Temporal bone thickness analysis in craniofacial anomalies: key considerations for bone conduction hearing implants.

Purpose: This study analyzes temporal bone thickness in pediatric craniofacial anomalies patients to determine the optimal age for preoperative high-resolution computed tomography (HRCT) and enhance surgical planning of bone conduction hearing implants.

Methods: A retrospective examination of 370 temporal bone HRCT scans from 158 pediatric patients with craniofacial anomalies was conducted. Statistical analysis included descriptive statistics and correlation between age and skull thickness.

Craniosynostosis: orofacial and oral health perspectives with masticatory insights.

Background: Craniosynostosis (CS), premature fusion of one or more cranial sutures, leads to abnormal skull development, impacting both facial esthetics and oral function. This study aimed to evaluate the specific orofacial and oral health characteristics, including masticatory performance, in Thai patients with CS.

Methods: A comparative study was conducted with Thai CS patients aged 6-17 years and a control group of healthy individuals with similar age distribution.

Sleep-disordered Breathing in Children With Craniofacial Anomalies.

Sleep-disordered breathing (SDB) is a common disorder in children, characterized by snoring and/or increased breathing force due to narrowing and increased upper airway collapse while sleeping. Over the last decade, it has been recognized that SDB occurs more frequently in children with craniofacial anomalies, but data in Thailand is quite limited. This study retrospective descriptive study aims to find the prevalence of SDB among children with craniofacial anomalies in Thailand and associated risk factors by collecting data among Thai children with congenital craniofacial anomalies younger than 15 years old who visited the Princess Sirindhorn Craniofacial Center at King Chulalongkorn Memorial Hospital between 2016 and 2021.

Epidemiological Characteristics of Children With Non-Cleft Lip/Palate Craniofacial Anomalies.

Non-cleft craniofacial anomalies are not as common as cleft lip and palate but resultant disability can be very severe. Although there are epidemiological studies of the clefts in the medical literature, the non-cleft group is still not well known. This study was to examine the epidemiological characteristics of the non-cleft craniofacial anomalies.

Severe craniofrontonasal syndrome in a male patient mosaic for a novel nonsense mutation in EFNB1.

Craniofrontonasal syndrome (CFNS) is an X-linked disorder caused by mutations in EFNB1. Uncommonly and paradoxically, female patients with CFNS exhibit significantly more severe symptoms than male patients. This is explained by "cellular interference".

Combined Intralesional Neodymium-Doped Yttrium Aluminium Garnet Laser and Intratumoral Ligation as Curative Treatment for Craniofacial Arteriovenous Malformations.

Craniofacial arteriovenous malformation (AVM), although very rare, has been a very difficult problem to treat especially when it is large and involves important structures. Surgical resection often results in unacceptable complications but still not curative. At our institution, treatment by combined intralesional neodymium-doped yttrium aluminium garnet laser and intratumoral ligation has been successful in venous malformation.

FGFR1 and FGFR2 mutations in Pfeiffer syndrome.

Pfeiffer syndrome (PS) (MIM 101600) is one of the most common syndromic forms of craniosynostosis. It is characterized by craniosysnostosis, midface hypoplasia, broad and medially deviated thumbs, and great toes with partial syndactyly of the digits. Here, we described clinical and genetic features of 12 unrelated Thai individuals with PS.

Cadaveric study of the nasal periosteum and implant position after augmentation rhinoplasty.

Augmentation rhinoplasty is one of the most common cosmetic procedures done in Asia. A technique believed to be helpful in reducing complications is subperiosteal placement of silicone implant. A deeply placed implant should benefit from having thicker soft tissue coverage.

PTPRF is disrupted in a patient with syndromic amastia.

Background: The presence of mammary glands distinguishes mammals from other organisms. Despite significant advances in defining the signaling pathways responsible for mammary gland development in mice, our understanding of human mammary gland development remains rudimentary. Here, we identified a woman with bilateral amastia, ectodermal dysplasia and unilateral renal agenesis.

Three novel mutations of the IRF6 gene with one associated with an unusual feature in Van der Woude syndrome.

Van der Woude syndrome (VWS) is a dominantly inherited disorder characterized by cleft lip with or without cleft palate and lip pits. It remains the most common syndromic form of oral clefts. Mutations in the interferon regulatory factor 6 (IRF6) gene have been identified in patients with VWS.

Quantitative study of new bone formation in distraction osteogenesis of craniofacial bones by bone scintigraphy.

Although distraction osteogenesis is widely accepted as a technique to augment the craniofacial skeleton, timing to start distraction after an osteotomy or to remove distractors is basically based on studies on long bones. Because bone scintigraphy is well known to be the gold standard for quantitative measurement of bone formation, we conducted this pilot study to evaluate its feasibility as a tool for assessing new bone formation by distraction osteogenesis. Five patients with midface hypoplasia and four with mandibular hypoplasia were studied.

Risk factors associated with the occurrence of frontoethmoidal encephalomeningocele.

Objectives: To determine factors associated with the occurrence of frontoethmoidal encephalomeningocele (FEEM), a congenital defect with unique geographical distribution.

Methods: The subjects of this study were 160 unrelated cases of FEEM. Subjects were recruited between 1999 and 2006 from 15 medical centers throughout Thailand.

Correction of the frontoethmoidal encephalomeningocele with minimal facial incision: modified Chula technique.

Background: At present all surgical techniques to correct the frontoethmoidal encephalomeningocele require extensive incisions over the mass and perinasal area, thus adding scars to the already-disfigured faces. This study demonstrates a possibility of doing definitive surgery with minimal facial incision.

Methods: The technique follows the principles of the "Chula technique," which is the one-stage definitive technique without formal frontal craniotomy.

Frontoethmoidal encephalomeningocele: new morphological findings and a new classification.

Given a lack of a comprehensive classification for the frontoethmoidal encephalomeningocele (FEEM), clinical, photographic, and computed tomography (CT) data of 23 nonoperated patients were reviewed. Extracranial pathological findings of interest included herniation masses, facial deformities, and frontonasal bone morphology. Intracranial pathological findings of interest included morphology of the anterior cranial floor and brain malformations.

Reduction malarplasty without external incision: a simple technique.

Background: The concept of Eastern facial beauty is different from that of the Western. Prominent malar bones are perceived as unattractive by Easterners, including the Thai. Many techniques for malar reduction, such as chiseling or burring of the zygomatic body, are ineffective in reducing facial width.

Normal palatal sutures in newborns and fetuses: a critical fact for successful palatal distraction.

Distraction osteogenesis (DO) has recently been applied to the palate. Successful posterior lengthening and medial advancement of the palates was continuously reported. Based on these studies, it is obvious that DO will play a major role in the management of problems related to palatal defects in the near future.

Frontoethmoidal encephalomeningocele: surgical correction by the Chula technique.

This study reevaluates a surgical technique known as the Chula technique, previously reported in 1991 for correction of frontoethmoidal encephalomeningocele. From 1986 to 1999, 108 patients were operated on with this technique, which could remove the herniation mass, repair dural and bone defects, reconstruct the naso-orbital area, and restore aesthetic facial appearance in a single stage. Formal frontal craniotomy was not necessary.

FGFR2 mutations among Thai children with Crouzon and Apert syndromes.

Crouzon and Apert syndromes have been reported to be associated with mutations in Fibroblast Growth Factor Receptor 2 (FGFR2) gene in various ethnic groups, but never in Southeast Asian subjects. Therefore, the authors conducted a study to characterize 11 Thai patients: four with Crouzon syndrome and seven with Apert syndrome. All cases are sporadic.