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A new cleft palate repair.

Cleft palate can be repaired by a palatal mucoperiosteal flap, but we have devised a new technique for cleft palate repair using a wide mucoperiosteal flap with an alveolar ridge mucosa and a cleft margin flap. This technique leaves the mucosa of the nasal cavity and vomer intact. The results include (1) good velopharyngeal competence and normal articulation, (2) few cases of fistula formation, and (3) minimal maxillary retardation.

Age Factors↗

Results of cleft palate repair with the double-reverse Z-plasty performed by residents.

The double-reverse Z-plasty (Furlow) repair of soft palate clefts has the advantages of lengthening the soft palate while reconstructing the palatal muscle sling to optimize velopharyngeal function. Because soft tissue from the hard palate is not mobilized to lengthen the soft palate, this technique has an additional theoretic advantage of minimizing disturbances of maxillofacial growth. Cited disadvantages of the technique include increased technical difficulty and operating time compared with other procedures. We performed a retrospective study of 20 consecutive Furlow repairs of the soft palate performed by residents under supervision of one attending surgeon between 1989 and 1992. All children had complete cleft of the soft palate; 14 had incomplete cleft and six had complete cleft of the hard palate, which were repaired by a modified Von Langenbeck technique or with vomer flaps. Mean age of patients was 15 months, and mean operating time (including placement of pressure-equalizing tubes) was 162 minutes, with an average estimated blood loss of less than 25 ml. Average time until adequate oral intake was achieved was 1.7 days, and duration of hospitalization averaged 2.4 days. Immediate postoperative complications included transient tongue edema in one patient and atelectasis and pneumonia in one patient with multiple congenital anomalies. Delayed postoperative complications included symptoms of sleep apnea in four patients, which subsequently improved after tonsillectomy and/or superior adenoidectomy. Fistulas occurred in two patients (10%), which compares favorably with published results of other techniques. Preliminary functional evaluations reveal that all repairs appear to have adequate length and good mobility; speech evaluations are in progress.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗

[Fetal mortality in families with labio-palatal clefts].

Fetal mortality in 110 families of patients with cleft lip with or without cleft palate is analyzed and correlated with liability. Because there was no significant difference, authors discussed these findings with references to multifactorial two-threshold hypothesis.

Abnormalities, Multiple↗

[Articulation and nasalization in palatal cleft].

The author studied 189 patients after operations for cleft palate who had not had earlier logopedic rehabilitation. The range of disturbances was presented as a sum of abnormal articulations against a background of functional disturbances of various degree. Preponderance of articulation disturbances was noted--84.7% in relation to nasalization--75.1%. Patients with moderate and high-grade nasalization achieved evidently worse results of speech rehabilitation and had a much smaller possibility of autocorrection in relation to those with--out impairing nasalization. Moreover, articulation changes were found in patients without nasalization and with low-grade nasalization, which suggests persistence of habits, and the need for early exercises to prevent the formation of harmful compensations.

Articulation Disorders↗

[Ultrasonic prenatal diagnosis of lip and palate cleft. Report of 10 cases].

Hare-lips and cleft palates are the most frequent manifestations of facial malformations (approximately 1 in 1,000 deliveries). The authors point out their technique for ultrasound assessment of the normal and pathological fetal face in the antenatal period. The antenatal examination gives an excellent diagnostic approach of the site and the nature and the extent of the abnormality. The screening for facial defects can be carried out routinely when studying the morphology in the second trimester of pregnancy or when there are other indications for doing this. Although the malformation is usually an isolated one, the management of these cases really depends mainly on any associated malformations than on the normality of the karyotype.

Cleft Lip↗

[Ear manifestations in adolescents after closure of lip-jaw-palate- or isolated palatal clefts].

We examined two groups of teenagers who had been surgically treated as small children for cleft palate. Most patients were between 13 and 21 years of age. One group had been looked after by the Dept. of Orthodontics at the University of Erlangen-Nürnberg, the other by the Dept. of Orthodontics at the University of Rostock. There were differences in sequence and time of the surgical closure between the two departments. Additionally, 60% of the people treated in Rostock had a velopharyngoplastic, which was rarely the case in Erlangen. In both groups only a few patients had been seen by an ENT-doctor regularly. Only some patients had been previously treated with tubes. There was one patient in each group with a bilateral, most likely genetically determined, sensorineural hearing loss. In Erlangen we examined 66 teenagers (132 ears). Six ears had been previously treated with one or more tympanoplasties. 10 ears needed further treatment due to a seromucotympanon, adhesions, perforations of the ear drum, suspicion of cholesteatoma or insufficient improvement of hearing after previous tympanoplasty. Another 18 ears showed signs of former inflammations. The control group in Rostock included 63 patients (i.e. 126 ears). 14 of the ears examined had undergone one or more tympanoplasties previously. 13 other ears needed further treatment for seromucotympanon, adhesions, perforations of the ear drum, insufficient improvement of hearing after tympanoplasty or cholesteatoma. Residuals due to prior inflammations were found in another 26 ears. Possible reasons for the different occurrence of middle ear problems in both groups are discussed.

Acoustic Impedance Tests↗

Surgery for speech in cleft palate patients.

INTRODUCTION: Superiorly based pharyngeal flaps and sphincter pharyngoplasties are the two main possibilities for the surgical treatment of hypernasality in velopharyngeal dysfunction. Videonaspharyngoscopy and multi-view videofluoroscopy can provide anatomical and physiological data for planning these surgical procedures for correcting hypernasality. AIM: This study was undertaken to assess the planning and outcome of pharyngeal flaps and sphincter pharyngoplasties for correcting velopharyngeal insufficiency. The surgical techniques were customized according to the findings of videonasopharyngoscopy and multiview videofluoroscopy. MATERIALS AND METHODS: Seventy patients with repaired palate clefts and residual velopharyngeal dysfunction were studied. The patients were randomly divided into two groups. The first group received a pharyngeal flap. The second group received a sphincter pharyngoplasty. Both procedures were individually customized according to the findings of videonasopharyngoscopy and multi-view videofluoroscopy. RESULTS: There was a non-significant difference (P >0.05) between the mean size of preoperative velopharyngeal closure gap between the two groups of patients (mean=27.5%; S.D.=7.7% versus mean=28.3%; S.D.=5.9%). Postoperatively, velopharyngeal dysfunction was completely corrected in 89% of the cases from group 1, and in 85% of the cases from group 2. There was a non-significant difference (P >0.05) between the success rate for correcting VPI in both groups of patients. CONCLUSIONS: Pharyngeal flap and sphincter pharyngoplasty seem to be safe and reliable procedures for treating residual velopharyngeal dysfunction. Although not all the patients studied for this paper achieved complete closure after the surgical procedures, all of them showed a reduction of the size of the velopharyngeal closure defect. The planning of the surgical procedure, in order to match the postoperative structure to the preoperative velopharyngeal dimensions and movements visualized through Videonasopharyngoscopy and videofluoroscopy, seems to be the most important aspect of the surgery for correcting residual velopharyngeal dysfunction.

Child↗

Consequences of unremitting middle-ear disease in early life. Otologic, audiologic, and developmental findings in children with cleft palate.

To learn whether chronic otitis media with effusion during early life has lasting otologic, audiologic, or developmental consequences, we evaluated 24 closely matched pairs of children with repaired palatal clefts whose treatment had been equivalent except with regard to persistent otitis media during early life. One group had undergone early (mean age, 3.0 months) myringotomy with placement of tympanostomy tubes, followed by assiduous monitoring and an aggressive treatment program to maintain ventilation in the middle ear. The other group had undergone initial myringotomy later (mean age, 30.8 months) or not at all (two subjects) and presumably had had continuous middle-ear effusion throughout most or all of the first few years of life. Eardrum scarring was equal in both groups. Hearing acuity and consonant articulation were impaired in both groups, but hearing acuity was less impaired (P = 0.05 to 0.10) and consonant articulation significantly less impaired (P = 0.03) in the group undergoing early myringotomy. Mean verbal, performance, and full-scale IQs and scores on psychosocial indexes were normal in both groups and did not differ significantly between the groups. These findings support the hypothesis that early, longstanding otitis media may result in impairment of hearing and of speech, but they do not support the hypothesis that cognitive, language, and psychosocial development are adversely affected.

Audiometry, Pure-Tone↗

Otitis media, cleft palate, and middle ear ventilation.

Chinchillas with unilateral tympanostomy tubes in place underwent palate-clefting in an effort to determine the histologic and bacteriologic effects of using tympanostomy tubes in the treatment of otitis media. The tympanostomy tube appeared to almost totally eliminate the occurrence of middle ear effusion but had much less, if any, effect on eliminating the middle ear inflammation which occurs in the clefted chinchilla.

Animals↗

Accuracy of information reported by parents and children evaluated by a cleft palate team.

Children with palatal clefts and their parents were interviewed to determine what information they had about the cleft and its management. Their responses were compared with the standards of 17 team members from seven disciplines. Both parent and child were consistently provided information about clefts that they were expected to remember. The children's responses met the standards 67 percent of the time. Their parents' responses met the standards 80.6 percent of the time. Parents accurately predicted their children's responses 92 percent of the time.

Adolescent↗

[Changes in autonomic homeostasis during anesthesia induction in patients operated on for congenital cleft palate].

Variation pulsometry was used to study vegetative homeostasis changes over the course of induction anesthesia and tracheal intubation in children operated on for palatal clefts. The same premedication scheme was employed in all the patients. The same neuroleptic doses were administered in induction anesthesia. Induction anesthesia parallel with electroanalgesia with an Electronarcol-1 apparatus helped reduce the promedol dose necessary for adequate anesthesiologic protection from 1.2-1.4. to 0.2-0.4 mg/kg. This was associated with the minimal changes in autonomic nervous system functioning, evidencing the efficacy of electroanalgesia in induction anesthesia for this patient population.

Anesthesia, Endotracheal↗

[Lipid peroxidation indices in children with congenital cleft palate].

Observed were 66 children with congenital palate clefts and a control group of 25 children. The data suggest that the disease involves the lipid peroxidation disorders and impairment of the antioxydative defence. The results can be used for designing the antioxydant and membrane-stabilizing therapies in out-patient departments.

Cleft Palate↗

[Stereocephalometric diagnosis of penetrating unilateral cleft palate].

The issues concerning precise information useful to clarify morphological characteristics of maxillofacial disorders in children are discussed. In children with congenital facial pathology this approach allowed to single out the syndrome of opened unilateral palate cleft.

Adolescent↗

Weight gain in children with cleft palate from birth to two years.

The weights from birth to 2 years of 77 babies with palatal clefts were compared with the weights of normal babies (Pomerance, 1979). Babies with clefts did not differ from normal infants in mean birth weight, but they lagged behind thereafter. Males were more impaired than females.

Birth Weight↗

Harelip and cleft palate conditions in chick embryos following local destruction of the cephalic neural crest. A preliminary note.

With the aid of micro-laser irradiation, in a total of 156 chick embryos unilaterally (a part of) the cephalic neural crest paralleling the posterior portion of the prosencephalon and mesencephalon, was eliminated. As a result of this treatment, 2 out of 22 embryos, studied 24 hours after irradiation, showed a considerable degree of underdevelopment of the mesenchyme in the homolateral first branchial arch. At the age of 7 days (6 days after irradiation) 11 of the 44 surviving embryos proved to have developed a homolateral harelip condition. In 2 of these embryos, a wide palatal cleft was observed as well. These results demonstrate that facial clefts may develop as a result of a local deficiency of neural crest cells.

Animals↗

[Genetic polymorphisms of GSTM1 and GSTT1 in mothers of children with isolated cleft lip with or without cleft palate].

Orofacial clefts are one of the most common developmental malformations (OMIM #119530, #119540), which aetiology is very complex and associated with both genetic and environmental factors. One of the main environmental factors increasing the risk of having a child with an oral cleft is maternal cigarette smoking. The effect of maternal smoking may be modified by genes involved in biotransformation of toxic compounds derived from tobacco. Very important role in this process is played by S-glutathione transferase M1 (GSTM1) and S-glutathione transferase T1 (GSTT1) which conjugate glutathione with xenobiotics and promote their removal from human body. The aim of this study was frequency analysis of homozygous deletion of GSTM1 and GSTT1 genes in women having infants with ororfacial clefts and in control group. In this case-control study we used DNA isolated from peripheral blood lymphocytes of 121 mothers having children with isolated cleft lip with or without cleft palate and from 80 control mothers. The common deletions of GSTM1 and GSTT1 were determined by polymerase chain reaction and agarose gel separation. The prevalence of genotype GSTM1(-)/GSTT1(-) was higher in mothers of children with cleft (6.6%) compared to controls (3.7%, p>0.05). Among multiparous mothers of children with cleft (one child with birth defect and at least 2 healthy children, mean number of children - 4) the prevalence of genotype GSTM1(+)/GSTTI(+) was significantly reduced (31.0% vs. 58.7%, p=0.011). A significantly increased risk of giving birth to a child with cleft was found in multiparous subgroup of mothers with GSTM1(-)/GSTT1(-) and GSTM1(-)/ GSTT1(+) genotypes as compared to those with GSTM1(+)IGSTT1(+) genotype (OR 6.96; 95%CI: 1.33-36.57, p<0.01 i 3.05; 95%CI: 1.153-8.05, p<0.02 respectively). We did not observe a significant correlation between smoking status, GSTM1/GSTT1 genotypes and risk of orofacial clefts. Our results suggest that homozygous deletions of GSTM1 and GSTT1 in mother genome might increase the risk of having child with cleft lip with or without cleft palate.

Child↗

Nasal airflow and olfactory function after the repair of cleft palate (with and without cleft lip).

OBJECTIVE: The objective of this study was to examine nasal airflow and olfactory functions in patients with repaired cleft palate compared with matching normal controls. STUDY DESIGN: The all-cleft group consisted of 25 patients with hard palate cleft comprising 15 patients with unilateral cleft palate and lip (UCLP); 2 with CP but no cleft lip (UCLP subgroup) and 8 patients with bilateral cleft lip and palate (BCLP subgroup). All had had surgical correction of the palate in infancy. The control group consisted of 20 nonaffected orthodontic patients. The median age of both groups was 14 years. The tests included the following: (1) nasal airflow measured by anterior rhinomanometry, (2) smell threshold for isoamyl-acetate determined using a 3-way forced choice method, (3) a self-administered questionnaire regarding the subjective perception of smell sense function, and (4) orthonasal and retronasal smell identification (correct/incorrect) and hedonics using visual analog scale (VAS). RESULTS: The respective test results follow. (1) When compared with the control group, the total airflow in the UCLP subgroup was significantly lower especially on the affected side; while in the BCLP subgroup it was lower than in the control group bilaterally. No significant difference was found between the cleft side of UCLP and BCLP subgroups. (2) The smell threshold of the UCLP subgroup was significantly higher than that of the control group and BCLP subgroup. No significant differences were found between right and left nostrils within the BCLP patients and between them and the control group. (3) No difference was found between the groups regarding the subjective perception of smell. (4) No significant differences were found between the UCLP and BCLP subgroups and between the all-cleft group and the control group, except for one item, regarding orthonasal and retronasal smell identification and hedonics. CONCLUSION: Although nasal airflow is significantly lower and the smell threshold higher on the cleft side, the day-to-day function of the sense of smell of cleft patients is similar to that of normal controls.

Adolescent↗

A prospective evaluation of the prevalence of submucous cleft palate in patients with isolated cleft lip versus controls.

Although there is an established relationship between cleft lip and overt cleft palate, the relationship between isolated cleft lip and submucous cleft palate has not been investigated. To test the hypothesis that patients with isolated cleft lip have a greater association with submucous cleft palate, a double-armed prospective trial was designed. A study group of 25 consecutive children presenting with an isolated cleft lip, with or without extension through the alveolus but not involving the secondary palate, was compared with a control group of 25 children with no known facial clefts. Eligible patients were examined for the presence of physical criteria associated with classic submucous cleft palate, namely, (1) bifid uvula, (2) absence of the posterior nasal spine, and (3) zona pellucida. Nasoendoscopy was subsequently performed just after induction of general anesthesia, and the findings were correlated with digital palpation of the palatal muscles. Patients who did not satisfy all three physical criteria and in whom nasoendoscopy was distinctly abnormal relative to the control group were classified as having occult submucous cleft palate. Classic submucous cleft palate was found in three study group patients (12 percent), all of whom had flattening or a midline depression of the posterior palate and musculus uvulae on nasoendoscopy and palpable diastasis of the palatal muscles under general anesthesia. An additional six study group patients (24 percent) had similar nasoendoscopic criteria and palpable diastasis of the palatal muscles; they were classified as having occult submucous cleft palate. No submucous cleft palate was identified in the control group. Seventeen patients in the study group had an alveolar cleft with a 53 percent (9 of 17) prevalence of submucous cleft palate. In the present study, classic submucous cleft palate in association with isolated cleft lip was 150 to 600 times the reported prevalence in the general population. All children with an isolated cleft lip should undergo peroral examination and speech/resonance assessment no later than the age of 3 years. Any child with an isolated cleft lip with velopharyngeal inadequacy or before an adenoidectomy should be assessed by flexible nasal endoscopy to avoid missing an occult submucous cleft palate.

Adolescent↗