• Journal of Chest Surgery
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• v.39 no.2 s.259
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• pp.145-149
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• 2006

The surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis remains a challenge. The Rastelli operation or Lecompte operation is the preferred surgical procedure, but its long-term results are not optimal because of a warped left ventricular outflow tract through a space-occupied intraventricular tunnel and a contrived right ventricular outflow tract. We performed a half-turned truncal switch operation as an alternative surgical procedure in a 3-year-old boy (weighing 9.6 kg) with this anomaly. Postoperative echocardiography showed laminar flow through straight and nonobstructive aortic and pulmonary ventricular outflow tracts.

• Archives of Plastic Surgery
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• v.39 no.1
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• pp.63-66
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• 2012

Nevus comedonicus is a type of hamartoma that arises from a developmental anomaly of the mesodermal part of the pilosebaceous gland. In most cases of nevus comedonicus, an acne-like skin condition develops. Repeated inflammation can cause a morphological change to the cyst, papule, to abscess. We experienced a case of congenital nevus comedonicus, which led to the formation of large multiple cysts. A 50-year-old man was referred with a $12.5{\times}10cm$ lobulated mass on the posterior neck and upper back. The patient had a widespread presence of nevus comedonicus in the region ranging from the right superior chest to the posterior neck. The patient had a 30-year history of six prior excisions. A magnetic resonance imaging review led to a diagnosis of nevus comedonicus. Surgical treatment consisted of excision of the mass and wide excision for the patch type of nevus comedonicus around the neck. On histopathology, multiple masses were diagnosed as typical cysts containing keratinized tissue. The diffuse comedone lesions were diagnosed as nevus comedonicus. This case shows that large, multiple cysts can occur as a long-term complication of nevus comedonicus, and also highlights the importance of radical resection to prevent its further invagination.

• The Journal of Korean Academy of Orthopedic Manual Physical Therapy
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• v.9 no.2
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• pp.5-11
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• 2003

Thoracic outlet syndrome is actually a collection of syndromes brought about by abnormal compression of the neurovascular bundle by bony, ligamentous or muscular obstacles between the cervical spine and the lower border of the axilla. First of all a syndrome is defined as a group of signs and symptoms that collectively characterize or indicate a particular disease or abnormal condition. The neurovascular bundle which can suffer compression consists of the brachial plexus plus the C8 and T1 nerve roots and the subclavian artery and vein. The brachial plexus is the network of motor and sensory nerves which innervate the arm, the hand, and the region of the shoulder girdle. The vascular component of the bundle, the subclavian artery and vein transport blood to and from the arm. the hand. the shoulder girdle and the regions of the neck and head. The bony, ligamentous, and muscular obstacles all define the cervicoaxillary canal or the thoracic outlet and its course from the base of the neck to the axilla or arm pit. Look at the scheme of this region and it all becomes more easily understood. Compression occurs when the size and shape of the thoracic outlet is altered. The outlet can be altered by exercise, trauma, pregnancy, a congenital anomaly, an exostosis, postural weakness or changes. Thoracic outlet syndrome has been described as occurring in a diverse population. It is most often the result of poor or strenuous posture but can also result from trauma or constant muscle tension in the shoulder girdle. The first step to beginning any treatment begins with a trip to the doctor. Make a list of all of the symptoms which seem to be present even if the sensations are vague. Make a note of what activities and positions produce or alleviate the symptoms and the time of day when symptoms are worst. Also, note when the symptoms first appeared. This list is important and should also include any questions one may have.

• Tuberculosis and Respiratory Diseases
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• v.68 no.6
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• pp.358-362
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• 2010

Intralobar pulmonary sequestration is a rare congenital lung anomaly. It is defined as a portion of nonfunctioning lung parenchyma that receives its blood supply from an anomalous systemic artery. Patients often present with chronic or recurrent pneumonia. A chest radiograph may show a cystic lesion with air-fluid levels in the lung base. A high index of suspicion is needed for a diagnosis. Surgical removal of a symptomatic intralobar pulmonary sequestration is generally the treatment of choice. Identifying the aberrant artery is a difficult problem when resecting a pulmonary sequestration. The thoracic and abdominal aortas are the most common origins for the abnormal blood supply. However, arterial supply from the celiac artery is quite rare. We present a case of intralobar pulmonary sequestration with the blood supply originating from the celiac artery.

• Journal of Genetic Medicine
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• v.12 no.1
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• pp.44-48
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• 2015

Townes-Brocks syndrome (TBS) is a rare genetic disorder characterized by the classic triad of congenital anomalies of the anus, thumbs, and ears, with variable expressivity. Additionally, renal malformations, cardiac anomalies, and endocrine and eye abnormalities can accompany TBS, although less frequently. TBS is inherited in an autosomal dominant fashion; however, about 50% of patients have a family history of TBS and the remaining 50% have de novo mutations. SALL1, located on chromosome 16q12.1, is the only causative gene of TBS. SALL1 acts as a transcription factor and may play an important role in inducing the anomalies during embryogenesis. Clinical features of TBS overlap with those of other multiple anomaly syndromes, such as VACTERL syndrome, Baller-Gerold syndrome, Goldenhar syndrome, cat eye syndrome, and Holt-Oram syndrome. Consequently, there are some difficulties in differential diagnosis based on clinical manifestations. Herein, we report a Korean family with two generations of TBS that was diagnosed based on physical examination findings and medical history. Although the same mutation in SALL1 was identified in both the mother and the son, they displayed different clinical manifestations, suggesting a phenotypic diversity of TBS.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.19 no.3
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• pp.233-242
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• 1997

Recently the goal of orthognathic surgery has been focused on esthetic improvement of the patients. Also early corrective surgery was favorable selected by most of the oral and maxillofacial surgeons. We should consider the etiologic factor of the patient's dentofacial deformities when treatment is planned, because this is the major factor in estimating the predictability or stability of result. The more researches were carried on the etiologic factors of the dentofacical deformities, The more possibility of the early surgical correction will be increased. The authors analyzed about etiologic factors and epidemiologic studies of the forth patients who had received the orthognathic surgery. The analyzed results were as follows: 1. The predilection ration between male and female was 17:23, and 32 patients (80%) of 40 patients were aged twenties. 2. 26patients(65%) complained estetic problems as well as functional problems. 10 patients(25%) complained only esthetic problems, and 4 of 40 patients complained only functional problems. 3. Mandibular prognathism was found to be done most frequently(25, 39%). Facial asymmetry (13, 20%) and angle hypertrophy were found to be next in sequence. 4. Sagittal split ramus ostetomy was done most frequently(27, 35%). Lefort I osteotomy(13, 17%), angle reduction (12,16%), and genioplasty(11, 15%) were done also. 5. The number of the cases due to nonspecific etiologic factor was 22(55%), that of cases due to inhertied tendency was 12(30%), that of cases due to congenital anomaly was 3(7.5%), and that of cases due to trauma was 3(7.5%). 6. The number of patients who got only maxilliary surgery was 2(5%), that of patients who got only mandibular surgery was 23(57.5%), and that of patients who got simultaneous two jaw surgery was 15(37.5%).

• Advances in pediatric surgery
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• v.9 no.1
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• pp.1-5
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• 2003

Preauricular sinus and preauricular skin tag are common childhood congenital anomalies. It is important for the pediatric surgeon to be familiar with the embryology and differentiation of head and neck structure to accurately diagnose and treat these lesions. Seventy eight patients with preauricular sinus and twenty-eight with preauricular skin tag treated in the Department of Pediatric Surgery at Hanyang University Hospital from January 1981 to May 2002 were reviewed to determine relative frequency, clinical classification and appropriate treatment. The male to female ratio of preauricular sinus was 1:1.2, and preauricular skin tag was 1:1. The most commonly presenting age of sinus and skin tag was before 5 year (62.8%) and before 1 year (53.6%). Twenty nine of 78 cases of preauricular sinuses were on the left, 25 on the right and 24 bilateral. Signs of infection were seen in 73.0% of patients with preauricular sinus at operation. Only 31.3% of lesions were infected in patients less than one year of age, but 89.5% between 3-5 years and 100% between 5-8 years. Cartilage was present in five patients with preauricular skin tag. Although re-operation due to wound infection was necessary in four cases, no recurrences were found. The preauricular sinus is a common anomaly in childhood, and has had a relatively high recurrence rate. But most of the recurrences were due to incomplete resection because of combined infection. Initial proper diagnosis and early operation are very important. Identification of the exact anatomical location of sinus tract is necessary because total excision of the lesions including those tracts is the only way to prevent recurrence.

• Physical Therapy Korea
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• v.9 no.2
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• pp.43-50
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• 2002

This study was designed to identify the effects of walking conditions (normal walking vs. toe-walking) on electromyographic (EMG) activity of gastrocnemius, tibialis anterior, and soleus muscle. Seven healthy adult males participated in this study. The exclusion criteria were orthopedic or neurologic disease, congenital anomaly or acquired deformity, or pain in low back or lower extremities. The maximal voluntary isometric contraction for each muscle was used for the reference contraction, and EMG activity of each muscle during normal walking and toe-walking was expressed as a percentage of reference contraction. The gait cycle was determined with two foot switches, and gait was normalized as 100% gait cycle for each condition. The maximal values of EMG activity in terminal stance (30~50% of gait cycle) of each condition were compared for data analysis. No significant differences were found in EMG activity of the tibialis anterior and soleus (p>.05), whereas significant decrement was found in EMG activity of gastrocnemius during toe-walking compared to normal walking (p<.05). There is a limitation to generalize the results of this study, because small number of subjects participated for this study and only EMG was used for data collection. The treatment methods should be developed to improve gait efficiency by substituting the weakened muscles secondary to upper motor neuron, or by strengthening the distal muscles in lower extremity.

• Tuberculosis and Respiratory Diseases
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• v.39 no.6
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• pp.548-553
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• 1992

The Unilateral absence of a pulmonary artery (UAPA) is an uncommon congenital anomaly. Approximately 160 cases have been reported in the literature since Frantzel's first report in 1968. Most of the patients with UAPA are asymptomatic but some patients may suffer from recurrent respiratory infections, hemoptysis, or pulmonary hypertension. The diagnosis could be suspected from the chest roentgenogram and lung scan, and definitely confirmed by pulmonary angiography. We experienced a case of UAPA in a 39-year-old male with the recurrent hemoptysis. Chest X-ray revealed that the left lung volume was moderately decreased and the heart and mediastinum were displaced to the left side. Lung perfusion scan showed that the left lung was not perfused. Pulmonary angiography revealed the absence of the left main pulmonary artery. Aortic arch and descending aorta on aortogram were right sided. Blood supply to the left lung was originated from numerous systemic collaterals from intercostal and brachiocephalic origin. No other intrinsic or internal abnormalities of the cardiac chambers were noted.

• Tuberculosis and Respiratory Diseases
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• v.42 no.5
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• pp.767-771
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• 1995

The bronchial artery-pulmonary vein malformation should be called the systemic artery-to-pulmonary vein arterioveonus malformation in the lung. Although pulmonary arteriovenous malformation has been well documented in intrapulmonary arteriovenous malformation, the systemic artery-to-pulmonary vein arteriovenous malformation is rare. Most patients with systemic artery-to-pulmonary vein arteriovenous malformation is asymptomatic and the diagnosis of these anomaly may be done by continuous murmur or abnormal chest X-ray on the physical examination. The pathogenesis of this condition is congenital malformation which explains these anastomoses between the pulmonary vein and accessory brachial arteries and acquired malformation which explains development of new blood vessel to supply large enough to cause significant systemic-pulmonary shunts due to inflammation secondary to infection, trauma, or previous surgery. We experienced a case of the bronchial artery-pulmonary vein malformation which was detected on angiography in 20-year-old women whose chief complain is hemoptysis. This massive hemoptysis was controlled by selective brachial artery embolization with Gelfoam and Ivalon particles.