A Case of Cleft Lip and Palate Associated with Seckel Syndrome

2004 ◽  
Vol 41 (2) ◽  
pp. 202-205 ◽  
Author(s):  
Jyotsna Murthy ◽  
Krishna G. Seshadri ◽  
Padmasani Venkat Ramanan ◽  
Arvind Rajamani ◽  
Altaf Hussain

Objective We report an unusual association of complete cleft of the primary and secondary palate with Seckel syndrome. Seckel syndrome is a very rare syndrome, with only 60 reported cases in the medical literature. It is an autosomal recessive disorder characterized by birdlike face, intrauterine growth retardation, dwarfism, and microcephaly. This young child of 5 years had a successful cleft lip repair under general anesthesia. The main features of the syndrome and the technical problems of anesthesia and surgery are discussed.

1993 ◽  
Vol 30 (2) ◽  
pp. 227-230 ◽  
Author(s):  
Andrew Mccance ◽  
David Roberts-Harry ◽  
Martyn Sherriff ◽  
Michael Mars ◽  
William J.B. Houston

The study models of a group of adult Sri Lankan patients with clefts of the secondary palate were investigated. Tooth-size and arch-dimension comparisons were made with a comparable control group. Significant differences were found between the cleft and control groups in tooth sizes, chord lengths, and arch widths. The cleft group dimensions were generally smaller than those of the control group. Overjets were larger in the cleft group.


2021 ◽  
pp. 105566562098280
Author(s):  
Robin A. Tan ◽  
Frans J. Mulder ◽  
Roderic M. F. Schwirtz ◽  
David G. M. Mosmuller ◽  
Henrica C. W. De Vet ◽  
...  

Objective: To gain more insight into the assessment of “atypical” nasal and lip appearance outcomes compared to “typical” appearance outcomes after unilateral cleft lip and palate (UCLP) repair, when judged by professionals, patients with repaired UCLP, and laypeople. Design: An online survey containing 3 series of photographs with various degrees of “typical” and “atypical” nasal and lip appearance outcomes after UCLP repair was sent to 30 professionals, 30 patients with repaired UCLP, and 50 laypeople in 2 countries. Participants were instructed to rank the photographs from excellent to poor based on overall appearance. Mean rank positions of photographs were analyzed and differences in mean rank score between “typical” and “atypical” results were assessed using a T-test. Agreement of ranking between the 3 groups was assessed with an analysis of variance analysis. Setting: Amsterdam UMC, location VUmc, Netherlands and Boston Children’s Hospital, Boston, USA. Patients: Photographs of 6- to 18-year-old patients with repaired UCLP. Results: “Atypical” appearance outcomes were ranked significantly less favorably (small nostril: P = 0.00; low vermillion border: P = 0.02; whistling deformity: P = 0.00) compared to “typical” outcomes. Difference between professionals, patients and laypeople in rank positioning the photographs was not statistically significant ( P = 0.89). Conclusions: Noses with a smaller nostril and lips containing a whistling deformity were perceived as poorer outcome compared to the “typical” results. Professionals, patients, and laypeople are in agreement when assessing these outcomes.


2021 ◽  
pp. 105566562098275
Author(s):  
Robertus Arian Datusanantyo ◽  
Magda Rosalina Hutagalung ◽  
Sitti Rizaliyana ◽  
Djohansjah Marzoeki

Objective: This study aimed to measure and analyze the outcome of primary unilateral cleft lip repair. Design: Observational cohort study. Setting: Surabaya Cleft Lip and Palate (CLP) Center, a major referral center for the Eastern part of Indonesia, affiliated with a tertiary center. Patients, Participants: From 69 patients who met the inclusion criteria, we excluded 31 patients who were more than 2 years of age and were operated on by junior residents under supervision. Interventions: We performed anthropometric measurements of the patients on photographs taken before, immediately after, and a year after the surgery. Main Outcome Measure(s): This study measured nasal width, vertical lip height, horizontal lip length, and philtral height ratios. Results: While nasal width and philtral height ratios decreased significantly ( P = .000 and P = .000, respectively) reaching symmetry immediately after surgery, the horizontal lip length, and vertical lip height ratios remained unchanged ( P = .862 and P = .981, respectively). A year after surgery, the nasal width and horizontal lip length ratios increased significantly ( P = .017 and P = .006, respectively), while philtral height and vertical lip height ratios remained unchanged ( P = .927 and P = .138, respectively). There was no difference in the ratios based on the initial size and completeness of the cleft. Conclusion: In Surabaya CLP Center, the symmetry of nasal width, philtral height, horizontal lip length, and vertical lip height were achieved by the unilateral cleft lip repair despite the initial size and completeness of the cleft.


2017 ◽  
Vol 3 (2) ◽  
pp. 52-60
Author(s):  
Rani Septrina ◽  
Gentur Sudjatmiko

Background: Cheiloplasty, the earliest surgical procedure in cleft lip and palate patient, has impact on functional and aesthetical appearance1. The Gentur’s technique is a method of cleft lip surgery that has been developed by him and has been used in RSUPN Cipto Mangunkusumo/Faculty of Medicine Universitas Indonesia2. It uses the rotation-advancement, small triangular, preventing notching concepts with some other details to overcome the wide cleft. This study was conducted to answer whether the Gentur’s technique gives symmetrical result in anthropometric measurements. Methods: Cross sectional analytic study will be taken from medical record in 14 unilateral cleft lip patients underwent cheiloplasty procedure. Direct anthropometric data before and after procedure were analyzed using SPSS17. Anthropometric data such as cupid’s bow, vertical height, horizontal height, vermillion and nostril were noted. Results: From 14 patients, we found 9 patients who underwent surgery in 3 months of age (64.3%) are mostly female (n=9, 64.3%), have complete defect (n=12, 85.8%) and left sided defect (n=8, 57.1%). Gentur’s technique is able to produce significant lip and nose symmetry (CI 95%, pvalue <0.005) in cupid’s bow, vertical height, horizontal height, thickness of vermillion and nose. By doing this technique, the author is able to create good lip and nose symmetry (78.57%) even in wide defect (64.3%) and palatal collapse (57.1%). Conclusion: Gentur’s technique is able to utilize tissue deficiency to create ideal lip and nose in unilateral cleft lip repair even in patients with wide gap.


2013 ◽  
Vol 4 (3) ◽  
pp. 86-91
Author(s):  
Chai Siew Cheng ◽  
SKL Jimeno ◽  
R Sasidaran ◽  
A Sergius

Objective: To identify number of cases and the type of cleft lip and/or palate managed in government tertiary center (Queen Elizabeth Hospital) in Kota Kinabalu; and to analyze the associative factors of cleft lip and/or palate. Methods: A retrospective cross-sectional study carried out in Hospital Queen Elizabeth, Kota Kinabalu, Sabah from January 2011 to December 2012. Data from 162 new cases, which were referred for cleft lip and/or palate, were included in the study. Result: Cleft lip and palate was the most common type. While cleft lip with or without palate had higher preponderance towards male patients, secondary palate however was more common among female patients. These results were statistically significant. Conclusion: Further and larger scale study need to be carried out to identify the incidence of cleft lip and/or palate in Sabah, and its associated genetic and environmental risk factors. Early identification and intervention for cleft lip and palate need to be emphasized. DOI: http://dx.doi.org/10.3126/ajms.v4i3.8170   Asian Journal of Medical Sciences 4(2013) 86-91


Author(s):  
Johno Breeze ◽  
Sat Parmer ◽  
Niall McLeod

This chapter contains eight clinical vivas on the subject of cleft surgery. This will provide candidates sitting the vivas component of the Oral and Maxillofacial Surgery FRCS examination with practical knowledge in the assessment and treatment of presentations such as cleft lip and palate as well as modalities of treatment including types of cleft lip repair. It will also provide insight for clinicians in allied specialties, such as otolaryngology and plastic surgery, in how to engage in a debate with a senior clinician in the contemporary management of such complex cases.


2020 ◽  
pp. 105566562094911
Author(s):  
Iva Burianova ◽  
Milos Cerny ◽  
Jiri Borsky ◽  
Kristyna Zilinska ◽  
Jana Dornakova ◽  
...  

Objective: There are minimal data available on nutrition after early repair of cleft lip and the factors influencing initiation of breastfeeding. This study assessed the impact of the length of surgery, length of ventilation support, and duration of hospital stay on breastfeeding rates after early cleft lip surgery. Design: This is a prospective observational cohort study comparing 2 hospitals providing early surgical repair of facial clefts from January 2014 to December 2016. Both hospitals are designated as Baby-Friendly Hospitals. Demographic and anthropometric data from mothers and newborns were recorded. Setting: Tertiary neonatal and pediatric surgery center. Patients: Hospital A: 61 newborns, Hospital B: 157 newborns. Interventions: Early (day 5 to 14) cheiloplasty in newborns with cleft lip or cleft lip and palate. Main Outcome Measures: Influence of duration of hospital stay, length of operation, and artificial ventilation on the rate of breastfeeding. Results: Significantly, more newborns were breastfed following early surgical repair of an isolated cleft lip compared to those with both cleft lip and palate, in both hospitals (hospital A 82% vs 0%, P = .0001, hospital B 66% vs 5%, P = .0001). Duration of hospital stay, length of operation, and duration of artificial ventilation did not significantly affect the rate of breastfeeding. Conclusions: The factors associated with early cleft lip repair (length of operation, length of ventilation support, and duration of hospital stay) do not affect breastfeeding rate.


2016 ◽  
Vol 10 (03) ◽  
pp. 435-438 ◽  
Author(s):  
Chitravelu Siva Subramanian ◽  
N. K. K. Koteswara Prasad ◽  
Arun B. Chitharanjan ◽  
Eric Jein Wein Liou

ABSTRACTNasoalveolar molding (NAM) can be done effectively to reshape the nasal cartilage and mold the maxillary dentoalveolar arch before surgical cleft lip repair and primary rhinoplasty. Presurgical NAM helps as an adjunct procedure to enhance the esthetic and functional outcome of the surgical procedures. We have developed a modified NAM device to suit to the needs of the patients coming from distant places for the treatment. This device helps in reducing the number of frequent visits the patient needs to take to the craniofacial center. The purpose of this presentation is to report this treatment technique and discuss its application.


2018 ◽  
Vol 55 (8) ◽  
pp. 1145-1152 ◽  
Author(s):  
Eugene Park ◽  
Gaurav Deshpande ◽  
Bjorn Schonmeyr ◽  
Carolina Restrepo ◽  
Alex Campbell

Objective: To evaluate complication rates following cleft lip and cleft palate repairs during the transition from mission-based care to center-based care in a developing region. Patients and Design: We performed a retrospective review of 3419 patients who underwent cleft lip repair and 1728 patients who underwent cleft palate repair in Guwahati, India between December 2010 and February 2014. Of those who underwent cleft lip repair, 654 were treated during a surgical mission and 2765 were treated at a permanent center. Of those who underwent cleft palate repair, 236 were treated during a surgical mission and 1491 were treated at a permanent center. Setting: Two large surgical missions to Guwahati, India, and the Guwahati Comprehensive Cleft Care Center (GCCCC) in Assam, India. Main Outcome Measure: Overall complication rates following cleft lip and cleft palate repair. Results: Overall complication rates following cleft lip repair were 13.2% for the first mission, 6.7% for the second mission, and 4.0% at GCCCC. Overall complication rates following cleft palate repair were 28.0% for the first mission, 30.0% for the second mission, and 15.8% at GCCCC. Complication rates following cleft palate repair by the subset of surgeons permanently based at GCCCC (7.2%) were lower than visiting surgeons ( P < .05). Conclusions: Our findings support the notion that transitioning from a mission-based model to a permanent facility-based model of cleft care delivery in the developing world can lead to decreased complication rates.


2017 ◽  
Vol 41 (6) ◽  
pp. 442-445 ◽  
Author(s):  
LaQuia Vinson

Objective: The specific aim of this retrospective cross-sectional study was to assess the efficacy of DynaCleft® as a method of presurgical orthopedics with infants with a unilateral cleft lip and cleft palate who used an oral obturator. Study design: Data was collected from 25 infants all of comparable age diagnosed with a unilateral complete cleft lip and palate. Eight patients used DynaCleft ® and an obturator (Group Alpha) and seventeen patients only had an obturator (Group Beta). Maxillary impression casts were obtained from each patient at the initial clinic visit and at the time of cleft lip repair. Differences in alveolar cleft width were compared between the two groups. Casts were measured twice by one observer using a digital caliper. Results: Group Alpha began treatment on an average age of 24.25 days and Group Beta an average of 15.35 days of age. The average cleft width of Group Alpha was 8.13 mm and after treatment it was 4.59 mm. The average cleft width of Group Beta was 8.09 mm and 6.92 mm after treatment. Results of paired t-tests and two-sample t-test showed that cleft width changes between the two groups were significant (P = .03). Conclusions: DynaCleft ® significantly decreased the size of the alveolar cleft width compared to infants who did not use it. Providers should consider using DynaCleft® for patients who may not have access to infant maxillary orthopedics.


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