• Journal of the korean academy of Pediatric Dentistry
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• v.40 no.3
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• pp.209-215
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• 2013

Cleft lip and palate, the most common craniofacial anomalies, are severe congenital defects that have an incidence of 0.28 to 3.74 per 1000 live births. Although there has been great improvement in the field of cleft surgery, surgical approach cannot be the single solution to resolve the various problems encountered in patients with cleft lip and palate. The concept of presurgical infant orthopedics (PSIO) for gradual closure of the cleft gap and simplified surgical performance was first introduced by McNeil in 1950. Recently, there are many attempts not only to approximate the alveolar segments but also to reshape the nasal cartilage. Three infants with unilateral cleft lip and palate were referred from the department of Plastic Surgery for presurgical nasoalveolar molding (PNAM). Maxillary appliances using resin with orthodontic wire were fabricated. Then these appliance was applied until patients underwent lip surgery. In all cases, the patients could wear the appliance all day since they were able to eat even with the appliance on, This resulted in significant improvements in the nasal symmetry were found. Our appliance, namely K-NAM, extends the wearing time within the limited period and as a result it is expected to maximize the treatment effects. Used properly, this appliance would play a major role in enhancing nasal symmetry with satisfactory results.

• Clinical and Experimental Pediatrics
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• v.56 no.7
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• pp.275-281
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• 2013

Temporal lobe epilepsy (TLE) is the most common type of medically intractable epilepsy in adults and children, and mesial temporal sclerosis is the most common underlying cause of TLE. Unlike in the case of adults, TLE in infants and young children often has etiologies other than mesial temporal sclerosis, such as tumors, cortical dysplasia, trauma, and vascular malformations. Differences in seizure semiology have also been reported. Motor manifestations are prominent in infants and young children, but they become less obvious with increasing age. Further, automatisms tend to become increasingly complex with age. However, in childhood and especially in adolescence, the clinical manifestations are similar to those of the adult population. Selective amygdalohippocampectomy can lead to excellent postoperative seizure outcome in adults, but favorable results have been seen in children as well. Anterior temporal lobectomy may prove to be a more successful surgery than amygdalohippocampectomy in children with intractable TLE. The presence of a focal brain lesion on magnetic resonance imaging is one of the most reliable independent predictors of a good postoperative seizure outcome. Seizure-free status is the most important predictor of improved psychosocial outcome with advanced quality of life and a lower proportion of disability among adults and children. Since the brain is more plastic during infancy and early childhood, recovery is promoted. In contrast, long epilepsy duration is an important risk factor for surgically refractory seizures. Therefore, patients with medically intractable TLE should undergo surgery as early as possible.

• Clinical and Experimental Pediatrics
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• v.51 no.4
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• pp.435-438
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• 2008

The Cerebro-oculo-facio-skeletal (COFS) syndrome is a rare autosomal recessive disorder characterized by multiple abnormalities that involve the brain, face, eyes, and extremities. COFS syndrome is regarded as a degenerative disorder of the brain and spinal cord caused by a mutation of the DNA repair genes. We report on an 8-month-old girl with COFS syndrome who exhibited growth and developmental delay, hypotonia, microcephaly, nystagmus, cleft palate, widely separated nipples, inguinal hernia, camptodactyly, and rocker-bottom feet with vertical talus.

• Archives of Craniofacial Surgery
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• v.13 no.1
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• pp.54-56
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• 2012

Purpose: Dandy-Walker syndrome is a rare congenital brain malformation that occurs one in every 25,000-35,000 live births, mostly in females. It is characterized by cystic enlargement of the fourth ventricle, agenesis or hypogenesis of the cerebellar vermis and enlargement of the posterior fossa. In this report, the authors aimed to address a rare case of a 14-months-old female Dandy-Walker syndrome patient that is presented with submucous cleft palate. Methods: A 14-months-old female patient admitted to our outpatient clinic, via the department of pediatrics, with the complaints of nasal regurgitation, choking and breathing difficulties. She was diagnosed as Dandy-Walker syndrome by magnetic resonance imaging evaluation, at another hospital and underwent a shunt operation for the hydrocephalus continuing treatments. On physical examination, she had structural abnormality of bifid uvula, and palpable notch in the posterior surface of the hard palate. Her submucous cleft palate was corrected, which used a double opposing Z-plasty under general anesthesia. Results: In a follow-up period of 2 months, no complications, such as wound dehiscence, necrosis and infection occurred, which shows satisfactory results. She consulted with pediatric neurologists and physical therapists for further evaluation and management of the abnormalities in the central nervous system. Conclusion: Dandy-Walker syndrome patient with a cleft palate is a very rare case to find, which only a few cases are reported around the world. Authors would like to share this case of Dandy-Walker syndrome patient, with submucous cleft palate, who underwent a double opposing Z-plasty that shows satisfactory results.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.40
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• pp.20.1-20.10
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• 2018

Background: In the absence of clear guidelines for facial aesthetic surgery, most surgeons rely on expert intuitive judgement when planning aesthetic and reconstructive surgery. One of the most famous theories regarding "ideal" facial proportions is that of the golden proportion. However, there are conflicting opinions as to whether it can be used to assess facial attractiveness. The aim of this investigation was to assess facial ratios of professional black models and to compare the ratios with the golden proportion. Methods: Forty photographs of male and female professional black models were collected. Observers were asked to assign a score from 1 to 10 (1 = not very attractive, 10 = very attractive). A total of 287 responses were analysed for grading behaviour according to various demographic factors by two groups of observers. The best graded photographs were compared with the least well-graded photographs to identify any differences in their facial ratios. The models' facial ratios were calculated and compared with the golden proportion. Results: Differences in grading behaviour were observed amongst the two assessment groups. Only one out of the 12 facial ratios was not significantly different from the golden proportion. Conclusions: Only one facial ratio was observed to be similar to the golden proportion in professional model facial photographs. No correlation was found between facial ratios in professional black models with the golden proportion. It is proposed that an individualistic treatment for each ratio is a rather better method to guide future practice.

• Pediatric Infection and Vaccine
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• v.29 no.2
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• pp.118-123
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• 2022

A dermatophyte is a zoonotic infection that causes infection on the skin, hair, and nails and is transmitted through contact. The species of Microsporum, Trichophyton, and Epidermophyton are the major dermatophytes that infect humans. Tinea capitis is a dermatophyte infection in the scalp, and it may progress to kerion celsi, including severe redness, swelling, and pus formation. Kerion celsi is sometimes misdiagnosed as a bacterial infection or a tumor. Trichophyton verrucosum is a zoophilic dermatophyte that mainly causes infection in cattle. It can be spread to dairy farmers or ranchers who have frequent contact with infected cattle. We report a pediatric case who received scalp tumor excision and a split-thickness skin graft for extensive and severe inflammatory scalp tumors that occurred after contact with cattle with ringworm. Finally, the patient was diagnosed with kerion celsi caused by T. verrucosum infection. This was based on the patient's medical history, clinical manifestations, and histopathologic findings.

• Clinical and Experimental Pediatrics
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• v.58 no.11
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• pp.454-458
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• 2015

Compartment syndrome is a rare but devastating condition that can result in permanent neuromuscular or soft tissue injuries. Extravasation injuries, among the iatrogenic causes of compartment syndrome, occur under a wide variety of circumstances in the inpatient setting. Total parenteral nutrition via a peripheral route is an effective alternative for the management of critically ill children who do not obtain adequate nutrition via the oral route. However, there is an inherent risk of extravasation, which can cause compartment syndrome, especially when detected at a later stage. Herein, we report a rare case of compartment syndrome and skin necrosis due to extravasation, requiring emergency fasciotomy and skin graft in a 7-month-old boy who was treated with peripheral parenteral nutrition via a pressurized infusion pump. Although we cannot estimate the exact time at which extravasation occurred, the extent and degree of the wound suggest that the ischemic insult was prolonged, lasting for several hours. Pediatric clinicians and medical teams should carefully examine the site of insertion of the intravenous catheter, especially in patients receiving parenteral nutrition via a peripheral intravenous catheter with a pressurized infusion pump.

• Pediatric Infection and Vaccine
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• v.25 no.2
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• pp.101-106
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• 2018

A 6-year-old boy with underlying hemolytic anemia of unknown etiology, atopic dermatitis, and recurrent urticaria visited our hospital because of acute respiratory failure induced by influenza A. Despite mechanical ventilation after endotracheal intubation along with inhalation of nitric oxide, respiratory acidosis and hypoxemia persisted. Veno-venous extracorporeal membrane oxygenation (VV ECMO) insertion was performed to provide respiratory support. After performing flexible bronchoscopy, we found that thick mucus plugs were obstructing the right bronchus intermedius and the upper lobe orifice. After bronchial washing and removal of the plugs, we were able to wean the patient off VV ECMO and transfer him to the general ward. He was discharged without any neurologic or pulmonary sequelae.

• Journal of Korean Neurosurgical Society
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• v.59 no.3
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• pp.204-213
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• 2016

Syndromic craniosynostosis has severe cranial stenosis and deformity, combined with hypoplastic maxillary bone and other developmental skeletal lesions. Among these various lesions, upper air way obstruction by hypoplastic maxillary bone could be the first life-threatening condition after birth. Aggressive cranial vault expansion for severely deformed cranial vaults due to multiple synostoses is necessary even in infancy, to normalize the intracranial pressure. Fronto-orbital advancement (FOA) is recommended for patients with hypoplastic anterior part of cranium induced by bicoronal and/or metopic synostoses, and posterior cranial vault expansion is recommended for those with flattening of the posterior part of the cranium by lambdoid synostosis. Although sufficient spontaneous reshaping of the cranium can be expected by expansive cranioplasty, keeping the cranial bone flap expanded sufficiently is often difficult when the initial expansion is performed during infancy. So far distraction osteogenesis (DO) is the only method to make it possible and to provide low rates of re-expansion of the cranial vault. DO is quite beneficial for both FOA and posterior cranial vault expansion, compared with the conventional methods. Associated hydrocephalus and chronic tonsillar herniation due to lambdoid synostosis can be surgically treatable. Abnormal venous drainages from the intracranial space and air way obstruction should be always considered at any surgical procedures. Neurosurgeons have to know well about the managements not only of the deformed cranial vault and the associated brain lesions but also of other multiple skeletal lesions associated with syndromic craniosynostosis, to improve treatment outcome.

• Clinical and Experimental Pediatrics
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• v.64 no.11
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• pp.559-572
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• 2021

The International Society for the Study of Vascular Anomalies classifies vascular anomalies into vascular tumors and vascular malformations. Vascular tumors are neoplasms of endothelial cells, among which infantile hemangiomas (IHs) are the most common, occurring in 5%-10% of infants. Glucose transporter-1 protein expression in IHs differs from that of other vascular tumors or vascular malformations. IHs are not present at birth but are usually diagnosed at 1 week to 1 month of age, rapidly proliferate between 1 and 3 months of age, mostly complete proliferation by 5 months of age, and then slowly involute to the adipose or fibrous tissue. Approximately 10% of IH cases require early treatment. The 2019 American Academy of Pediatrics clinical practice guideline for the management of IHs recommends that primary care clinicians frequently monitor infants with IHs, educate the parents about the clinical course, and refer infants with high-risk IH to IH specialists ideally at 1 month of age. High-risk IHs include those with life-threatening complications, functional impairment, ulceration, associated structural anomalies, or disfigurement. In Korea, IHs are usually treated by pediatric hematology-oncologists with the cooperation of pediatric cardiologists, radiologists, dermatologists, and plastic surgeons. Oral propranolol, a nonselective beta-adrenergic antagonist, is the first-line treatment for IHs at a dosage of 2-3 mg/kg/day divided into 2 daily doses maintained for at least 6 months and often continuing until 12 months of age. Topical timolol maleate solution, a topical nonselective beta-blocker, may be used for small superficial type IHs at a dosage of 1-2 drops of 0.5% gel-forming ophthalmic solution applied twice daily. Pulse-dye laser therapy or surgery is useful for the treatment of residual skin changes after IH involution.