1/15. Neuro-ophthalmic complication after maxillary surgery.We report an unusual case of ophthalmoplegia after maxillofacial surgery. A thirteen year old girl with unilateral left cleft lip and palate underwent maxillary advancement by distraction osteogenesis. Postoperatively she developed diplopia. The diplopia was not due to orbital lesions, most often seen after maxillofacial surgery, but to a haemorrhage posterior to the cavernous sinus.- - - - - - - - - - ranking = 1keywords = cleft, palate (Clic here for more details about this article) |
2/15. Facio-genito-popliteal syndrome presenting with bilateral choanal atresia and maxillary hypoplasia.The most classic characteristics of facio-genito-popliteal syndrome are cleft lip and/or palate, anomalies of the external genitalia and popliteal pterygium. A case of facio-genito-popliteal syndrome with bilateral choanal atresia and maxillary hypoplasia is presented. No previous report of such an association was found in the literature.- - - - - - - - - - ranking = 1keywords = cleft, palate (Clic here for more details about this article) |
3/15. Bilateral Tessier no. 4 facial cleft with left eye anophthalmos: a case report.Craniofacial clefts are very rare and manifest in a variety of patterns. Tessier classified these clefts in 1973 and numbered them 0 to 14. Tessier No. 4 Facial cleft is a rare variant of craniofacial cleft. Not more than 50 cases are reported in world literature, amongst which only 5 cases are true bilateral in nature. However, combinations of two different variants are not very uncommon. A case of male Indian child aged 4 years with a true bilateral Tessier No. 4 Facial cleft is reported. He also had anophthalmos of the left eye. This is probably the first case in which true bilateral Tessier No. 4 Facial cleft with anophthalmos of one eye is noticed.- - - - - - - - - - ranking = 8.3850747739083keywords = cleft (Clic here for more details about this article) |
4/15. prenatal diagnosis of partial monosomy 18p(18p11.2-->pter) and trisomy 21q(21q22.3-->qter) with alobar holoprosencephaly and premaxillary agenesis.A prenatal diagnosis of partial monosomy 18p(18p11.2-->pter) and trisomy 21q(21q22.3-->qter) in a fetus with alobar holoprosencephaly (HPE) and premaxillary agenesis (PMA) but without the classical down syndrome phenotype is reported. A 27-year-old primigravida woman was referred for genetic counselling at 21 weeks' gestation due to sonographic findings of craniofacial abnormalities. Level II ultrasonograms manifested alobar HPE and median orofacial cleft. cytogenetic analysis and fluorescence in situ hybridization (FISH) on cells obtained from amniocentesis revealed partial monosomy 18p and a cryptic duplication of 21q,46,XY,der(18)t(18;21)(p11.2;q22.3), resulting from a maternal t(18;21) reciprocal translocation. The breakpoints were ascertained by molecular genetic analysis. The pregnancy was terminated. autopsy showed alobar HPE with PMA, pituitary dysplasia, clinodactyly and classical 18p deletion phenotype but without the presence of major typical phenotypic features of down syndrome. The phenotype of this antenatally diagnosed case is compared with those observed in six previously reported cases with monosomy 18p due to 18;21 translocation. The present study is the first report of concomitant deletion of HPE critical region of chromosome 18p11.3 and cryptic duplication of a small segment of distal chromosome 21q22.3 outside down syndrome critical region. The present study shows that cytogenetic analyses are important in detecting chromosomal aberrations in pregnancies with prenatally detected craniofacial abnormalities, and adjunctive molecular investigations are useful in elucidating the genetic pathogenesis of dysmorphism.- - - - - - - - - - ranking = 0.83850747739083keywords = cleft (Clic here for more details about this article) |
5/15. Nasomaxillary hypoplasia and severe orofacial clefting in a child of a mother with phenylketonuria.The offspring of mothers with untreated or poorly controlled phenylketonuria (PKU) (McKusick 261600) are at risk of having congenital anomalies including microcephaly, congenital heart defects and developmental delay (Rouse et al 1997). We report a child born to a mother with poorly controlled PKU and suggest that the facial abnormalities seen in this child could be part of the specturn of maternal PKU embryopathy.- - - - - - - - - - ranking = 3.3540299095633keywords = cleft (Clic here for more details about this article) |
6/15. Composite Z plasty for cicatricial ectropion of Tessier III cleft.Tessier III clefts represent one of the most difficult and challenging malformations of the face to repair. ectropion caused by a Tessier III cleft may be secondary to a vertical loss of both the anterior and posterior lamellae of the lower eyelids.A composite Z plasty to treat recurrence of cicatricial ectropion of the lower eyelids in Tessier III cleft is described. This is not only a technically easy and effective surgical method but also has a short operation time. To the best of the authors' knowledge, this is the first report of the application of a composite Z plasty in the successful treatment of ectropion.- - - - - - - - - - ranking = 5.8695523417358keywords = cleft (Clic here for more details about this article) |
7/15. Direct skeletal traction for Le Fort I halo distraction replacing an intraoral dental splint and connecting traction hook.To obviate dental inconveniences after Le Fort I halo distraction using an intraoral dental splint and connecting traction hook, the authors initiated direct skeletal traction using an traction wire at the parapyriformis buttress area. Halo distraction using this procedure was conducted for 11 cleft lip and palate patients (age range, 13-21 years; 6 females and 5 males). Distraction amount ranged from 11 to 15 mm. A satisfactory occlusion was obtained in all patients. All 11 patients complained of pain during the distraction period, but it was controlled by regular oral intake of the usual amount of analgesics. No other particular complications were encountered during the postoperative follow-up of 8 to 18 months. This form of direct skeletal traction proves effective for Le Fort I halo distraction.- - - - - - - - - - ranking = 1keywords = cleft, palate (Clic here for more details about this article) |
8/15. A surgical conundrum: Tessier number 4 cleft.OBJECTIVE: Among the rarest of the craniofacial clefts is the Tessier no. 4 cleft; and hence little has been published about its management and treatment. Complete forms of the cleft yield poor surgical results because of the shortened oculoalar and oculo-oral distance and inadequate soft and bony tissue. Multiple sequential corrective operations are required. A primary early concern is the protection of the eye, particularly in severe forms in which corneal exposure occurs. This article presents two cases of Tessier no. 4 clefts, one unilateral and the other bilateral, and discusses the problems encountered during their surgical and postoperative managements.- - - - - - - - - - ranking = 6.7080598191266keywords = cleft (Clic here for more details about this article) |
9/15. Perinatal findings and molecular cytogenetic analysis of de novo partial trisomy 16q (16q22.1-->qter) and partial monosomy 20q (20q13.3-->qter).OBJECTIVES: To present the perinatal findings and molecular cytogenetic analysis of de novo partial trisomy 16q and partial monosomy 20q and a review of the literature. CASE AND methods: Obstetric ultrasound at 33 weeks' gestation revealed intrauterine growth restriction (IUGR) and dolichocephaly in a 27-year-old primigravid woman. Prenatal cytogenetic diagnosis was not offered because of the late stage of gestation. A 2800-g male baby was delivered at 41 weeks' gestation by cesarean section because of fetal distress. The infant postnatally presented characteristic craniofacial dysmorphism, hypotonia, cleft palate, congenital heart defects, a subependymal cyst, and hypospadia. cytogenetic analysis revealed an additional material attached to the terminal region of chromosome 20q. The parental karyotypes were normal. spectral karyotyping (SKY), fluorescence in situ hybridization (FISH), and polymorphic dna markers were used to investigate the origin of the de novo aberrant chromosome. RESULTS: SKY using 24-color probes, FISH using specific 16p, 16q, 20 centromeric, and 20q telomeric probes, and polymorphic dna marker analysis confirmed maternal origin of the duplication of distal 16q and the deletion of terminal 20q. karyotype of the proband was designated as 46,XY.ish der(20)t(16;20)(q22.1;q13.3)(SKY ,16qTEL ,20qTEL-). CONCLUSIONS: Partial trisomy 16q (16q22.1-->qter) and partial monosomy 20q (20q13.3-->qter) may be associated with the perinatal findings of IUGR, dolichocephaly, hypotonia, cleft palate, congenital heart defects, a subependymal cyst, and hypospadia. SKY, FISH, and genetic marker studies help in delineating the parental origin and the regions of the deletion and duplication in the de novo unbalanced translocation.- - - - - - - - - - ranking = 2.6441707287332keywords = cleft, cleft palate, palate (Clic here for more details about this article) |
10/15. sleep disturbances detected by a sleep apnea monitor in craniofacial surgical patients.sleep apnea in craniofacial surgery was investigated. Between January 1999 and December 2003, 18 patients were measured at an at least 6-month interval before and after surgery. Eight patients underwent palatoplasty for cleft palate, and the other 10 patients underwent orthognathic surgery, syndromic craniosynostosis, and postpharyngeal flap surgery. All patients included in the study demonstrated clinical signs of obstructive sleep apnea, such as snoring and cessation of breathing during sleep. An apnomonitor was used for presurgical and postsurgical sleep apnea status by measuring: 1) position during sleep; 2) percutaneous oxygen saturation; 3) respiratory analysis, such as the type of apnea-hypopnea, frequency of the events, and duration of apnea-hypopnea; 4) heart rate; and 5) snore analysis, such as trains, time, mean, and minimal and maximal amplifications.The apnea-hypopnea index (AHI) was significantly improved after surgery, especially in cases other than palatoplasty (7.4 /- 8.73/h and 1.6 /- 0.43/h, before and after surgery, respectively; P < 0.05 excluding palatoplasty). The percentage of snoring to total sleep was also improved significantly (22.4 /- 19.74% and 9.0 /- 8.54%, before and after surgery, respectively; P < 0.01 in all patients).Therefore, changes in sleep apnea parameters were elucidated in craniofacial surgery. Palatoplasty did not necessarily worsen the sleep apnea status, although there were snoring and anatomic abnormalities. Detachment of the pharyngeal flaps improved sleep apnea, and bimaxillary advancement was effective in normalizing sleep apnea.- - - - - - - - - - ranking = 1.3220853643666keywords = cleft, cleft palate, palate (Clic here for more details about this article) |
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