The pectus carinatum or anterior protrusion of the sternum is a less common than pectus excavatum. It occurs more frequently in boys than girls and associated musculoskeletal abnormalities, spinal scoliosis is most common. Ravitch first reported correction of chondromanubrial prominence in 1952, resecting the multiple deformed costal cartilages and performing a double osteotomy on sternum. We have experienced one case of pectus carinatum and obtained satisfactory postoperative results. The deformity was corrected by the subchondral resection of multiple deformed costal cartilage, bilaterally, with single osteotomy on sternum and fracture of the posterior cortex to correct anterior angulation. Postoperative course was uneventful. We report this case with brief review of the literature.
Cartilage ; Congenital Abnormalities ; Female ; Funnel Chest ; Humans ; Musculoskeletal Abnormalities ; Osteotomy ; Scoliosis ; Sternum

Management of infants born with cleft lip and palate entails an interdisciplinary team effort that begins from infancy to adulthood. The goal of pre-surgical infant orthopedics is to reduce the severity of the cleft deformity before surgery. However, traditional methods do not address the deformity of the nasal cartilages and alveolar ridges simultaneously. The Nasoalveolar Molding (NAM) technique takes advantage of the malleability of immature nasal cartilage and its ability to maintain a permanent correction of its form. The NAM device is used to actively mold the alar dome, nasal cartilages, premaxilla, and alveolar ridges into a more normal anatomic form and position. It permits non-surgical elongation of the columella through application of tissue expansion principles. This results in better facial aesthetics and may help reduce the extent, number and cost of surgeries. The three cases presented illustrate the application of the NAM device for the pre-surgical infant orthopedics in unilateral and bilateral cleft lip and palate patients treated at the Philippine Children's Medical Center-Pediatric Dentistry Division (PCMC-PDD).
Human ; Male ; Infant Newborn ; CLEFT LIP ; CLEFT PALATE ; MUSCULOSKELETAL DISEASES ; JAW DISEASES ; JAW ABNORMALITIES

Distraction osteogenesis is a technique of bone lengthening by gradual movement and subsequent remodeling. Distraction forces applied to bone also create tension in the surrounding soft tissues, distraction histiogenesis. Distraction osteogenesis is used to correct facial asymmetry, such as patients with hemifacial microsomia, maxillary or mandibular retrusion, cleft lip and palate, alveolar defect and craniofacial deficiency. Hemifacial microsomia is characterized by unilateral facial hypoplasia, often with unilateral shortening of the mandible and subsequent malocclusion. This report describes two cases of hemifacial microsomia(type IIB). In these two cases, distraction osteogenesis was used to correct a facial asymmetry. Two patients underwent unilateral mandibular distraction osteogenesis of ascending ramus of the mandible with extraoral devices. Successful distraction osteogenesis was achieved in the patients with hemifacial microsomia.
Bone Lengthening ; Cleft Lip ; Facial Asymmetry ; Goldenhar Syndrome* ; Humans ; Malocclusion ; Mandible ; Osteogenesis, Distraction* ; Palate ; Retrognathia

Stickler's syndrome is a progressive, hereditary disorder with ocular and systemic features. Ocular findings are vitreous veil or vitreous strand due to vitreous condensation, vitreoretinal degeneration, moderate to severe myopia, vitreous liquefaction, chorioretinal atrophy, retinal detachment and cataract. Systemic findings are midfacial hypoplasia, cleft palate, hearing loss, musculoskeletal abnormalities. The authors experienced 3 cases of stickler's syndrome in three brothers of one family.
Atrophy ; Cataract ; Cleft Palate ; Hearing Loss ; Humans ; Musculoskeletal Abnormalities ; Myopia ; Retinal Detachment ; Siblings

No abstract available.
Arthrogryposis*

No abstract available.
Arthrogryposis*

No abstract available.
Hallermann's Syndrome*

Crouzon syndrome, an autosomal dominant disorder, has characteristic features of craniosynostosis, hypertelorism, exophthalmos, maxillary hypoplasia and relative mandibular prognathism. Mutations of fibroblast growth factor receptor 2 (FGFR2) gene are associated with craniosynostotic conditions, such as Crouzon syndrome, Jackson-Weiss syndrome, Pfeiffer syndrome, Apert syndrome and Beare-Stevenson cutis gyrata. We found one child with common morphological features of Crouzon syndrome. Interestingly, she was found to have Cys342Ser mutation in FGFR2 exon lllc which has been previously observed in Jackson-Weiss syndrome. This finding supports the variable expression of FGFR2 in human and allelic heterogeneity in these apparently clinically distinct craniosynostotic conditions.
Acrocephalosyndactylia ; Child ; Craniofacial Dysostosis* ; Craniosynostoses ; Exons* ; Exophthalmos ; Humans ; Hypertelorism ; Population Characteristics ; Prognathism ; Receptor, Fibroblast Growth Factor, Type 2

Craniosynostosis is defined as the premature fusion of one or more of the cranial sutures. It leads not only to secondary distortion of skull shape but to various complications including neurologic, ophthalmic and respiratory dysfunction. Craniosynostosis is very heterogeneous in terms of its causes, presentation, and management. Both environmental factors and genetic factors are associated with development of craniosynostosis. Nonsyndromic craniosynostosis accounts for more than 70% of all cases. Syndromic craniosynostosis with a certain genetic cause is more likely to involve multiple sutures or bilateral coronal sutures. FGFR2, FGFR3, FGFR1, TWIST1 and EFNB1 genes are major causative genes of genetic syndromes associated with craniosynostosis. Although most of syndromic craniosynostosis show autosomal dominant inheritance, approximately half of patients are de novo cases. Apert syndrome, Pfeiffer syndrome, Crouzon syndrome, and Antley-Bixler syndrome are related to mutations in FGFR family (especially in FGFR2), and mutations in FGFRs can be overlapped between different syndromes. Saethre-Chotzen syndrome, Muenke syndrome, and craniofrontonasal syndrome are representative disorders showing isolated coronal suture involvement. Compared to the other types of craniosynostosis, single gene mutations can be more frequently detected, in one-third of coronal synostosis patients. Molecular diagnosis can be helpful to provide adequate genetic counseling and guidance for patients with syndromic craniosynostosis.
Acrocephalosyndactylia ; Antley-Bixler Syndrome Phenotype ; Cranial Sutures ; Craniofacial Dysostosis ; Craniosynostoses* ; Diagnosis ; Genetic Counseling ; Humans ; Skull ; Sutures ; Synostosis ; Wills

Orthognathic surgery for Class III malocclusion requires an elaborate preoperative planning using cephalometries or Mock surgery models which enable the surgeon to anticipate postoperative skeletal changes of maxilla and mandible as well as dentition. After surgery, patient's satisfaction is greatly influenced by appearance of soft tissue change. Therefore, it is imperative to predict a relatively accurate soft tissue change prior to surgery. A 5 year retrospective study was designed to evaluate the soft tissue change after sagittal split osteotomy of ramus(SSRO) for class III malocclusion. Analyses of preoperative and postoperative anthropometric measurements were performed. Patients who were treated only by SSRO for class III malocclusion and could follow up for 6 months were studied. Among them, the patients who had history of cleft palate and lip or hemifacial microsomia were excluded. Soft tissue changes were estimated by using the frontal and lateral photographs. Skeletal changes were observed by measuring amount of set back and angular changes of mandible to the reference line by using cephalometries. Relapses were also measured 6 months after the operation. We could observe skeletal changes were more profound than soft tissue changes concerning amount of set back, but soft tissue changes were also profound in angle. Relapse was more profound in skeleton than soft tissue but the amount was not significant. In spite of the variables which may affect proper assessment of the soft tissue change after skeletal relocation, this study can serve as a guide for exact prediction of the postoperative change of soft tissue and skeleton.
Cleft Palate ; Dentition ; Follow-Up Studies ; Goldenhar Syndrome ; Humans ; Lip ; Malocclusion ; Mandible ; Maxilla ; Orthognathic Surgery ; Osteotomy* ; Recurrence ; Retrospective Studies ; Skeleton