palate (CLP) and isolated cleft palate (CP). The incidence of specific cleft types in a population and their relative numbers show specific differences between ethnic groups and races. However, there are no available data about the incidence and relative numbers of orofacial cleft types (CL, CLP, CP) in the gypsy ethnic group. The aim of this study was to compare relative numbers of specific types of orofacial clefts between the Czech gypsy and non-gypsy populations. We conducted a retrospective epidemiological study using a set of all living patients with orofacial clefts born in the Czech Republic from 1964 until 2002. The cleft patients were subdivided into three groups: 5304 non-gypsy children, both parents of whom were non-gypsies (NN), 98 gypsy children, both parents of whom were gypsies (GG) and 18 children with one parent non-gypsy and one parent gypsy (NG). The relative number of isolated CP was 37.1% in NN children. However, the relative number of CP was significantly reduced to 5.1% (P < 0.01) in the GG group. Conversely, the relative number of CLP was higher (P < 0.01) in the GG group (62.2%) in comparison to the NN group (39.2%). The tendency to decrease in the relative number of CP and increase in the relative number of CLP was also apparent in the NG group, but not so well expressed. We hypothesize that the decrease in CP and increase in CLP and CL in gypsies might be caused by their genetic predis-position to CL. Since the CP originates later than CL during embryonic development, some CP arise in embryos with already existing CL giving rise to CLP. Consequently, the missing isolated CP might be hidden in the group of CLP patients postnatally.     

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.     

Separate clefts of the lip and the palate. A variant of cleft lip and palate

Separate clefts of the lip and of the palate (CL-CP) may belong to the same etiological class as the cleft lip with or without cleft palate CL(P), or a child may have two separate anomalies, CL and CP. This theory was tested in Finnish cleft patients. Among 2471 cleft cases, there were 66 CL-CP (2.7%). Adequate medical records were available for 62 children: 45 boys (73%) and 17 girls (27%). Familial occurrence was recorded in 6 cases (10%). Of the cleft cases among the near relatives, 5 were CL(P) and one CP. The prevalence of hypodontia was 37% among 38 subjects studied, as compared with 8.2% in the CL-, 29.8% in the CP- and 48.1% in the CLP controls. Conical elevations of the lower lip were observed in none, as compared with 0.8% of the CL(P)- and 39% of the CP controls. It was therefore assumed that the CL-CP belongs to the same etiological class as the CL(P).     

Clefts with associated anomalies and syndromes in Finland.

Probands with clefts born during an 11-year period, 1975-85, were evaluated; 1,586 probands were found of whom 345 (171 males and 174 females, 21.8%) had an associated anomaly. More male patients had cleft lips, with or without cleft palate (CL(P)) and more female patients had cleft palates (CP). The anomalies were subdivided according to anatomical site, and the biggest category was that of the extremities (29.7%) followed by cardiovascular (14.8%) and other facial anomalies (13.0%). The smallest category was chromosomal anomalies (2.7%) followed by miscellaneous anomalies (4.1%). A total of 560 malformations were found. Most anomalies per proband with clefts were found in the bilateral cleft lip and palate subgroup (mean 1.04). The lowest (0.14) was found in the subgroup with the least severe cleft deformity, the cleft lip with or without cleft alveolus. In the CP groups a similar trend was found with 0.21 in the subgroup of submucous cleft palate and 0.33 for the subgroup cleft palate, submucous clefts excluded. A total of 133 probands with 39 different recognised syndromes was delineated, 25 in the CL(P) group and 108 in the CP group (8.4% of the total 1,586 patients with clefts). There was no difference in parental age between probands with an associated anomaly and those with a solitary cleft. Anomalies were more than three times as frequent among probands with clefts as among the general population.     

Craniofacial cephalometric morphology in 6-year-old children with isolated cleft lip, isolated submucous cleft palate, and combined cleft lip and submucous cleft palate.

One hundred and twenty-one cleft children (67 with isolated cleft lip (CL), 32 with isolated submucous cleft palate (SMCP), and 22 with combined cleft lip and submucous cleft palate (CL + SMCP)) were compared retrospectively from lateral cephalograms taken at a mean age of 6.2 years (range 5.5-7.9). None of the children had had their palates repaired or been operated on to treat velopharyngeal insufficiency (VPI). The children with CL + SMCP and CL had similar morphology. They had greater maxillary length, greater maxillary and mandibular prominence, less vertical growth pattern, less deep nasopharyngeal airways, and thinner upper lips than those with isolated SMCP. After 6 years of age one patient with CL + SMCP, none with CL, and 16 with SMCP needed operations for VPI. This small series suggests that children with CL + SMCP and SMCP have different morphology. Although CL + SMCP is a combination of two types of clefts, it seems to be associated with similar morphology to CL.     

Craniofacial cephalometric morphology in 6-year-old children with isolated cleft lip,isolated submucous cleft palate,and combined cleft lip and submucous cleft palate

One hundred and twenty-one cleft children (67 with isolated cleft lip (CL), 32 with isolated submucous cleft palate (SMCP), and 22 with combined cleft lip and submucous cleft palate (CL + SMCP)) were compared retrospectively from lateral cephalograms taken at a mean age of 6.2 years (range 5.5–7.9). None of the children had had their palates repaired or been operated on to treat velopharyngeal insufficiency (VPI). The children with CL + SMCP and CL had similar morphology. They had greater maxillary length, greater maxillary and mandibular prominence, less vertical growth pattern, less deep nasopharyngeal airways, and thinner upper lips than those with isolated SMCP. After 6 years of age one patient with CL + SMCP, none with CL, and 16 with SMCP needed operations for VPI. This small series suggests that children with CL + SMCP and SMCP have different morphology. Although CL + SMCP is a combination of two types of clefts, it seems to be associated with similar morphology to CL.     

Adopted children with cleft lip or palate, or both, require special needs cleft surgery

In recent years adoption of children with cleft lip, with or without cleft palate (CLP), and other birth defects has become more common. The aim of the present study was to describe the characteristics and initial care and treatment of adopted children with CLP. A total of 25 children were referred to our department between 2008 and 2010, 7 (28%) of whom had bilateral CLP and 16 (64%) had unilateral CLP. Two children had atypical clefts. Twenty of the patients (80%) had been operated on with a lip plasty in China before adoption. Most patients (n = 14) was seen by the cleft team within two months of arrival, and 13 were operated on within a month of the first visit at our department. In total, 22 primary palatoplasties, 6 lip plasties, and 1 lip adhesion were done. There were 5 fistulas (14%) three months after the palatoplasty. On arrival, 11 (44%) of the children were carriers of methicillin-resistant Staphylococcus aureus (MRSA). Adoption of children with CLP creates new challenges for the cleft teams, as we no longer have control over the overall treatment plan as regards preoperative and surgical treatment and timing of the operations. The patients are also often carriers of resistant bacteria, which create nursing challenges. In cases where the child is older than a year and has not been operated on, we advocate that the palatoplasty, or combined lip plasty and palatoplasty, is always given priority so that speech development is not compromised.     

Facial clefting in Kuwait and England: a comparative study

A retrospective study of all new cases of facial clefts seen between January 1985 and December 1987 was carried out at the lbn Sina Hospital, Kuwait, and West Midlands Regional Plastic and Jaw Surgery Unit at Wordsley Hospital, Stourbridge, England; 234 arab children in Kuwait and 181 white Caucasian children in the West Midlands were studied. Despite vast differences in racial characteristics, environment and customs the pattern of clefting was very similar in the two groups. The incidence of clefts was calculated for the two regions and this demonstrated an increase in the incidence of cleft lip and palate (CL/P and CP) in the West Midlands since the last study was reported from this region in 1953.     

Physical characteristics of young men with cleft lip, with or without cleft palate, and cleft palate only.

We report the weight, stature, body mass index (BMI), and muscular strength of men about 19 years old who have cleft lip, either with or without cleft palate (CLP), or cleft palate only (CP). Data were obtained from the Swedish National Service enrolment register for the years 1991 -97, and concerned 335 men with CLP and 88 with CP, who were compared with a control group of 272 879 men. The data showed that those with CLP and CP were significantly lighter than controls. Their stature in the CLP group was similar to that in controls, but those with CP significantly shorter. These findings imply that men with CLP had a significantly lower BMI whereas men with CP had a similar BMI compared with controls. Those with CLP did not differ as regards to muscular strength, but those with CP were significantly weaker than controls.     

Physical characteristics of young men with cleft lip,with or without cleft palate,and cleft palate only

We report the weight, stature, body mass index (BMI), and muscular strength of men about 19 years old who have cleft lip, either with or without cleft palate (CLP), or cleft palate only (CP). Data were obtained from the Swedish National Service enrolment register for the years 1991–97, and concerned 335 men with CLP and 88 with CP, who were compared with a control group of 272 879 men. The data showed that those with CLP and CP were significantly lighter than controls. Their stature in the CLP group was similar to that in controls, but those with CP significantly shorter. These findings imply that men with CLP had a significantly lower BMI whereas men with CP had a similar BMI compared with controls. Those with CLP did not differ as regards to muscular strength, but those with CP were significantly weaker than controls.     

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.     

Speech after repair of isolated cleft palate and cleft lip and palate.

The speech of children with isolated cleft palate (CP) repaired by one surgeon has been compared with the speech of children with some form of unilateral cleft lip and palate (CLP) repaired by the same surgeon. All palate repairs included an intravelar veloplasty. We identified 57 children (5--12 years old) with cleft palates repaired in infancy, of which three patients with other medical problems were excluded. Of the 54 patients, 44 (81%) attended for review (27 CP, 17 CLP). Video recordings were analysed by two speech and language therapists, using the Cleft Audit Protocol for Speech. The CP patients had no evidence of permanent fistulas. Final speech outcomes were similar for CP and CLP patients. Intelligibility was normal in 10 (37%) CP and nine (53%) CLP patients. Mild consistent hypernasality was present in five (18.5%) CP and four (23.5%) CLP patients. No patients had moderate or severe hypernasality or nasal emission. Mild consistent hyponasality was present in five (18.5%) CP and five (29%) CLP patients. Moderate consistent hyponasality was present in one (4%) CP patient. Dysphonia was present in eight (30%) CP and seven (41%) CLP patients. Cleft-type characteristics were noted in 11 (41%) CP and nine (53%) CLP patients. No CLP patients but 10 (37%) CP patients had required a pharyngoplasty (P=0.004, Fisher's exact test). Possible reasons for this (age, cleft type, surgeon and surgery) are discussed.     

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.     

Incidence of cleft Lip and palate in the state of Andhra Pradesh, South India

Objective:

To assess the incidence of cleft lip and palate defects in the state of Andhra Pradesh, India.

Design Setting:

The study was conducted in 2001 in the state of Andhra Pradesh, India. The state has a population of 76 million. Three districts, Cuddapah, Medak and Krishna, were identified for this study owing to their diversity. They were urban, semi-urban and rural, respectively. Literacy rates and consanguinity of the parents was elicited and was compared to national averages to find correlations to cleft births. Type and side of cleft were recorded to compare with other studies around the world and other parts of India.

Results:

The birth rate of clefts was found to be 1.09 for every 1000 live births. This study found that 65% of the children born with clefts were males. The distribution of the type of cleft showed 33% had CL, 64% had CLP, 2% had CP and 1% had rare craniofacial clefts. Unilateral cleft lips were found in 79% of the patients. Of the unilateral cleft lips 64% were left sided. There was a significant correlation of children with clefts being born to parents who shared a consanguineous relationship and those who were illiterate with the odds ratio between 5.25 and 7.21 for consanguinity and between 1.55 and 5.85 for illiteracy, respectively.

Conclusion:

The birth rate of clefts was found to be comparable with other Asian studies, but lower than found in other studies in Caucasian populations and higher than in African populations. The incidence was found to be similar to other studies done in other parts of India. The distribution over the various types of cleft was comparable to that found in other studies.     

Palatal Fistulae Following Cleft Palate Surgery

The occurrence and treatment of palatal fistulae have been studied in 1108 CLP patients who had their primary operations performed during the years 1954–69. No fistulae were recorded in 263 patients with incomplete cleft of the primary palate only. These patients were excluded, leaving 845 patients for analysis. The Le Mesurier or Millard technique had been used for the primary lip operation, and the von Langenbeck procedure for closure of the palate; in complete clefts, the anterior part of the palate had been closed using Veau's vomer flap operation simultaneously with lip closure. The observation period ranged from 7 to 22 years, during which time each patient was examined at least once and the majority on several occasions by members of the cleft palate team. The overall incidence of fistulae was 18%. Fistulae were recorded in 11.3% of all complete clefts of the primary palate, and in 36.1% of all complete total clefts. In cases of cleft palate only, fistulae were found in 3.5% of the incomplete clefts, and in 20% of the complete clefts. In patients with bilateral complete clefts, closure of both sides of the lip and anterior palate in one operation seemed to have greatly increased the risk of fistula formation. There was a much higher incidence of fistulae in patients operated on during the years 1954–61 than in those treated in the period 1962–69. Fistula symptoms requiring surgical intervention were recorded in 113 patients. Closure of the fistula was achieved in 84.1%. Of 18 patients with a residual fistula. 17 were asymptomatic or had symptoms so slight that they were considered insignificant and not justifying operation.     

Failure to thrive in babies with cleft lip and palate.

We established the frequency of failure to thrive (FTT) in children undergoing primary cleft procedures by using growth charts and standard-deviation scores. Initially, 147 babies with cleft lip and/or palate undergoing 186 primary lip-and-palate repairs were studied between 1993 and 1996. Rates of FTT were categorised according to cleft type. There was an increasing rate of FTT from 32% for unilateral cleft lip and palate to 38% for bilateral cleft lip and palate to 49% for cleft palate. There was a high incidence of FTT in palatal clefts, especially if these were associated with a syndrome or anomaly (P= 0.001). The incidence of FTT with the Pierre Robin sequence was 100%. In view of the high rates of FTT, two changes were instituted: a feeding-support nurse was appointed to supervise and monitor patients at risk and all patients with the Pierre Robin sequence had supervised airway management. Thereafter, the incidence of FTT was prospectively studied in 68 babies undergoing 84 primary procedures between 1997 and 1999. There was a decrease in the incidence of FTT in comparison with the earlier cohort (9% for unilateral cleft lip and palate, 20% for bilateral cleft lip and palate, 26% for cleft palate). There was a significant decrease in the incidence of FTT in the group with the Pierre Robin sequence, from 100% to 40%. As a result of the provision of a feeding-support nurse and airway management of patients with the Pierre Robin sequence, the incidence of FTT was reduced and the audit loop closed.     

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.     

General body growth in children with clefts of the lip, palate, and craniofacial structure

This paper discusses general body growth in children with craniofacial clefts. Body growth is important in such patients because morphology reflects the cumulation of metabolism over time. The same hormones that direct general body growth also govern the ontogeny of the head and face. Body growth varies in children with different types of clefts. We found no average differences from US norms for those with isolated clefts of the lip alone or those with bilateral clefts of the lip and palate. Children with unilateral clefts of the lip and palate and with isolated cleft palate were significantly shorter than their unaffected peers. Males with these defects were also thinner than normal based on average standard deviation scores for body mass indices. Both unilateral and bilateral clefts of the lip and palate predominated in males, while isolated cleft lip was more frequent in females. Our results indicate that congenital metabolic variation contributes to the development of orofacial clefting and influences postnatal development in certain types of cleft. Accordingly, cleft type is important to growth prognosis, and growth status is relevant to optimization of therapy in orofacial cleft patients.     

Cephalometric pharyngeal changes after Le Fort I osteotomy in different types of clefts

Fifty patients with clefts (30 unilateral cleft lip and palate (UCLP), 9 bilateral cleft lip and palate (BCLP), and 11 cleft palate only (CP), mean age 25 years) treated with Le Fort I osteotomy were compared retrospectively from cephalograms taken shortly before operation, and at six months and one year postoperatively. Patients with bimaxillary surgery or previous velopharyngoplasty, or both, were excluded. Maxillary advancement was moderate in all groups. One year postoperatively there was a significant change (73%–90% of the surgical advancement) in the sagittal depth of the nasopharyngeal airway but not in the depth of the oropharyngeal airway, the length of the soft palate or the position of the hyoid bone. The nasopharyngeal airway was largest in the CP group both preoperatively and postoperatively. Eleven patients (7 CP, 4 UCLP) had a velopharyngoplasty after the osteotomy to improve their speech. There was no difference in the nasopharyngeal airway in the patients treated by velopharyngoplasty compared with those not so treated, but they seemed to have the shortest maxillas and the greatest surgical changes vertically.     

Cephalometric pharyngeal changes after Le Fort I osteotomy in different types of clefts.

Fifty patients with clefts (30 unilateral cleft lip and palate (UCLP), 9 bilateral cleft lip and palate (BCLP), and 11 cleft palate only (CP), mean age 25 years) treated with Le Fort I osteotomy were compared retrospectively from cephalograms taken shortly before operation, and at six months and one year postoperatively. Patients with bimaxillary surgery or previous velopharyngoplasty, or both, were excluded. Maxillary advancement was moderate in all groups. One year postoperatively there was a significant change (73%-90% of the surgical advancement) in the sagittal depth of the nasopharyngeal airway but not in the depth of the oropharyngeal airway, the length of the soft palate or the position of the hyoid bone. The nasopharyngeal airway was largest in the CP group both preoperatively and postoperatively. Eleven patients (7 CP, 4 UCLP) had a velopharyngoplasty after the osteotomy to improve their speech. There was no difference in the nasopharyngeal airway in the patients treated by velopharyngoplasty compared with those not so treated, but they seemed to have the shortest maxillas and the greatest surgical changes vertically.