Prenatal compared with postnatal cleft diagnosis: What do the parents think?

Abstract

Since the 1980s, prenatal detection of cleft lip with or without cleft palate (CLP) is possible with a transabdominal ultrasound. The improvement of prenatal detection has resulted in an increased demand for prenatal information and counselling. The aim of this study was to, by means of a questionnaire, evaluate how families, who received the diagnosis prenatally and postnatally, respectively, think about the prenatal and postnatal consultation. Patients referred prenatally or postnatally to the department, due to CLP, between January 2006 and September 2010 were included in the study. Thirty-six of the families (95%) answered the questionnaire concerning prenatal counselling. A total of 46 parents (82%) answered the questionnaire concerning postnatal counselling. In the prenatal group, half of the patients were first informed by the ultrasound unit, whereas 16% were initially informed by the cleft team. More of the families that were informed by the cleft team were content with the information received than the families who were informed by the ultrasound unit. The majority of the families were satisfied with the structure of the consultation. Most of the patients in the postnatal group were happy with the postnatal consultation. Most of the parents in the postnatal group would have wanted to know about the diagnosis prenatally. The great majority of the parents was happy with the consultation with the cleft team and thought that they received enough information. However, the information received at the referring units could be improved. Parents seem to want information on the cleft diagnosis and its treatment as soon as possible.     

Prenatal compared with postnatal cleft diagnosis: What do the parents think?

Abstract Since the 1980s, prenatal detection of cleft lip with or without cleft palate (CLP) is possible with a transabdominal ultrasound. The improvement of prenatal detection has resulted in an increased demand for prenatal information and counselling. The aim of this study was to, by means of a questionnaire, evaluate how families, who received the diagnosis prenatally and postnatally, respectively, think about the prenatal and postnatal consultation. Patients referred prenatally or postnatally to the department, due to CLP, between January 2006 and September 2010 were included in the study. Thirty-six of the families (95%) answered the questionnaire concerning prenatal counselling. A total of 46 parents (82%) answered the questionnaire concerning postnatal counselling. In the prenatal group, half of the patients were first informed by the ultrasound unit, whereas 16% were initially informed by the cleft team. More of the families that were informed by the cleft team were content with the information received than the families who were informed by the ultrasound unit. The majority of the families were satisfied with the structure of the consultation. Most of the patients in the postnatal group were happy with the postnatal consultation. Most of the parents in the postnatal group would have wanted to know about the diagnosis prenatally. The great majority of the parents was happy with the consultation with the cleft team and thought that they received enough information. However, the information received at the referring units could be improved. Parents seem to want information on the cleft diagnosis and its treatment as soon as possible.     

Ambulatory surgery for cleft lip repair

Standard of care for cleft lip repair has included preadmission testing, surgical correction, and postoperative hospital care. Driven not by managed care economics but to speed the safe home care of infants by parents, the authors have gained experience in ambulatory cleft lip repair. In this retrospective study the authors evaluated the outcome of patients who underwent ambulatory cleft lip repair compared with those patients who were hospitalized after surgery. From 1989 to 1998, 24 cleft lip repairs in 24 patients performed by the senior author were evaluated. Two groups were treated. Group 1 (N = 11) consisted of ambulatory unilateral cleft lip repairs and group 2 (N = 13) consisted of inpatient unilateral cleft lip repairs. Important surgical factors considered were technique of cleft lip repair, performance of ancillary procedures, type of local anesthetic administered, and intravenous steroid administration. Time to first postoperative feeding and complications, including bleeding, spontaneous or traumatic wound dehiscence, and infection, were considered important outcome parameters. There were no differences in surgical technique or use of antibiotics and postoperative analgesics between the two groups. None of the patients in group 1 underwent ancillary procedures. Four patients underwent soft palate repair and 3 patients underwent insertion of myringotomy tubes among group 2 patients. The use of a 1:1 mixture of 1% lidocaine and 0.5% bupivacaine with epinephrine vs. 1% lidocaine with epinephrine as a local anesthetic and intravenous steroid administration was greater in group 1 (92%) than in group 2 (33%) patients. The average time to the first postoperative feeding was more than 1 hour sooner in the ambulatory group (p < 0.05) compared with the hospitalized group (excluding the 4 patients who underwent soft palate repair). There were no complications among patients with ambulatory cleft lip repair, and there were two cases of minor wound separation in patients who received postoperative hospital care. Although many variables factor into the outcome after cleft lip repair, these data support the safety and continued practice of ambulatory cleft lip repair.     

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.     

Psychological issues in cleft lip and palate

The treatment of cleft lip and palate extends over the infancy, childhood, and adolescence of individuals who are born with this condition. The children and their families contend with multiple surgeries throughout these years. Depending on the severity of the cleft, the families may also need to cope with speech therapy, ear infections, learning disabilities,and various orthodontic treatments. The end result of these treatments and interventions should be a child, teen, or adult who is an appropriately contributing member of society at each stage of development. The psychological support needed to enable a child to meet that goal should be provided by family, school, the surgeon, and other members of the cleft treatment team. These adults should demonstrate a belief in the child's ability to cope with the challenges of cleft lip and palate treatment and should focus the child on the efficacy he will gain from having had the experience of growing up with a cleft lip and palate.     

用医学美学的理念指导唇腭裂患儿的临床护理

目的:总结以医学美学的理念指导先天性唇腭裂患儿临床护理的经验。方法:本组患儿309例,男169例,女140例;年龄:1月~18岁,体重1.5~66Kg;单纯唇裂79例,唇裂伴腭裂181例,单纯腭裂49例。所有患者均行手术整复。主要的护理内容有:从医学美学的角度进行唇腭裂相关知识的宣教;帮助患儿及父母的心理达到平衡状态;以医学美学的理念指导常规护理操作和舒适护理;美化病房环境;出院的美学指导。结果:309例唇腭裂患儿的护理均取得了较满意的效果,唇裂患儿术后容貌明显美观;仅8例腭裂患儿发生腭瘘或复裂,其余患儿创口愈合良好,悬雍垂成形,语音明显改善。结论:以医学美学的理念指导临床护理,有助于提高唇腭裂患儿的医护质量和患者满意度,并有利于提高护理人员的美学修养。     

Patient satisfaction with cleft lip and palate services in a regional centre.

We sent a questionnaire to the parents of 478 children aged between 3 and 14 years who are under the care of the cleft team at the Queen Victoria Hospital, East Grinstead, and received 341 replies. A wide variety of questions were asked about aspects of patient satisfaction, and the results are reported and discussed. There was a high level of satisfaction with the service provided but 30% of parents would like to be more involved in treatment-planning decisions; 33% thought they had either not enough or no knowledge about cleft lip and palate and its treatment. Only 8% of parents would rather have seen the specialists separately than together in the joint clinic.     

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.     

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.     

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。     

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.     

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.     

Prenatal diagnosis of cleft lip and palate.

A prospective study was conducted over a period of 8 years to compare the severity of the cleft lip and palate detected by ultrasound scanning of the foetus with the severity at birth. In the UK all pregnant women undergo a routine perabdominal ultrasound scan at 20 weeks' gestation. There were approximately 250000 scans performed in our catchment area during the study period. A total of 270 children were born with cleft lip and/or palate, of which 130 had cleft lip with or without cleft palate. Out of these, 23 were positively diagnosed by the ultrasound scan. The specificity was 100% and the sensitivity was 17.5%. Only two diagnoses led to termination of the pregnancy.     

Congenital duodenal obstruction: the impact of an antenatal diagnosis

In a series of 34 infants with congenital duodenal obstruction, 15 were diagnosed by antenatal ultrasound between the fifth month of gestation and term. Fourteen patients were available for follow-up. In 10 of 14 cases, patients were available for follow-up. In 10 of 14 cases, parents were informed of the diagnosis antenatally and, in 9, were advised of the possibility of surgery in the neonatal period. Parents felt the information helped them prepare for the surgical and medical interventions necessary in the postnatal management of their infants. The mean birth weight and gestational age of these infants were comparable with those diagnosed postnatally. Associated congenital anomalies were more common in the patients with a postnatal diagnosis. In the total group of 34 infants with duodenal obstruction, 32 had surgical repair with a survival rate of 94%. Surgical procedures were comparable between the two groups. Surgical intervention occurred earlier in the neonatal period when an antenatal diagnosis was made. Overall survival in the entire series was 88% with four deaths attributed to severe associated congenital anomalies. We conclude that (1) the antenatal diagnosis of duodenal obstruction influences parents positively in coping with the anomaly, and (2) although surgery was performed sooner, the outcome of infants with duodenal obstruction was not changed by providing an antenatal diagnosis.     

正颌联合正畸矫治唇腭裂术后牙颌面畸形

目的以正颌外科手术联合正畸治疗的方法矫治唇腭裂术后牙颌面畸形。方法1996年以来共矫治此类畸形2l例，采用手术方法如下：Le FortⅠ型截骨术7例；多片段Le FortⅠ型截骨术5例；Le FortⅠ型截骨术加双侧下颌升支矢状劈开术（BSSRO）4例；BSSRO加颏成形术2例：Le FortⅠ型截骨术加下颌体部截骨术2例；BSSRO1例。结果2l例术后均达到模型外科设计要求，术后随访14例，平均25．6个月，九严重并发症及明显复发。12例行术前后正畸治疗者希关系及面部外形均满意，另2例个别牙齿矛台关系欠佳。结论以正颌外科手术联合正畸治疗的方法矫治成年期唇腭裂术后牙颌面畸形可取得满意结果。     

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。     

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.     

唇腭裂病人成年后的综合美容

既使是近乎完美的唇腭裂早期治疗，随着人体面部器官的生长发育，仍有可能出现鼻部、唇部畸型，因此，唇腭裂后期治疗至关重要。通过对１６０２例早期及后期唇腭裂病人的治疗体会与总结，指出各类型唇腭裂病人成年后常见的各种面颌部畸形。阐述了适用于该类病人的手术前设计、治疗原则、治疗步骤及方法。针对唇腭裂病人成年后所出现的畸形往往是多方面的这一现象，结合口腔颌面外科、正畸修复科、整形美容外科等专科特点，提出了综合美容整形的设计思路及治疗要点     

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.     

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.