The Arkansas Tessier Number 3 Cleft Experience: Soft Tissue and Skeletal Findings With Primary Surgical Management Four-Step Approach

Tessier No 3 facial cleft (oro-nasal-ocular clefts) is the rarest and most challenging of all the Tessier clefts. Reports on Tessier No 3 clinical findings, surgical techniques, and outcomes are varied due to the scarcity of patients and the wide range of phenotypic findings. The authors present our experience of 2 children born with Tessier No 3 clefts who were both managed at the Arkansas Children's Hospital. Our purpose is to add knowledge on this rare craniofacial cleft by providing detailed soft tissue findings, skeletal findings, operative techniques, early postoperative outcome, and suggestions of a treatment protocol. Both were born at 38 weeks gestation and had multiple associated anomalies including: syndactyly, limb anomalies, cardiac defects, and encephalocele in Patient 1 and hydrocephalus and dysphagia in Patient 2. While both patients had a bilateral cleft lip and palate, Patient 1 had a severe left-sided cleft and Patient 2 had a right-sided incomplete cleft. A multidisciplinary team of specialists in Plastic Surgery, Otolaryngology, and Oculoplastics were assembled to devise a top–down approach for repair. In brief, our surgical sequence for both infants was a dorsal nasal Reiger flap to level the ala, cheek advancement flap along with medial canthal repositioning, and more traditional bilateral cleft lip repair using a modified Millard technique. Postoperatively, Patient 1 experienced some early scarring, medial canthal rounding, lagophthalmos, and ci...
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Clinical Studies Source Type: research

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Discussion Holoprosencephaly (HPE) is a clefting problem of the brain. “[HPE] the result of incomplete or absent midline division of the embryonic forebrain into distinct cerebral hemispheres (prosencephalon) between the 18th and 28th day after conception.” There are four distinct subtypes: Alobar – both hemispheres are completely fused and are not separated into the left and right hemispheres. There is agenesis of the corpus callosum, arrhinencephaly and a single ventricle with fused thalami. Facial features are almost always affected. Semilobar – the cerebral hemispheres are fused anteriorly bu...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
Authors: Malik M, Khanna P, Verma R Abstract Objectives: The present study was aimed at assessing Association of Maternal Risk Factors to congenital anomalies of infants. Material and Methods: This community based retrospective and cross-sectional study was carried out in 23 rural sub-centres of block Beri, district Jhajjar (Haryana, India) among 920 mothers. A predesigned pretested semistructured questionnaire was used to collect information. Univariate analysis along with logistic regression analysis was performed. Results: The prevalence of congenitally malformations was 1.2%. Most common congenital malf...
Source: Journal of the Association of Physicians of India - Category: General Medicine Tags: J Assoc Physicians India Source Type: research
Conclusion: We identified a novel non-sense mutation in MTHFR gene in a single Egyptian family with severe MTHFR deficiency. The present investigation is clinically important, as it adds to the growing list of MTHFR mutations, which might help in genetic counseling of families of affected children and proper genotype-phenotype correlation. Background Severe 5,10-Methylenetetrahydrofolate reductase (MTHFR; OMIM 236250) deficiency is a rare inborn error of metabolism and inherited in an autosomal recessive fashion. It is a very common disorder of folate metabolism and is clinically characterized with low plasma methion...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
We describe a preterm neonate who presented with respiratory failure and clinical features of CD. Our case had only 10 pairs of ribs, and to the best of our knowledge this is the first case report of CD with 10 pairs of ribs.
Source: Indian Journal of Radiology and Imaging - Category: Radiology Authors: Source Type: research
Stable occlusion and a pleasing esthetic appearance are often difficult to achieve in patients with congenital defects and severe midfacial deficiencies. Conventional therapy, such as orthodontic treatment followed by orthognathic surgery, is often not sufficient to fully correct the dental and esthetic problems. An interdisciplinary approach for these patients should include prosthodontic treatment that will assist in establishing a harmonious occlusion and improve facial appearance. This clinical report describes the interdisciplinary approach for a young patient with a history of bilateral cleft lip and palate, spina bi...
Source: Journal of Prosthetic Dentistry - Category: Dentistry Authors: Tags: Clinical Report Source Type: research
SummaryThe present study aimed to examine the value of ultrasonic soft markers in prenatal screening by analyzing the clinical outcome of fetuses with ultrasonic soft markers during the second trimester of pregnancy. A retrospective analysis was performed to evaluate the outcome of 591 fetuses with ultrasonic soft markers from January 2015 to August 2016 in Zhongnan Hospital of Wuhan University, China. It was found that 591 fetuses showed ultrasonic soft markers in 4927 cases with the occurrence rate being 12.0%. Among them, 564 fetuses (95.4%) were delivered and the remaining 27 cases (4.6%) were aborted. Five hundred and...
Source: Journal of Huazhong University of Science and Technology -- Medical Sciences -- - Category: Research Source Type: research
We report herein an early fetal case with an apparently isolated abnormal corpus callosum diagnosed by ultrasonography, for which a medical termination of the pregnancy was achieved at 22 weeks of gestation. Postmortem examination displayed facial dysmorphism consisting of hypertelorism, short philtrum and flat and broad nose, cleft palate and left duplex ureter. Neuropathological examination revealed a mega corpus callosum that has never been reported so far in this syndrome. Array-CGH performed on thymic DNA tissue revealed a 17q21.31 microdeletion, which allowed for the confirmation of early occurring Koolen-de Vries sy...
Source: European Journal of Medical Genetics - Category: Genetics & Stem Cells Authors: Tags: Eur J Med Genet Source Type: research
CONCLUSIONS: Having a baseline on the prevalence of congenital defects in Risaralda is very useful in the design of prevention policies oriented to decrease congenital defects incidence and severity. Inclusion of maternity hospitals in the Collaborative Study of Congenital Malformations strengthens national recording and reporting of birth defects. PMID: 27992982 [PubMed - in process]
Source: Biomedica : Revista del Instituto Nacional de Salud - Category: Global & Universal Tags: Biomedica Source Type: research
We describe the case of a term infant with broad and protruding forehead, micrognathia, wide cleft palate, short neck and stocky body with small thoracic cage, short limbs, bilateral clubfeet and triventricular hydrocephalus. A G313S mutation in the gene for type II-collagen(COL2A1)was demonstrated. Soon after birth, the infant had respiratory distress with repeated obstructive apnea, leading to nasotracheal intubation. Despite mechanical ventilation(MV)with high positive pressure, he showed desaturations, leading to tracheotomy. An endoscopy showed severe tracheo-bronchomalacia. At 5 months a polyflex self-expanding silic...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: 7.7 Paediatric Bronchology Source Type: research
Abstract: More than 50% of the population in most developing countries is younger than 18 years, and it has been estimated that 85% of these children may require some sort of surgery before their 15th birthday. Common congenital surgical requirements are for cleft lip and palate, inguinal hernia, meningomyelocele, as well as hydrocephalus. In addition, there is a greater incidence of trauma experienced by pediatric patients. Burn and scald injuries are also common because of the proximity of domestic open fires and boiling pots of water. Infectious conditions such as osteomyelitis and skin abscesses are more frequent in de...
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Original Articles Source Type: research
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