四维超声成像技术在胎儿唇腭裂畸形中的诊断价值分析

目的 分析四维超声成像技术在诊断胎儿唇腭裂畸形中的应用价值。方法 选择2019年1月-2022年 1月于我院进行常规超声检查的12 334例孕妇作为研究对象,发现胎儿疑似唇腭裂畸形者,运用四维超声 成像技术帮助诊断,重点观察胎儿上唇皮肤及上腭骨的连续性,以引产后或分娩后胎儿的颜面部实际情况 作为评价标准,分析四维超声成像技术在胎儿唇腭裂畸形诊断中的准确性。结果 共发现5例唇腭裂畸形胎 儿,与引产后或分娩后胎儿颜面部实际情况一致,四维超声成像技术诊断准确性为100.00%。结论 四维超 声成像技术在胎儿唇腭裂畸形中的诊断准确性较高,对于诊断胎儿唇腭裂畸形具有较好的临床应用价值。     

Correlation of cleft type with incidence of perioperative respiratory complications in infants with cleft lip and palate

BACKGROUND: A retrospective survey of 339 infants who had undergone primary plastic surgery for cleft lip and palate was performed to evaluate the concomitant preoperative assessment based on severity grading of the common cold and the correlation of cleft type with the incidence of perioperative respiratory complications. METHODS: We assessed the severity of common cold symptoms in the preoperative period using the Common Cold Score, which comprises 10 symptoms and findings. We then determined the association of the incidence of perioperative respiratory complications with the increasing severity of common cold symptoms and also compared the complication incidence in the three cleft types in healthy infants without a common cold. RESULTS: The incidence of perioperative respiratory complications was greater in the group with a suspected presence of a common cold. Infants with severer cleft, who had bilateral cleft lip and palate, even without common cold symptoms, had a significantly higher incidence of perioperative respiratory complications (8.9%) than infants with simple cleft lip (1.7%, P < 0.05). CONCLUSIONS: Clinicians should consider postponing primary plastic surgery for cleft lip and palate in infants with a suspected presence of a common cold. Our results also suggest that the presence of a wide cleft is a risk factor for causing perioperative respiratory complications in infants with cleft lip and palate. We believe that a careful preoperative assessment of common cold symptoms in these infants can decrease the incidence of perioperative respiratory complications.     

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.     

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.     

The clinical observation of difficult laryngoscopy and difficult intubation in infants with cleft lip and palate

BACKGROUND: The aims of this study were to evaluate the incidence of difficult laryngoscopy in infants with cleft lip and palate and to observe its relationships with age, sites, and degrees of deformities. METHODS: A total of 985 infants aged 1 month to 3 years, undergoing repair of cleft lip and palate were included in this study. The infants suffering from unilateral cleft lip, simple cleft palate, and combined bilateral cleft lip and palate were 465, 421, and 79 respectively. They were divided into three groups according to age; 1-6 months group, 6-12 months group and 1-3 years group. RESULTS: The total incidence of difficult laryngoscopy was 4.77%. The incidence of difficult laryngoscopy was closely related to age, sites and degrees of deformities, and micrognathia. The incidence of difficult laryngoscopy was 7.06% in 1-6 months group, 2.90% in 6-12 months group, and 3.13% in 1-3 years group, and was greatest for infants with combined bilateral cleft lip and palate, less for those with left cleft lip and least for those with right cleft lip and simple cleft palate. The incidences of difficult laryngoscopy in infants with and without micrognathia were 50% and 3.83% respectively. The incidences of moderately difficult, difficult, and failed intubations were 1.02%, 0.91%, and 0.102% respectively. CONCLUSIONS: Infants with cleft lip and palate, left cleft lip and alveolus, combined bilateral cleft lip and palate, micrognathia, and age <6 months were the important risk factors for difficult laryngoscopy. Difficult intubation occurred mainly in infants with laryngoscopic views of grade III and IV.     

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。     

Cleft lip and palate repair: the experience from two West African sub-regional centres.

Compliance with primary surgical treatment in people with cleft lip and palate is a well-known problem, especially in developing countries fraught with poverty and ignorance. Different protocols of treatment exist. In this paper, we retrospectively review a cohort from two centres, with a discussion on the outcome and its implications. The records of all patients with cleft lip and palate seen in the National Orthopaedic Hospital, Enugu, Nigeria, from January 1993 to December 1999, were sought, and all available case notes reviewed retrospectively. This included new cases seen in the period, and also cases operated during this period. Follow up took place until January 2005, when the data were collated. The records of all such patients seen at Ladoke Akintola University Teaching Hospital Osogbo, Nigeria, from September 2004 to June 2006 were also collated and analysed. During this period, 102 patients were seen (93 at Enugu and nine at Osogbo). Fifteen had isolated cleft palate, 42 had isolated cleft lip and 45 had combined cleft lip and palate. Presentation time ranged from 1 day to 43 years. The palate was not repaired in 20 people after lip repair; two patients with cleft lip and palate completely defaulted; and only one person with isolated cleft palate failed to undergo surgery in this period. Two patients in Osogbo absconded. The West African sub-region has a high drop out rate after lip repair.     

Early postoperative complications in primary cleft lip and palate surgery

We studied the occurrence of early complications of cleft lip and palate surgery by the retrospective study of 132 consecutive cases (160 procedures) operated between three and six months of age. Three children presented with major complications within the immediate postoperative period. All were treated successfully. A local complication directly related to the operation occurred in 7.5% of the cases within the first six postoperative days. In 17%, a general complication not directly related to the operation was observed. The majority of these general complications were cross infections during the hospital stay. These observations led us to reduce the hospital stay to one day for cleft lip and five days for cleft palate and cleft lip and palate operations without compromise to safety but with a reduction of the morbidity and hence the treatment costs and of the child-family disturbance.     

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.     

The impact of antenatal diagnosis on the effectiveness and timing of counselling for cleft lip and palate.

Birth of a cleft child is emotionally distressing to the parents. The opportunity for forewarning the parents is now possible with antenatal ultrasound diagnosis of the cleft. This has opened up a new chapter of antenatal counselling. We present a retrospective postal study of 124 primary cleft lip and/or palate repairs operated over the last 5 years. In our study, 30% were diagnosed antenatally and received antenatal counselling. The rest received postnatal counselling. Of those who had an antenatal diagnosis 85% felt that the diagnosis prepared them psychologically for the birth of the cleft child. A total of 92% (n = 23) of parents who had antenatal as well as postnatal counselling, as compared with 71% (n = 46) of those counselled only postnatally, said the information given to them by the counselling cleft team was detailed and satisfactory. The majority of those who felt counselling was confusing had only postnatal counselling. Of those who had antenatal diagnosis 92% said that they had never contemplated termination of pregnancy. Only one parent had actually terminated her pregnancy due solely to the antenatal diagnosis of a bilateral cleft lip and palate. Our study has highlighted the importance of antenatal diagnosis of cleft and the importance of antenatal counselling for the delivery of a cleft child to be a positive experience.     

Management of cleft lip and palate by the Doctor Magalon's team in Marseille

The goal of the treatment of patients with cleft lip and/or cleft palate was: obtain a normal appearance, a normal speech and a normal growth without severe pertubations of the quality of life. Millard technique was used to repair the cleft lip in neonatal period. Between 6 and 18 months, the palate cleft was closed by wardill-kilner technique. A gingivoperiostoplasty with osseous graft was performed between 8 and 12 years. The orthodontic treatment began at 7 years. Pharyngoplasty was necessary in several cases. Sequellae and rhinoplasty was performed at the end of the growth. The authors explained their choices.     

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.     

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.     

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.     

Early cleft lip repair in children with unilateral complete cleft lip and palate: a case against primary alveolar repair

All children with complete unilateral cleft lip and palate will develop some degree of malocclusion regardless whether the alveolar cleft is repaired primarily or bone grafting is deferred. To evaluate the impact of early gingivoperiosteoplasty on occlusal relationships, dental models were obtained in 5-year-old patients who underwent early cleft lip and palate repair with primary boneless bone grafting (Skoog's method) (56 children) and without alveolar intervention (51 children). The Goslon's occlusion grading system was applied to evaluate occlusal relationships in both groups. Patients with early surgical intervention to repair alveolar cleft demonstrated poor occlusal relationship with the Goslon score 4 and 5, which will likely need an orthognathic corrective procedure (50% vs. 19.6% in patients without early primary dissection of the alveolar process). Results reaffirm that an inclusion of the alveolar process into the early primary lip repair adds to the severity of occlusal maldevelopment.     

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.     

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.     

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.     

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.     

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.