Abnormalities of the maxillary incisors in children with cleft lip and palate

Dental anomalies of the maxillary anterior teeth were studied in seventy-seven children affected by unilateral and bilateral clefts of the lip and alveolar process, with or without involvement of the palate. As for the permanent lateral incisor in the cleft area, our results show that its congenital absence is the most frequent abnormality followed by anomalies in size and shape and supernumerary teeth. Enamel hypoplasia was found to affect the permanent central incisor on the cleft side more frequently. Early recognition of tooth abnormalities during the primary dentition phase for an interceptive treatment of potentially severe problems was emphasized.     

The adolescent with cleft lip and palate

Research studies afford an in-depth look at the problems experienced by adolescents born with a cleft lip, a cleft palate or cleft lip and palate. If not addressed, their problems can multiply with each successive stage of development. An interdisciplinary team, where the adolescents themselves play an important role, is one of the best means of helping them come to terms with the consequences of the abnormality. The nurse should be a key member of the team. To help adolescents cope with their situation now and in the future, the author promotes a holistic and humanistic approach. Here, she sees nurses creating therapeutic alliances as educators. She recommends that nurses assert themselves more as professionals and as essential resources. Her doctoral thesis proposes a training model to this effect.     

Improved treatment of cleft lip and palate is now available

When cleft lip and palate treatment was introduced at Gothenburg in 1957, the procedure used was early bone grafting (EBG). By 1965, EBG had been omitted from the regimen, bone grafting being postponed until the appearance of mixed dentition. Analysis of the results of both techniques showed maxillary retrusion of different degrees. Accordingly, this routine was abandoned in 1975, being replaced by a procedure which is characterised by delayed closure of the hard palate (DCHP). Thus, the surgical procedure comprised the following steps: 1, lip closure at 1-2 months of age; 2, soft palate repair at 6-8 months; 3, final lip-nose surgery at 12 months; and 4, closure of the left in the hard palate, and bone grafting to the alveolar process during mixed dentition at about 8-10 years of age. Follow-up has shown the majority of patients to manifest acceptable speech development during childhood, though problems may occur in some cases. Maxillary growth has been found to be improved after DCHP, and at present the need of maxillary advancement surgery has been reduced to approximately 5% of cases, as compared with the former rates of 50% of cases among those treated with EBG, and of 25% among those treated with the vomer flap procedure.     

Effects of presurgical orthopedic treatment in infants with complete bilateral cleft lip and palate

Recently, it has been demonstrated that the endothelium of corpus cavernosum (CC) plays an important role on smooth muscle relaxation, which is crucial to initiate and maintain erection. We investigated the effect of long-lasting additional testosterone propionate (TP) therapy on endothelium-dependent and -independent relaxations of isolated rabbit CC. Isolated CC strips were mounted in organ baths and isometric tension was recorded. Addition of a specific inhibitor of nitric oxide synthesis, NG-nitro-L-arginin methyl ester (L-NAME), into the organ bath had no effect on the relaxation responses to adenosine (ADO), adenosine 5'-triphosphate (ATP) and sodium nitroprusside (SNP) in isolated CC strips precontracted with phenylepherine, but completely inhibited relaxation responses produced by ADP. Adenosine and adenine nucleotides relaxed the phenylepherine-induced contractile response in control strips with the potency order: ADO (62.8 +/- 3.2%) > ATP (37.1 +/- 5.2%) > ADP (25.8 +/- 2.5%). The relaxation responses to ADO, ATP and SNP in isolated rabbit CC strips were not significantly altered by additional TP therapy. The relaxation responses produced by ADP were significantly enhanced following 1 and 2 months TP therapy as compared with controls. However, in the group treated with TP for 2 months followed by a 2 months drug-free period, relaxation responses were significantly reduced compared to 1 and 2 months treatment groups, and approached control values. Increased relaxation responses to ADP following 1 and 2 months additional TP therapy may be a result of increased endothelial purinergic receptor density, or it may be due to stimulation and/or release of endothelial nitric oxide (NO) by TP.     

Pharyngoplasty in patients with cleft lip and palate after maxillary advancement

The development of velopharyngeal incompetence and increased hypernasality after maxillary advancement has been described previously by several authors. If speech and velopharyngeal function deteriorate after maxillary advancement, pharyngoplasty is frequently the treatment procedure of choice because of the natural cause of the deficit. Of 91 cleft lip and palate patients who have undergone maxillary advancement at the Australian Cranio-Facial Unit, 23 patients received a pharyngoplasty after surgery. Thirteen of these patients who had pre- and postoperative speech evaluations were included in this study. Of the 13 patients, six patients received a superiorly based pharyngeal flap, two patients underwent an orticocheal pharyngoplasty, and five patients received either a revision or augmentation of the previous flap based on results of preoperative examinations. Serial nasendoscopic evaluations were available for 11 of these 13 patients, and they demonstrated that velopharyngeal function improved after pharyngoplasty in six patients and was unchanged in five patients. Of the 13 patients, 10 improved and three patients were unchanged on an intelligibility rating. Nine of the 13 patients demonstrated decreased hypernasality and four patients were unchanged. Hyponasality decreased in two patients increased in one patient, and was unchanged in one patient. Because the results obtained are considered acceptable, the authors conclude that pharyngoplasty can be used effectively to treat velopharyngeal dysfunction subsequent to Le Fort I maxillary advancement.     

Evaluation of the results of cleft lip and palate surgical treatment: preliminary report

We have analysed randomized controlled studies which reported the incidence of postoperative nausea and vomiting (PONV) after propofol anaesthesia compared with other anaesthetics (control). Cumulative data of early (0-6 h) and late (0-48 h) PONV were recorded as occurrence or non-occurrence of nausea or vomiting. Combined odds ratio and number-needed-to-treat were calculated for propofol as an induction or maintenance regimen, early or late outcomes, and different emetic events. This was performed for all control event rates and within a range of 20-60% control event rates. We analysed 84 studies involving 6069 patients. The effect of propofol on PONV was dependent mainly on the method of administration, time of measurement and range of control event rates. When all studies were included the number-needed-to-treat to prevent PONV with propofol was more than 9 when used for induction of anaesthesia and at best 6 when used for maintenance. Within the 20-60% control event rate range, best results were achieved with propofol maintenance to prevent early PONV: the number-needed-to-treat to prevent early nausea was 4.7 (95% confidence interval 3.8-6.3), vomiting 4.9 (4-6.1) and any emetic event 4.9 (3.7-7.1). Within the 20-60% control event rate, of five patients treated with propofol for maintenance of anaesthesia, one will not vomit or be nauseated in the immediate postoperative period who would otherwise have vomited or been nauseated. This may be clinically relevant. In all other situations the difference between propofol and control may have reached statistical significance but was of doubtful clinical relevance. Treatment efficacy should be established within a defined range of control event rates for meaningful estimates of efficacy and for comparisons.     

Association between non-right-handedness and cleft lip with or without cleft palate in a Chinese population

The ori region of an Erwinia stewartii plasmid, pSW1200 (106 kb), has been cloned and sequenced. This region consists of a gene encoding a protein which has 91% similarity and 73% identity with the RepA protein of bacteriophage P1. The ori region also consists of eight copies of 19-bp iterons which are highly homologous to the iterons of P1. Similar to plasmid P1, pSW1200 replicon has a copy number of approximately 1. On the other hand, the copy number increases about ninefold if three of the iterons located downstream from repA gene are deleted. We also demonstrate that pGEM-5Z consisting of a copy of P1 iteron is incompatible with a pSW1200 derivative, pSW1201, suggesting that pSW1200 and P1 DNA are incompatible and both belong to the IncY group.     

Current knowledge in cleft lip and palate treatment from an orthodontist's point of view

The aim of this review was to put new clinical research findings into proper perspectives relative to previously accepted knowledge on treatment of patients with cleft lip and palate. The first part of the paper deals with various aspects of infant orthopedic treatment, such as its influence on primary surgery, maxillary arch form and dimensions, feeding, psychological situation of the parents and speech development. Following parts analyze general maxillofacial growth outcome after surgery and also maxillofacial growth in relation to particular surgical procedures (palatal repair, periosteoplasty/gingivoplasty, bone grafting). The last part of the review discuss the effects of certain orthodontic/orthopedic treatment approaches as well as the role of dental implants in treatment of cleft lip and palate patients.     

Two patients with multiple facial clefts including cleft lip and palate

Report of 2 patients with cleft lip and palate and multiple facial clefts are described using the anatomical classification of Tessier. The first patient was severely polymalformed, with heart, vertebral, and facial malformations. He was examined clinically and with an experimental three-dimensional computer reconstruction that identified an association of a classic cleft lip and palate with cleft nos. 4 and 11 on the right side and cleft nos. 3 and 11 on the left. The second patient, a partial form, with cleft lip and palate and cleft nos. 3 and 11 on the right side, also presented with bilateral cleft no. 7, preauricular tags, and an important microgenia. This second patient was operated for the cleft lip using the principles of Millard and Onizuka with a supplementary Z plasty. We comment on the difficulties of delimitation and treatment when the clefts are very close together and the difference in "quality" of the tissues compared with classic cleft lip and palate.     

Assessment of the patient with cleft lip and palate. A developmental approach

Children with cleft lip and palate require interdisciplinary team care from infancy through adolescence. An understanding of developmental stages allows the cleft palate team to adapt and integrate its services into the rapidly changing life of the child. This article discusses the maturational, developmental stages of childhood and the services the child with cleft lip and palate and the child's family deserve through each stage. Health care providers in all settings may continue to provide appropriate care for all patients with cleft lip and palate, despite the challenges of a changing health care environment, by emphasizing the needs of the child in all developmental stages.     

Analyses of craniofacial and dental morphology in monozygotic twins discordant for cleft lip and unilateral cleft lip and palate

AIMS: To compare the efficiency of flow cytometry and computed image cytometry; and to see if a reliable set of guidelines regarding interpretation of histograms could be drawn up. METHODS: The two methods were applied to a series of 111 formalin fixed renal cell carcinomas. Data generated by both methods were compared. RESULTS: The methods agreed in 85 cases. Hypodiploidy was detected by computed image cytometry in seven cases in which flow cytometry had shown only an aneuploid peak. Aneuploidy in seven in which the corresponding flow cytometry histogram was diploid. There was an overlap in the second peak proportions on flow cytometry histograms between those classed as diploid or tetraploid by computed image cytometry. In six cases the flow cytometry histograms had unacceptably high coefficients of variation and in all of these cases computed image cytometry demonstrated aneuploidy. CONCLUSIONS: Computed image cytometry is particularly useful for clarifying difficult areas in flow histograms--specifically, high coefficients of variation, high G2M phase, as well as possible near diploid aneuploidy and hypodiploidy.     

The effect of muscle repair on postoperative facial skeletal growth in children with bilateral cleft lip and palate

Systems for testing genetic toxicology are components of carcinogenic and genetic risk assessment. Present routine genotoxicity-testing is based on at least 20 years of development during which many different test systems have been introduced and used. Today, it is clear that no single test is capable of detecting all genotoxic agents. Therefore, the usual approach is to perform a standard battery of in-vitro and in-vivo tests for genotoxicity. Work-groups of the European Union (EU), the Organization for Economic Co-operation and Development (OECD), and, very recently, the work-group of the International Conference on Harmonization of Technical Requirements for Registration of Pharmaceuticals for Human Use (ICH) have defined such standard battery tests. These and some currently used supplementary or confirmatory tests are briefly discussed here. Additional test systems for the assessment of genotoxic and carcinogenic hazard and risk are seriously needed. These tests must be more relevant to man than are current assays and less demanding in respect of cost, time and number of animals. Another aspect for reassessment derives from the actual situation in the pharmaceutical industry. Companies have to prepare for the world economy of the 21st century. Therefore, pharmaceutical research is speeding up tremendously by use of tools such as genomics, combinatorial chemistry, high throughput screening and proteomics. Toxicology and genotoxicology need to re-evaluate their changing environment and must find ways to respond to these needs. In conclusion, genetic toxicology needs to answer questions coming from two major directions: hazard and risk identification and high throughput testing.     

Trisomy 13 mosaic presenting as cleft lip and palate

A mosaic trisomy 13 presenting as a case of cleft lip and palate in the newborn is described. However, when the child was admitted to hospital at the age of 6 weeks because of failure to gain weight and a malformation of the great vessels was demonstrated, cytogenetic studies were carried out. The diagnosis of mosaic trisomy 13 (90% normal, 10% trisomic) was established from a leukocyte culture. Since, occasionally, mosaic trisomy 13 may mimic cleft lip and palate in the newborn, cytogenetic studies are indicated in the presence of any additional anomaly.     

One-stage closure of uni- and bilateral cleft lip and palate

A preliminary report of an "all-in-one' one-staged closure of all forms of cleft lip and palate during the first year of life. The one-stage repair of complete uni- and bilateral clefts includes the anatomical reconstruction of soft palate, hard palate closure in two layers, alveoloplasty with bone grafting and lip repair. This surgical technique is described and early results presented.     

Craniofacial morphology in adult patients with unoperated complete bilateral cleft lip and palate

With recent advances in DNA technology, questions have arisen as to how this technology should be appropriately used. In this article, results obtained from a survey designed to elicit attitudes of college students to prenatal testing and gene therapy for human attributes and psychiatric conditions are reported. The eleven hypothetical disease phenotypes included schizophrenia, alcoholism, tendency toward violent behavior, attention deficit/hyperactivity disorder, depression requiring medical treatment, obesity, involvement in "dangerous" sports activities, homosexuality, borderline normal IQ (80-100), proportional short stature, and inability to detect perfect pitch. Most students supported prenatal genetic testing for psychiatric disorders and behavior that might result in harm to others (i.e., tendency towards violent behavior) and found prenatal genetic testing for human attributes less desirable. However, the lack of unilateral agreement or disagreement toward any one condition or attribute suggests the potential difficulties ahead in the quest for guidelines for the application of new technologies available to manipulate the human genome.     

Alveolar bone grafting in unilateral complete cleft lip and palate patients

Autogenous bone graft of an alveolar cleft area has the following advantages: (1) assistance in the closure of buccoalveolar oronasal fistula; (2) provision of bony support for unerupted teeth and teeth adjacent to the cleft; (3) formation of a continuous alveolar ridge to facilitate orthodontic correction of malocclusion; (4) supporting the nostril floor and alar base to improve nasal aesthetics. It has been well accepted in most craniofacial centers as routine procedure in cleft lip and palate rehabilitation. A new surgical technique for alveolar bone grafting has been introduced to the Chang Gung Craniofacial Center since July 1991. It provided a good exposure of the alveolar cleft, primary closure of the fistula and adequate volume of bone graft. A review of 27 consecutive alveolar bone grafting procedures performed in unilateral cleft lip and palate patients from July 1991 to June 1992 was presented. Patients have been followed up for at least 6 months. The alveolar bone graft was evaluated clinically and radiologically at one week, six months and one year after the surgery. The preliminary results indicated that the new surgical technique produced less chance of recurrent fistula, good postoperative gingival height, and improvement of nasal aesthetics. Based on the results of this new study we strongly advocate the use of this new surgical technique.