Neonatal Care of Infants with Cleft Lip and/or Palate: Feeding Orientation and Evolution of Weight Gain in a Nonspecialized Brazilian Hospital

2007 ◽  
Vol 44 (3) ◽  
pp. 329-334 ◽  
Author(s):  
Lívia Gobby Amstalden-Mendes ◽  
Luis Alberto Magna ◽  
Vera Lúcia Gil-da-Silva-Lopes
Keyword(s):  
Weight Gain ◽  
Surgical Procedure ◽  
Cleft Lip ◽  
Tertiary Hospital ◽  
Postnatal Period ◽  
Rehabilitation Centers ◽  
Spontaneous Reports ◽  
Cleft Lip Palate ◽  
Feeding Resources

Objectives: To survey the feeding orientation received during the postnatal period by the parents of cleft babies, as well as the location where they receive the orientation; to identify resources used in feeding; and to assess the correlation of the child's weight with the surgical procedure schedule. Design: During consultation for diagnosis and genetic counseling in a general tertiary hospital, 26 parents of cleft babies born in different hospitals were interviewed based on a semistructured protocol and spontaneous reports. Results: Cleft palate was present in 42.31% (11/26), cleft lip/palate in 50% (13/26), and cleft lip in 7.69% (2/26) of the cases. Feeding orientation was given in maternities to 72% (18/25) and in specific rehabilitation centers to 24% (6/ 25) of the parents. Breast-feeding was encouraged in every case. Nevertheless, other feeding resources were necessary, especially bottles. Surgical procedure delays caused by poor weight gain occurred in 66.7% (12/18). Conclusions: Neonatal feeding orientation was not systematically given in every case. Because it is an important way to achieve an effective weight gain, educational programs for nonspecialized health professionals, as well as regular pediatric follow-up and specialized multi-professional teams, could improve nutritional intake and could move the schedule for surgical procedures forward. The results also suggest that specific neonatal health care for cleft babies should be part of health policy.

Association between 10 Microsatellite Markers and Nonsyndromic Cleft Lip Palate in the Chilean Population

2004 ◽  
Vol 41 (2) ◽  
pp. 163-167 ◽  
Author(s):  
Rafael Blanco ◽  
José Suazo ◽  
JoséLuis Santos ◽  
Mónica Paredes ◽  
Hsiao Sung ◽  
...  
Keyword(s):  
Cleft Lip ◽  
Statistical Significance ◽  
Positive Family History ◽  
Case Control ◽  
University Hospital ◽  
Control Group ◽  
Exact Test ◽  
Rehabilitation Centers ◽  
Cleft Lip Palate ◽  
Chilean Population

Objective The objective of this case-control study was to evaluate the possible association between nonsyndromic cleft lip/palate (NSCLP) and 10 genetic markers in four chromosomal regions in the admixed Spanish-Amerindian Chilean population. Setting Study participants included 56 patients with NSCLP identified and interviewed for positive family history during the course of clinical examinations at different rehabilitation centers in the cities of Santiago and Talca, Chile. A control group of 59 normal individuals without known familial antecedents of clefting was obtained from blood bank donors of the University Hospital, University of Chile. Cases and controls belonged to low- to low-middle socioeconomic strata. Results Ten markers from chromosome 4p, 4q, 6p, 17q, and 19q were assessed (MSX1, D4S175, D4S192, F13A1, EDN1, D6S89, D6S105, D6S109, D17S579, BCL3). Four of them showed significant deviations from Hardy-Weinberg expectations in controls, according to the exact test (D4S192, BCL3, F13A1, and D6S89). The case-control comparison by means of the CLUMP program showed significant differences only in BCL3, and D6S109 almost reached statistical significance. Conclusions Most of the genetic regions with positive results in Caucasian populations may not be involved in NSCLP in Chile, regardless of the positive evidence for the candidate region on chromosome 19. Similar findings have been reported recently in the Chinese population.

scholarly journals Follow-up association studies of chromosome region 9q and nonsyndromic cleft lip/palate

2010 ◽  
Vol 152A (7) ◽  
pp. 1701-1710 ◽  
Author(s):  
Ariadne Letra ◽  
Renato Menezes ◽  
Manika Govil ◽  
Renata F. Fonseca ◽  
Toby McHenry ◽  
...  
Keyword(s):  
Cleft Lip ◽  
Chromosome Region ◽  
Association Studies ◽  
Cleft Lip Palate

scholarly journals Detection of non-affine shape outliers for matched-pair shape data

2012 ◽  
Vol 51 (1) ◽  
pp. 83-90 ◽  
Author(s):  
Stanislav Katina
Keyword(s):  
Outlier Detection ◽  
Large Scale ◽  
Cleft Lip ◽  
Scar Tissue ◽  
Matched Pair ◽  
Small Scale ◽  
Affine Subspaces ◽  
Cleft Lip Palate ◽  
Shape Data

ABSTRACT Cleft lip/palate (CLP) is a relatively common birth defect so dis- figuring that nowadays it is almost always corrected surgically as early as possible. The postnatal surgical correction does not, however, result in a normally growing upper jaw, but instead, owing to scar tissue, one that grows abnormally. It is important to decide if a clinical treatment group is homogeneous. The example involves data from digitally processed lateral X-ray films of 48 boys who have complete unilateral CLP but no other malformation. 22 landmarks were represented by their Procrustes shape coordinates, principal components of matched- pair differences were examined, and the distribution of the 48 shape changes was studied for outliers in the affine and non-affine subspaces of the full Procrustes shape and form space. To separate outliers from inliers we use bagplots. There are no outliers apparent in the affine subspace. In the non-affine subspaces, we found no outliers in the subspace of bending patterns at large scale but four out- liers in the subspace of local changes at small scale. Almost the same outliers were found by form-space PCA. These latter are associated with possible creases of the corresponding thin-plate splines. In those cases we can use the same spline formalism to relax the outlying form to an inlier by optimal relaxation along the curve d´ecolletage that weighs bending energy against Procrustes distance and stop relaxation on the fence. These maneuvers suggest a possibly novel and interesting fusion of the Procrustes-spline toolkit with outlier detection. They also have practical implications for craniofacial management of CLP follow-up as well as suggestive implications for outlier detection in applied craniometrics and anthropometrics more generally.

scholarly journals Novel missense mutation of the TP63 gene in a newborn with Hay-Wells/Ankyloblepharon-Ectodermal defects-Cleft lip/palate (AEC) syndrome: clinical report and follow-up

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Gregorio Serra ◽  
Vincenzo Antona ◽  
Mario Giuffré ◽  
Federica Li Pomi ◽  
Lucia Lo Scalzo ◽  
...  
Keyword(s):  
Cleft Palate ◽  
Missense Mutation ◽  
Cleft Lip ◽  
Ectodermal Dysplasia ◽  
Clinical Signs ◽  
Clinical Report ◽  
Sequencing Analysis ◽  
Genetic Syndrome ◽  
Cleft Lip Palate

Abstract Introduction Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome, also known as Hay-Wells syndrome, is a rare genetic syndrome with ectodermal dysplasia. About 100 patients have been reported to date. It is associated to a heterozygous mutation of the tumor protein p63 (TP63) gene, located on chromosome 3q28. Typical clinical manifestations include: filiform ankyloblepharon adnatum (congenital adherence of the eyelids), ectodermal abnormalities (sparse and frizzy hair, skin defects, nail alterations, dental changes and hypohidrosis), and cleft lip/palate. Diagnostic suspicion is based on clinical signs and confirmed by genetic testing. Patient’s presentation We hereby report on a female newborn with erythroderma, thin lamellar desquamations, extensive skin erosions, sparse and wiry hair, filiform ankyloblepharon adnatum, agenesis of the lachrymal puncta, cleft palate and nail dysplasia. Her phenotype was compatible with AEC syndrome. Then, based on the clinical suspicion, sequencing analysis of the TP63 gene was performed, and revealed a de novo novel missense mutation. Eyelids adherence and cleft palate underwent surgical correction, while skin erosions were treated with topical antibiotics/antifungals and emollient/re-epithelizing creams. A surgical reconstruction is presently planned for the agenesis of the lachrymal puncta. The infant currently is 17 months of age and is included in a multidisciplinary follow-up. At present shows growth impairment and mild developmental delay, and typical signs of ectodermal dysplasia with small areas of dermatitis lesions on the scalp, without further abnormalities. Conclusions Our report underlines the relevance of an early and careful clinical evaluation in neonates with ankyloblefaron, facial dysmorphism, and signs of ectodermal dysplasia. In these cases, the suspicion of AEC syndrome must be promptly raised, and sequencing analysis of TP63 early performed as well. An individualized, multidisciplinary and long-term follow-up should be guaranteed to affected subjects and their families, also to identify associated morbidities and prevent possible serious complications and adverse outcomes.

scholarly journals Feeling Normal? Long-Term Follow-up of Patients with a Cleft Lip–Palate after Rhinoplasty with the Derriford Appearance Scale (DAS-59)

2016 ◽  
Vol 32 (02) ◽  
pp. 219-224 ◽  
Author(s):  
Andreas Reichelt ◽  
Gilbert Nolst-Trenité ◽  
Dirk Menger ◽  
Andreas Albers
Keyword(s):  
Cleft Lip ◽  
Long Term Follow Up ◽  
Cleft Lip Palate

scholarly journals Geospatial Analysis of Risk Factors Contributing to Loss to Follow-up in Cleft Lip/Palate Care

2018 ◽  
Vol 6 (9) ◽  
pp. e1910 ◽  
Author(s):  
Banafsheh Sharif-Askary ◽  
Peter G. Bittar ◽  
Alfredo E. Farjat ◽  
Beiyu Liu ◽  
Joao Ricardo Nickenig Vissoci ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cleft Lip ◽  
Geospatial Analysis ◽  
Loss To Follow Up ◽  
Cleft Lip Palate

scholarly journals Perioperative Complications And Concomitant Diseases Of Patients Followed In Intensive Care Unit After Cleft Lip-Palate Surgery

2021 ◽  
Author(s):  
Murat Tümer ◽  
Ayşe Ayyıldız ◽  
Başak Akça ◽  
Aysun Ankay Yılbaş ◽  
Filiz Üzümcügil ◽  
...  
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Difficult Airway ◽  
Difficult Intubation ◽  
Cleft Lip ◽  
Systemic Disease ◽  
Fiberoptic Bronchoscopy ◽  
Close Monitoring ◽  
Cleft Lip Palate

INTRODUCTION: Cleft lip/palate (CLCP) patients might need postoperative care in Intensive Care Unit (ICU) due to several reasons like difficult airway management, associated abnormalities and perioperative respiratory complications. Our aim was to evaluate the factors associated with difficult airway and need for postoperative ICU follow-up in CLCP patients operated in our institution. METHODS: CLCP patients followed in ICU after surgery between 2005-2013 were retrospectively reviewed. RESULTS: Twenty patients were included to the study. Ten had CLCP together, ten had isolated cleft palate. Difficult intubation was seen in 8 patients. Difficult mask ventilation was seen only in one patient with 22q11 deletion. All patients with difficult intubation had micrognathia. Nasal fiberoptic bronchoscopy was more commonly used in patients with difficult intubation. There was a statistically significant relationship between the presence of any systemic disease and difficult intubation. Main reasons for ICU follow-up were the need for close monitoring and airway related problems. DISCUSSION AND CONCLUSION: Difficult airway is a frequent problem in CLCP patients even in the absence of a diagnosed syndrome. Patients with difficult airway or risk of postoperative airway obstruction could be better followed in ICU during early postoperative period for rapid diagnosis and treatment of possible complications by experienced staff.

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