• Journal of The Korean Dental Society of Anesthesiology
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• v.2 no.2 s.3
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• pp.101-106
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• 2002

Background: The management of the behavior of handicapped children when providing required dental care is often a problem, whether in the dental office or in a hospital setting. Because of the high incidence of poor cooperation, many of these patients are scheduled for dental care under general anesthesia with preoperative medical assessment. The purpose of this study was to carry out a clinico-statistical survey on dental treatment for handicapped children under general anesthesia. Methods: After approval from the institutional review board, the medical records of 64 handicapped children between 1997 and 2002 were reviewed to determine the patient profiles, anesthesia management, and complications. The charts of these patients, who underwent dental examination, scaling and prophylaxis, and restoration and extraction of teeth under general anesthesia, were reviewed. Results: The mean age was 12.8 years old, and males (53%) predominated females (47%). Twenty-four patients had mental retardation, twelve had autism, six had cerebral palsy, 4 had behavior disorder, others had heart disease, convulsive disorder, etc. Sixty-two had intravenous thiopental with neuromuscular blocker, 2 had intravenous ketamine induction. Nasotracheal intubation was uneventful in 55 patients, nine had orotracheal intubation because of difficult visualization of the larynx. Twenty-one patients experienced postoperative complications in the recovery room, including epistaxis, nasal obstruction, vomiting, airway obstruction, respiratory depression. Conclusions: General anesthesia is a very effective way of completing the dental treatments for disabled children. We emphasize the need to train anesthesiologists in the care of disabled patients.

• Archives of Plastic Surgery
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• v.40 no.2
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• pp.97-103
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• 2013

Background Velopharyngeal insufficiency (VPI) may persist after primary repair of the cleft palate, and surgical correction is necessary in many cases. The purpose of this study is to evaluate the effect of double opposing Z-plasty (DOZ) in cleft palate patients suffering from VPI after primary two-flap palatoplasty. Methods Between March 1999 and August 2005, we identified 82 patients who underwent two-flap palatoplasty for cleft palate repair. After excluding the patients with congenital syndrome and mental retardation, 13 patients were included in the final study group. The average age of the patients who underwent DOZ at was 5 years and 1 month. Resonance, nasal emission, and articulation were evaluated by a speech pathologist. The velopharyngeal gaps were measured before and after surgery. Results Six patients attained normal speech capabilities after DOZ. The hypernasality grade was significantly improved after surgery in all of the patients (P=0.0015). Whereas nasal emission disappeared in 8 patients (61.5%), it was diminished but still persisted in the remaining 5 patients. Articulation was improved in all of the cases. In two cases, the velopharyngeal gap was measured using a ruler. The gap decreased from 11.5 to 7 mm in one case, and from 12.5 to 8 mm in the second case. Conclusions The use of DOZ as a surgical option to correct VPI has many advantages compared with other procedures. These include short surgery time, few troublesome complications, and no harmful effects on the dynamic physiological functioning of the pharynx. This study shows that DOZ can be another option for surgical treatment of patients with VPI after two-flap palatoplasty.

• Journal of Yeungnam Medical Science
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• v.21 no.2
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• pp.242-250
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• 2004

In general, the skeletal class III has the characteristics of mandibular overgrowth with a normal maxillary growth or maxillary undergrowth with a normal mandibular growth And clinical and radiographic evaluations of the patient are needed. However, the treatment plan is not dependent on these evaluations alone, because patient's general condition and hope for aesthetics varies. The aim of this report is to consider the treatment of a medically compromised patient with an anterior open bite and skeletal class III, which showed a severe mandibular overgrowth. In 2003, a 17-year-old boy with epilepsy, mental retardation presented at our clinic complaining of concave profile. A clinical examination showed severe mandibular prognathism with an anterior open bite. The radiographic examination revealed a short cranial base, a moderate maxillary overgrowth, severe mandibular overgrowth and skeletal open bite tendency. In 2004, he was verified to have no potential of growth by hand-and-wrist radiographs and an endocrine examination. He completed the preoperative orthodontic treatment and orthognathic surgery (sagittal split ramus osteotomy, genioplasty). He was evaluated on the first visit, the preoperative period and the postoperative period with a clinical and radiographic examination. At the first visit, the patient showed moderate overgrowth of the maxilla, severe overgrowth of the mandible, and a subsequential skeletal open bite. After the preoperative orthodontic treatment (preoperative period), the patient showed the same skeletal problem as before and a decompensated dentition for orthognathic surgery. After orthognathic surgery, his profile had improved, but he had still a skeletal openbite tendency because the maxillary orthognathic surgery was not performed. Severe mandibular prognathism with a maxillary overgrowth and anterior open bite should be treated by bimaxillary orthognathic surgery. However, one-jaw orthognathic surgery on the remaining the skeletal open bite tendency was performed for his medical problem and facial esthetics. This subsequential open bite should be resolved with a postoperative orthodontic treatment.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.2 no.1
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• pp.116-124
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• 1991

Nursing problems of 48 hospitalized patients with Conduct Disorder at a Child-Adolescent psychiatry inpatient were analyzed by reviewing nursing records. The results showed that the problems such as ineffective individual coping, impaired social interaction, disturbance in self-concept, potential for violence, alteration in parenting, altered growth and development were continued from early to later phase of the hospitalization and the other problems such as self-care deficit, anxiety, sleep disturbance, altered nutrition, hyperthermia were temporary. The etiologic factors related to these problems were underdeveloped ego, low self-esteem, dysfunctional parent-child relationship, some situational crises in family and handicap like mental retardation or epilepsy. Therefore nursing approach for the patients with Conduct Disorder should focus on ego growth and improvement of interpersonal relationship through systematic and long-term nursing plans and interventions for these patients and their family.

• The Journal of Korean Physical Therapy
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• v.15 no.4
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• pp.199-207
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• 2003

This review describes the neurophathological mechanisms that are implicated in perinatal brain injury. Perinatal brain injury is the most important cause of morbidity and mortality to infants, often leading to spastic motor deficits, mental retardation, seizures, and learning impairments. The immature brain injury is usually caused by cerebral hypoxia-ischemia, hemorrhage, or infection. The important form of perinatal brain injury is the hypoxic-ischemic injury and the cerebral hemorrhage. The pathology of hypoxic-ischemic injury include delayed energy failure by mitochondrial dysfunction, neuronal excitotoxicity and vulnerability of white matter in developing brain. The immature brain has the fragile vascular bed of germinal matrix and can not effectively centralize their circulation. Therefore, the cerebral hemorrhage process is considered to be involved in the periventricular leukomalacia.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.552-555
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• 2001

Noonan syndrome is characterized by a Turner-like phenotype and a normal karyotype associated with congenital abnormalities, such as short stature, variable mental retardation, hypertelorism, webbed neck, low posterior hair line, skeletal malformation and congenital cardiovascular defect. Two third of Noonan syndrome have cardiac anormalies, half with pulmonary stenosis. We have experienced two cases of pulmonary stenosis associated with other cardiac anomalies in Noonan syndrome. The first 31-year-old male patient had characteristic appearance of Noonan syndrome with severe infundibular pulmonic stenosis and patent foramen ovate. The second 28-year-old male patient had valvular and subvalvular Pulmonary stenosis with typical Noonan\`s face and stature. Pulmonary valvotomy and hypertrophied muscle bundles in the right ventricular cavity were resected in both cases. Patent foramen ovate was closed directly in the first case. Postoperative follow-up examinations revealed no symptoms and successful outcome.

• Journal of The Korean Dental Society of Anesthesiology
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• v.14 no.4
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• pp.205-211
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• 2014

Background: General anesthesia is a necessary method for successful dental treatment for children, compromised patients and the disabled who have difficulty in cooperation. The aim of this study was to assess dental treatment under general anesthesia at the department of pediatric dentistry, Ajou university hospital on children and the disabled. Methods: 217 general anesthesia from June 2010 to June 2014 were assessed for this study. Patient's distribution, treatment pattern, reasons for general anesthesia, distribution of combined operation, duration of anesthesia, treatment, frequency of general anesthesia and agents for general anesthesia were examined. Results: The proportion of male, the disabled were higher and above 19 years age group was the highest. Combined operation with otolaryngology was highest. Main reasons for general anesthesia were mental retardation for the disabled and uncooperative behavior for the non-disabled. Percentage of restorative treatment was the highest. Average anesthesia duration was 186 minutes and average treatment time was 143 minutes. Most of the airway was maintained by nasotracheal intubation and induction was done by sevoflurane. Conclusions: General anesthesia for dental treatment has been increasing for children, the disabled who have a difficulty of cooperation. The demand for dental treatment under general anesthesia is expected to continuously increase. Therefore, continuous research and studies should be done to establish efficiency and safety of general anesthesia and provide an enhanced environment for treatment.

• Journal of the korean academy of Pediatric Dentistry
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• v.23 no.3
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• pp.601-608
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• 1996

Robinow syndrome or fetal face syndrome is a rare inherited disorder characterized by short stature, mesomelic brachymelia, hypoplastic genitalia, and a typical facial appearence ("fetal face") with frontal bossing, hypertelorism, ear abnormalities, a short upturned nose, long philtrum, micrognathia, and macrocephaly. Intraoral features have included quite a few dental cavities, crowding, hypoplastic uvula, cleft lip or/and cleft palate, gingival hyperplasia, alveolar hyperplasia, enamel hypoplasia, delayed eruption, and congenital missing of the permanent teeth. We report on a 10 years old girl with Robinow syndrome. The patient had most of the typical anomalies of the syndrome and negative family history but, in addition, had mental retardation, hearing loss, and serous otitis media. Intraoral findings included dental cavities, crowding, hypoplastic uvula, repaired cleft palate, and mouth breathing. Dental treatment and V-tube insertion(by dept. of ENT) were performed under general anesthesia. In all cases of Robinow syndrome, thorough evaluation and united treatments with medical specialists should be performed.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.14-18
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• 2016

Pediatric epilepsy can be caused by various conditions, including specific syndromes. 1p36 deletion syndrome is reported in 1 in 5,000-10,000 newborns, and its characteristic clinical features include developmental delay, mental retardation, hypotonia, congenital heart defects, seizure, and facial dysmorphism. However, detection of the terminal deletion in chromosome 1p by conventional G-banded karyotyping is difficult. Here we present a case of epilepsy with profound developmental delay and characteristic phenotypes. A 7-year-and 6-month-old boy experienced afebrile generalized seizure at the age of 5 years and 3 months. He had recurrent febrile seizures since 12 months of age and showed severe global developmental delay, remarkable hypotonia, short stature, and dysmorphic features such as microcephaly; small, low-set ears; dark, straight eyebrows; deep-set eyes; flat nasal bridge; midface hypoplasia; and a small, pointed chin. Previous diagnostic work-up, including conventional chromosomal analysis, revealed no definite causes. However, array-comparative genomic hybridization analysis revealed 1p36 deletion syndrome with a 9.15-Mb copy loss of the 1p36.33-1p36.22 region, and fluorescence in situ hybridization analysis (FISH) confirmed this diagnosis. This case highlights the need to consider detailed chromosomal study for patients with delayed development and epilepsy. Furthermore, 1p36 deletion syndrome should be considered for patients presenting seizure and moderate-to-severe developmental delay, particularly if the patient exhibits dysmorphic features, short stature, and hypotonia.

• Journal of mucopolysaccharidosis and rare diseases
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• v.2 no.1
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• pp.19-22
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• 2016

Purpose: Mucolipidosis type II (ML II), also known as I-cell disease is an autosomal recessive inherited disorder of lysosomal enzyme transport caused by a deficiency of the uridine diphosphate (UDP)-N-acetylglucosamine:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase (GlcNAc-phosphotransferase). Clinical manifestations are skeletal abnormalities, mental retardation, cardiac disease, and respiratory complications. A severely and rapidity progressive clinical course leads to death before 10 years of age. Methods/Results: In this study we diagnosed three cases of prenatal ML II in two different at-risk families. We compared two procedures -biochemical analysis and molecular analysis - for the prenatal diagnosis of ML II. Both methods require an invasive procedure to obtain specimens for the diagnosis. Biochemical analysis requires obtaining cell cultures from amniotic fluid for more than two weeks, and would result in a late diagnosis at 19 to 22 weeks of gestation. Molecular genetic testing by direct sequence analysis is usually possible when mutations are confirmed in the proband. Molecular analysis has an advantage in that it can be performed during the first-trimester. Conclusion: Molecular diagnosis is a preferable method when a prompt decision is necessary.