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.     

Free flap closure of recurrent palatal fistula in the cleft lip and palate patient

Recurrent palatal fistulas present a particularly vexing problem for the cleft surgeon. In this setting, the cycle of repair followed by breakdown results in increasing scar formation with associated soft tissue contracture and a resultant increase in fistula size. This pernicious cycle of events renders random local tissue transfers obsolete. As such, the cleft surgeon must look to tongue flaps or local axial pattern flaps as a means of bringing well-vascularized, pliable tissue into the defect. Although this approach has been the standard of care for the last few decades, we believe that the modern-day success rates of free tissue transfers (95%) make them a viable, one-stage means of closing these defects. In this report we present our clinical experience with recurrent palatal fistulas and highlight the effective use of the dorsalis pedis-first dorsal metatarsal artery free flap as a means of repair.     

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.     

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.     

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.     

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.     

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.     

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.     

Role of the pediatrician in the treatment of children with cleft lip and palate

The authors studied the relation between human central dopamine (DA) metabolism and the clinical effects of neuroleptics. The neuroleptic-induced increase in central DA turnover (an indicator for the degree of DA receptor blocking) was found to be positively correlated with the therapeutic effect of neuroleptics and the development of hypokinetic-rigid symptoms. This supplies a direct argument in support of the contention that DA antagonism is related to the occurrence of clinical effects. The authors also found indications that neuroleptics of different chemical types do not significantly differ in their intrinsic ability to provoke hypokinetic-rigid symptoms, that development of these symptoms depends on the patient's individual susceptibility, and that individual susceptibility is based on relatively low DA turnover.     

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.     

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.     

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.