Dysphasia related to oral anomaly is a common situation in oral and maxillofacial surgery. The etiology of oral anomalies causing dysphasia can be divided into congenital and acquired disease. Congenital diseases include teratoma or benign tumors and congenital defects such as cleft lip and palate. Benign tumors include cystic hygroma in the neck and hemangioma in the tongue. Certain syndromes with macroglossia and micrognathia are also related to difficulty in swallowing. The three common syndromes are Pierre-Robin syndrome, Beckwith-Widermann syndrome and ectodermal dysplasia. Taken together, these congenital diseases require a multi-discipline approach to obtain optimal results. Representative disease of acquired dysphasia is the oral cavity cancer. Cancer ablation results in tissue defect and decreased motor function. Free flap reconstruction is the choice of treatment following oral cavity caner operation; however, dysphasia after cancer operation is inevitable. In this review article, the full scopes of oral anomaly associated with dysphasia were classified and treatment was suggested.
Aphasia ; Cleft Lip ; Congenital Abnormalities ; Deglutition ; Ectodermal Dysplasia ; Free Tissue Flaps ; Hemangioma ; Lymphangioma, Cystic ; Macroglossia ; Micrognathism ; Mouth ; Mouth Neoplasms ; Neck ; Palate ; Pierre Robin Syndrome ; Surgery, Oral ; Teratoma ; Tongue

Pierre Robin sequence (PRS) describes the clinical triad of micro- and/or retrognathia, glossoptosis and cleft soft palate. Glossopexy has been demonstrated to be an effective treatment in selected cases of obstruction caused by glossoptosis (generally 6 to 10 months of glossopexy period). If radiofrequency therapy (RF) can reduce tongue volume in PRS, it will be helpful in early releasing of the glossopexy. Two-day-old patient showed a PRS triad. Intermittent cyanosis, respiratory difficulty and feeding problems were also observed. The respiration was not improved and prolonged intubation increased the possibility of respiratory complications like pneumonia. The surgical intervention-glossopexy and RF was done 20 days after birth. We applied RF combined with conventional glossopexy and could get successful results while reducing the overall treatment time to 6 weeks. The follow-up until 12 months after birth was uneventful. Considering that early recovery is highly beneficial to PRS patients by reducing risks associated with glossopexy and low energy RF application is very simple and low risk to patient, our combination therapy should be considered for the treatment of airway problem related to PRS.]]>
Cleft Palate ; Cyanosis ; Follow-Up Studies ; Humans ; Intubation ; Palate, Soft ; Parturition ; Pierre Robin Syndrome ; Pneumonia ; Respiration ; Retrognathia ; Tongue

Cleft Lip ; complications ; Cleft Palate ; complications ; Humans ; Maxillofacial Abnormalities ; pathology ; Orthodontics, Corrective

OBJECTIVETo study the application of extraoral distraction devices in correction of various maxillo-mandibular deformities.

METHODSFrom 1997, 11 cases with various maxillo-mandibular deformities were corrected by distraction devices of designed by myself. 7 cases of then were the patients with cleft palate who have severe secondary maxillary hypoplasia. 1 case with micrognathia, 1 case with partial mandibular mentum defect, 1 case with lateral deviation of micrognathia and 1 case with hemimandibular hypertrophy. The mandibles were elongated, from 12 mm to 20 mm, the maxillae were elongated from 7 mm to 22 mm, the vertical mandibular distraction was 15 mm.

RESULTSAll cases were corrected satisfactorily and haven't any complications. In 1 cases the 1 Kischners pin had been pull out by loosen during distration. 4 Kischners pin were curved during vertical mandibular distration. Another case the maxillary hypoplasia appeared edge-to-edge and mild open occlusion after 4 weeks fixation.

CONCLUSIONThe extraoral distration osteogenesis provides a new method with many advantages for those maxillo-mandibular deformities that are difficulty corrected by ordinary orthognathic surgery.

Cleft Palate ; therapy ; Humans ; Jaw Abnormalities ; therapy ; Mandible ; abnormalities ; Maxilla ; abnormalities ; Micrognathism ; therapy ; Osteogenesis, Distraction ; instrumentation ; Treatment Outcome

Pierre Robin sequence (PRS), also called Robin sequence, is a congenital anomaly characterized by a triad of micrognathia, glossoptosis, and upper airway obstruction. Infants with PRS can present with varying degrees of respiratory difficulty secondary to upper airway obstruction. There has been no consensus for the treatment of upper airway obstruction in infants with PRS, but recent studies recommend attempting non-surgical interventions before surgical treatment. In this case report, we present 3 cases of infants diagnosed with PRS who showed persistent respiratory difficulties after birth. Before considering surgical intervention, insertion of a nasopharyngeal airway was attempted in these infants. Following this procedure, symptoms of upper airway obstruction were relieved, and all infants were discharged without surgical interventions; the nasopharyngeal airway was removed 1 to 2 months later. To date, no infant has shown signs of upper airway obstruction. Nasopharyngeal airway insertion is a highly effective and less invasive treatment option for infants with PRS. However, it is not widely known and used in Korea. Nasopharyngeal airway insertion can be preferentially considered before surgical intervention for upper airway obstruction in such infants.
Airway Obstruction ; Consensus ; Glossoptosis ; Humans ; Infant ; Korea ; Micrognathism ; Parturition ; Pierre Robin Syndrome

Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and airway obstruction. PRS does not have a single pathogenesis, but rather is associated with multiple syndromes. This report presents the case of a 35-year-old woman with PRS and scoliosis. Among the syndromes related to PRS, cerebro-costo-mandibular syndrome (CCMS), which is characterized by posterior rib gap defects and vertebral anomalies, was suspected in this patient. However, no posterior rib gap defect was detected on radiological examinations. Although over 80 cases of CCMS have been reported to date, few cases of PRS with scoliosis alone have been reported. Therefore, this report demonstrated the clinical, radiological, and cephalometric characteristics of an adult patient with PRS and scoliosis, but without rib anomalies.
Adult ; Airway Obstruction ; Female ; Glossoptosis ; Humans ; Micrognathism ; Pierre Robin Syndrome ; Ribs ; Scoliosis

Pierre Robin sequence as a symptom triad of micrognathia, glossoptosis, and cleft palate results in upper airway obstruction and feeding problems. If mild, it is often managed in the prone position. When positional treatment fails, however, surgical intervention such as tongue-lip adhesion, tracheostomy, and mandibular distraction osteogenesis is mandatory to relieve airway obstruction. There has been growing interest in the application of distraction osteogenesis for the management of craniofacial abnormalities. The mandibular distraction osteogenesis to newborns may prevent the airway obstruction, decrease the potential tracheostomy, and reduce the likehood of orthognathic surgery after growth. We experienced an infant with Pierre Robin sequence who showed mandibular hypoplasia, glossoptosis, incomplete cleft palate, intermittent cyanos is, depression of the chest, and respiratory difficulty associated with airway obstruction. We treated the airway obstruction by tongue-lip adhesion at 2 weeks of age, and treated the mandibular retrognathism and depression of the chest byusing internal mandibular distraction osteogenesis at 7 month of age. The mandible moved forwardly, the upper airway space was enlarged, and the antero-posterior distance of the mandible was elongated after the mandibular distraction. Mandibular distraction osteogenesis may be a promising technique to avoid the need of tracheostomy and orthognathic surgery, and to correct airway obstruction in infants with congenital craniofacial malformation.
Airway Obstruction ; Cleft Palate ; Craniofacial Abnormalities ; Depression ; Humans ; Infant ; Infant, Newborn ; Mandible ; Orthognathic Surgery ; Osteogenesis, Distraction ; Pierre Robin Syndrome ; Prone Position ; Retrognathia ; Songbirds ; Thorax ; Tracheostomy

OBJECTIVEThe purpose of this study was to retrospect the prognosis of simultaneous repair of cleft lip and closure of cleft hard palate with vomer flaps in patients with unilateral complete cleft lip and palate.

METHODSA retrospective study was carried out in 47 patients with unilateral complete cleft lip and palate and, simultaneously received repair of cleft lip and closure of cleft hard palate with vomer flaps. The duration of operation, as well as the blood loss during the operation was recorded, and compared with those patients who only received cleft lip repair.

RESULTSAll the operations were successful, and the wound healed well. The procedure of simultaneous repair of cleft lip and closure of cleft hard palate with vomer flaps did not prolong the operating time, compared with simple cleft lip repair. No blood transfusion was needed due to closure of cleft hard palates with vomer flaps.

CONCLUSIONSimultaneous repairs of cleft lip and closure of cleft hard palate with vomer flaps are safe for patients with unilateral complete cleft lip and palate.

Abnormalities, Multiple ; surgery ; Cleft Lip ; surgery ; Cleft Palate ; surgery ; Female ; Humans ; Infant ; Male ; Maxillofacial Development ; Palate, Hard ; surgery ; Palate, Soft ; surgery ; Reconstructive Surgical Procedures ; methods ; Surgical Flaps

Adult ; Female ; Humans ; Maxillofacial Abnormalities ; Nose ; abnormalities

The Pierre Robin sequence (PRS) is the nonrandom association of micrognathia, cleft palate, and glossoptosis, leading to respiratory and feeding difficulties that appear neurogenic rather than mechanical in causation. Genetic determinants are thought to underlie this functional and morphological entity, based on the existence of Mendelian syndromes with PRS. Here, we demonstrate the association of PRS with trisomy 8p due to duplication of a segment as the karyotype 46,XX,dup(8)(p21.3p23.1) and confirm the additional materials as chromosome 8 via whole chromosome paint probes. Our observation supports the hypothesis regarding a genetic basis for nonsyndromic PRS, strengthens the possible genetic association with isolated cleft palate, and provides a candidate PRS locus in chromosomal region 8(p21.3p23.1).
Chromosomes, Human, Pair 8 ; Cleft Palate ; Karyotype ; Paint ; Pierre Robin Syndrome ; Trisomy