Presurgical columellar elongation and primary retrograde nasal reconstruction in one-stage bilateral cleft lip and nose repair

We present a new combined approach to primary bilateral cleft lip, nose, and alveolus repair using presurgical nasoalveolar molding combined with a one-stage lip, nose, and alveolus repair. Presurgical alveolar molding is used to bring the protruding premaxilla back into proper alignment with the lateral segments in the maxillary arch. Presurgical nasal molding produces tissue expansion of the short columella and nasal lining. A coordinated surgical approach involves a one-stage repair of the lip, nose, and alveolus. The nasal repair uses a retrograde approach in which the prolabial flap and columella are reflected over the nasal dorsum by continuing the dissection behind the prolabium up the membranous septum and over the septal angle. Tissues are dissected out from between the tip cartilages, and the domes are sutured together in the midline. This method joins a new class of bilateral cleft repairs that place the primary emphasis on correction of the deformity of the nasal tip cartilages.     

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.     

Modified bilateral cleft lip repair with orbicularis reconstruction and prolabial self-elongation

PURPOSE: This article describes the details of a modified method for bilateral cleft lip repair and presents some examples of patients whose condition was repaired by this technique. PATIENTS AND METHODS: Sixty-five patients ranging in age from 2 to 5 years were treated with this modified procedure. The method was characterized by prolabial lengthening and reconstruction of the orbicularis oris muscle in one stage. RESULTS: Satisfactory results were achieved with this method. The Cupid's bow and the central tubercle appeared more natural. None of the patients developed dehiscence of the wound. CONCLUSIONS: This modified procedure is of special value for complete bilateral cleft lip patients with a short prolabium. The long-term effect of muscle reconstruction on maxillary growth remains to be evaluated.     

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.     

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.     

Sagittal craniofacial morphology in repaired unilateral and bilateral complete cleft lip and palate cases

Using guidelines, students independently complete a written preparation for class assignment to develop a knowledge base needed for active participation in one or more class activities. The authors present a two-part method that is key to developing critical-thinking skills, using case studies.     

Correction of the secondary nasal deformity in bilateral cleft lip with a new technique

Bilateral cleft lip often has nasal deformities of various degrees. It is not enough to elongate the nasal columella for such deformities. Based on the thoughts that secondary deformities of the nose and lip should be treated as a whole, the authors introduced a new technique, in which five aspects of the deformities were considered and treated at the same time. Thirteen patients have undergone surgical correction with satisfactory results.     

Photogrammetric comparison of two methods for synchronous repair of bilateral cleft lip and nasal deformity

The principle of synchronous repair of bilateral complete cleft lip and nasal deformity is established, and the techniques are evolving. We undertook photogrammetric comparison of the method described by Mulliken (group I, n = 15) and that described by Trott (group II, n = 10). Facial proportions and angles were measured on preoperative and postoperative photographs using defined anthropometric points. The following criteria were quantified for each test group: nasolabial angle, nasal tip angle and projection, nasal width, columellar length and width, and philtral width. All parameters in both groups were compared against each other and against normal age-matched values. Results were analyzed by t test. The nasolabial angle, nasal tip angle, and nasal width were abnormally wide for both techniques (p < 0.01 for both), and there was no difference between them. Nasal tip projection was greater than normal in both techniques (p < 0.01); group I had significantly greater projection than group II (p = 0.02). Columellar length as a proportion of nasal tip protrusion approached normal in group I but was significantly shorter than normal in group II (p < 0.001). Columellar width, as a proportion of nasal width, was normal for both groups. Philtral width, in proportion to nasal width, was normal in group I and abnormally high in group II (p < 0.001). This difference was significant between the two groups (p < 0.001). In a separate cohort of 10 group I patients, the nasolabial angle was measured on lateral photographs taken 1 year post-repair and at intervals to late childhood and adolescence. Nasolabial angle changed with age from obtuse to normal in 7 of 10 patients followed to 9 years of age and in 5 of 6 patients followed until age 15 years. Columellar and upper labial inclination to the vertical were measured in 7 of these patients at 3 to 4 years and again at 13 to 15 years. There was an increase in inclination of both columella and the upper lip, between the ages of 3 to 4 and 13 to 15 years, reflecting improved lip support and growth of septum and lateral cartilages.     

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.     

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 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.     

Congenital cleft lip and fluctuating dermatoglyphic asymmetry

Buried as a foreign menance more than twenty years ago, exhumed, examined and reinterred once or twice since then, brainwashing has risen again, this time to take its place on the stage of domestic horrors.     

Effect of lip adhesion on labial height in two-stage repair of unilateral complete cleft lip

A lip-adhesion procedure before definitive repair of unilateral complete cleft lip has been widely used but rarely analyzed over the past 25 years. This report is a quantitative prospective assessment of one possible benefit of lip adhesion, an increased vertical height of the cleft labial elements. Lip adhesion was performed on 43 consecutive infants with unilateral complete cleft of the primary palate. Markings for rotation-advancement repair were placed before lip adhesion and again at the time of the complete nasolabial correction. Using a standardized caliper, anthropometric measurements of the vertical height of the medial and lateral lip elements were made from these markings. The relative increase in vertical height of the medial and lateral labial segments during the mean interoperative interval of 3.2 months was calculated. Analysis of these measurements revealed a small disproportionate increase in vertical lip height of the cleft side compared with the normal side after lip adhesion. Discrepancy of the lateral lip height between the cleft and noncleft sides decreased from 3.0 to 2.7 mm (10 percent, p = 0.05), and the prolabial or medial height discrepancy decreased from 3.4 to 2.8 mm (17 percent, p < 0.01). Subgroup analysis of infants whose adhesion was done either at a young age, without premaxillary orthopedics, or with an intact secondary palate, revealed no statistically significant improvement in the labial height measures. It is arguable whether the small relative increase in height of the medial and lateral cleft elements justifies lip adhesion before definitive repair of unilateral complete cleft lip.     

A model for the cleft lip nasal deformity

The underlying pathology of the cleft lip nasal deformity has yet to be fully realized, and cleft lip rhinoplasty continues to challenge the reconstructive surgeon. A new model is proposed, which is composed of elements that represent known anatomical structures of the nose. These structures are considered elemental to the mechanism of the primary cleft lip nasal deformity. The lobule is reduced to four arches. Five points on the skull provide foundations for these arches, which react interdependently to extrinsic forces and positional change. When certain changes are imposed on the model, predictable alterations in the configuration of the model imitate the observed deformities in the spectrum of the cleft lip nasal deformity, unilateral and bilateral, mild through severe. The model is described with illustrations, anatomic dissection, physical models, and selected clinical cases. A better understanding of the mechanisms of the cleft nasal deformities can be obtained through analysis of the model.     

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.     

The child with lip, maxillary, palatal cleft

Clefting of lip, alveolus and palate may occur in multiple variations. It causes aesthetic and functional detractions. Soft palate clefts may result in hearing-, speech- and swallowing-disorders. Therefore the otolaryngologist is a very important member in the interdisciplinary team directory. A cleft-palate child belongs to an interdisciplinary consulting hour in special hospitals, where different medical specialties are involved. Most important for a sufficient medical rehabilitation are maxillofacial surgery, otolaryngology, paediatrics, plastic surgery, speech therapy, psychology and human genetics. Also many other specialties may be involved. The cleft demands a complete follow up from the child's birth until it is grown up. Hearing disorders are caused by eustachian tube disfunction. There is a high prevalence of hearing loss and middle ear diseases in cleft palate patients. Hearing losses due to middle ear effusions in the very young child. Without therapy up to 50% of the cleft palate population will develop chronic middle ear diseases with and without cholesteatomas. Early and consequent therapy with myringotomy and insertion of a tympanostomy ventilation tube is necessary and helps to avoid chronic hearing problems. There is no general accepted system of speech disorders in cleft palate patients because of the difference in shaping of the cleft and rehabilitation development. Essential for speech rehabilitation are an intact velopharyngeal system and a keen sense of hearing. Both of it is disturbed in cleft palate children. Speech disorders are treated by speech therapists with prior consultation of the interdisciplinary team. The author presents a system of primary, secondary and tertiary speech disorders in cleft palate children. Primary speech disorders are caused by faulty velopharyngeal valving, offering in hypernasality, weak plosives, fricatives and affricates. Secondary speech disorders are substitute mechanisms for plosives, nasal and pharyngeal sounds. Tertiary speech disorders are hyper- and hypofunctional dysphonias following primary and secondary speech dysfunctions.     

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.     

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.