• Archives of Craniofacial Surgery
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• v.13 no.1
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• pp.57-59
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• 2012

Purpose: Congenital sinus of the upper lip is extremely rare and only 3 cases have been reported domestically. We report a case of congenital sinus of midline upper lip, which was found in an adolescent patient. Methods: A 14-year-old girl presented with a small pit on midline of the upper lip, which was visible at birth. The patient had never been treated for the congenital sinus because it was asymptomatic. Surgical excision under local anesthesia was performed. Results: The sinus had a tract extending into 5 mm posteroinferior and had not penetrated the oral cavity. Histological examination showed a fistulous tract lined by keratinized squamous epithelium. After complete excision, there was no recurrence and we obtained a satisfactory cosmetic result. Conclusion: Congenital sinus of the midline upper lip is extremely rare. This is a special case that is reported because it did not cause symptoms for the patient until she reached adolescence.

• Journal of Korean Neurosurgical Society
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• v.37 no.1
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• pp.29-33
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• 2005

Objective: Congenital dermal sinus is a rare congenital disease that results from the failure of the neuroectoderm to separate from the surface ectoderm during the process of neurulation, where there is communication between the skin and the deeper structures. Their pathogenesis, clinical course and treatment strategy are well known. We analyze our series and compare our results with other series. Methods: Twenty patients were diagnosed as congenital dermal sinus and confirmed pathologically from October 1986 to July 2003 at our hospital. We studied the patients' clinical manifestations, radiological findings and pathological profiles. Results: Seven cases were located in the suboccipital area and 13 cases were located in the spinal area. Interestingly, 4 of 13 spinal lesion cutaneous openings were located lower than the 3rd sacral body level. 8 of 20 lesions were terminated at neural structures, 4 of 20 lesions were terminated at the intradural portion and others terminated at the extradural portion. Nine anomalies were combined with the dermal sinus, including 4 lipomas, 2 Currarino's triad, 1 encephalocele, 1 myelomeningocele and 1 diastematomyelia. Eleven patients had dermoid tumors. Conclusion: Congenital Dermal Sinus must be surgically removed immediately if they are diagnosed. The surgical procedure of congenital dermal sinus is complete removal, but in some cases, complete removal is impossible. In those cases, we removed all epithelial tissues. We consider sacrococcygeal dimple almost invariably have no connection with intraspinal structures. But, if other cutaneous manifestations are combined with cutaneous pits, it can communicate with the sacrococcygeal dimple.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.265-272
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• 1985

Congenital aneurysm of sinus of Valsalva is one of the rare congenital heart disease, which is usually asymptomatic until rupture. The aneurysm usually ruptures into a cardiac chamber and produces an aorto-intracardiac fistula. Ruptured aneurysm is a grave lesion in that it causes heart failure and subsequent death. If, however, it is discovered in its early stages and operated on properly, it can be corrected with considerable success. Form January 1975 through December 1984, 18 consecutive patients with congenital aneurysm of sinus of Valsalva underwent corrective surgery using total cardiopulmonary bypass in our department of Thoracic Surgery. 1. The incidence was about 0.9% of surgical cases of congenital heart disease during that period. 2. 13 were males and 5 females, with ages ranging 12 years to 52 years. 3. Associated anomalies were VSD in 14, infundibular PS in 1, aberrant muscle band in RVOT in 1, and secondary aortic insufficiency in 9. 4. 17 were suggested to arise from right coronary sinus and 1 from noncoronary sinus; Among 17, 12 ruptured into right ventricle, and one from noncoronary sinus into right atrium. 5. Surgical correction was performed by means of direct suture closure with combined pledget or patch graft after aneurysm resection, and associated lesions were also corrected simultaneously. 6. There was only one case of operative mortality, and all the other patients were relatively uneventful in their follow-up studies.

• Archives of Plastic Surgery
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• v.37 no.6
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• pp.827-830
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• 2010

Purpose: Congenital spinal dermal sinus tract is a rare lesion connecting skin to deeper structures including neural tissue. It results from the failure of the neuroectoderm to separate from the cutaneous ectoderm in the third to fifth week of gestation. The common locations are the lumbosacral and occipital regions. Sometimes it extends to spinal canal. In this paper we report a case of congenital spinal dermal sinus tract in the coccyx. Methods: A 21-month-old male child born after an uncomplicated full-term pregnancy was admitted to our institute with a midline dermal sinus and a cartilaginous protrusion in the coccygeal region. There were no signs of infection. Neurologic examination showed no functional deficit in both lower limbs. He was treated with complete excision of the tract and an underlying accessory cartilage. Results: The spinal dermal sinus tract was extended from the skin to the coccyx. The stalk was loosely attached to the accessory cartilage of coccyx. At that point, it was dissected from the accessory cartilage and resected. The accessory cartilage was also resected at the bone and cartilage junction. During the follow-up period of 6 months, the wound healed well without any complication nor recurrence. Conclusion: Congenital spinal dermal sinus tract is known as a form of spinal dysraphism. In order to prevent complications, timely surgical intervention including complete resection of sinus tract with correction of associated abnormalities is of utmost importance.

• Journal of Chest Surgery
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• v.27 no.7
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• pp.631-633
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• 1994

Congenital bronchoesophageal fistula is a rare anomaly that can appear in adult uncommonly. Especially sick sinus syndrome with bronchoesophageal fistula is very uncommon.The patient was 53 years old male who admitted for chronic coughing recurrent lobar pneumonia on RLL since few years ago. And he had familial history of sick sinus syndrome.We confirmed the fistula by barium swallow examination and performed ligation of the fistula and pacemaker implantation.

• Archives of Plastic Surgery
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• v.38 no.1
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• pp.73-76
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• 2011

Purpose: Congenital midline upper lip sinus is a rare lesion. There are two postulates that can account for the formation of the upper lip sinus based on two major theories of the development of the face: the fusion theory and the merging theory. However, congenital midline lower lip sinus is very rarely reported and described. We report a case of a congenital midline sinus of the lower lip in a 6-year-old female. Methods: A 6-year-old girl presented with a nipple like swelling on the midline lower lip. Physical examination revealed about $5{\times}5$ mm protruding round mass with a just small opening that was non-tender to palpation. The mass was not associated with any skin changes. It umbilicated at the apex and contains a fistulous tract, discharging clear fluid. Only, simple radiologic finding shows bony spur on the lower one third of mandibular symphsis. Results: A small transverse ellipse is made around the opening and elevated mass with sharpe dissection. The tract is excised using the probe and dye as the guide. The tract was extended to periosteum of the lower one third of the mandible. The tract and involved periosteum were excised en bloc, and removed protrusion of the mandibular bone using diamond burr. Microscopic examination of the resected sinus revealed the fistulous tract itself, consisting of fibrous connective tissue covered with cornified stratified squamous epithelium, was observed in the center of the sample. In 6 months follow-up, This patient had a good result was obtained by the method of fistulectomy alone. Conclusion: Midline cranoifacial fistulas represent rare lesions resulting from abnormal fusion of embryologic structures. Our case report describes the excision of a congenital midline sinus of the lower lip in a 6-year-old female. This case represents the first report of a lower lip sinus presenting in a girl as a mass in the skin of the chin with extension to the midline of the mandible. However, the etiology of this rare congenital sinus remains obscure.

• Archives of Craniofacial Surgery
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• v.16 no.2
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• pp.63-66
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• 2015

Background: Preauricular sinuses represent a common congenital abnormality in children. Classically, a preauricular sinus manifests as a small opening, usually near the anterior limb of ascending helix. The difficulty in the surgical treatment of preauricular sinus is the high recurrence rate. The aim of this article is to review the outcomes of preauricular sinus and to introduce our surgical technique and its prognosis. Methods: A single-institutional retrospective review was performed for all patients who had undergone excision of congenital periauricular sinus between October 2007 and April 2014. Medical records were reviewed for demographic information, wound complication, and recurrence rate. The sinus tract was visualized with the aid of preoperative dye instillation and intraoperative probe insertion. The skin next to the sinus opening was incised elliptically, and the tract itself was dissected medially to the end of the sinus tract and posteriorly to the cartilage of the ascending helix. Results: The review identified 44 patients for a total of 57 preauricular sinus tracts. The mean age at time of operation was 16.3 years with a range from 9 months to 65 years. Unilateral preauricular sinus tract was present in 31 patients (11 right and 20 left preauricular tract), and 13 patients had bilateral sinus tract. None of the patients had experienced wound issues postoperative, and there were no recurrent sinus tract formation or infection. Conclusion: Using a combination of dye instillation, probe insertion, and modified dissection, we were able to achieve a recurrence free series of preauricular sinus tract excision among a heterogenous group of patients. A large patient series is necessary to replicate the results of this study.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.661-666
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• 1989

Congenital coronary arterial fistulae are the most prevalent hemodynamically significant congenital coronary artery malformations. Definition of congenital coronary arterial fistula is a direct communication between a coronary artery and the lumen of one of the four cardiac chambers or coronary sinus or SVC, pulmonary artery or pulmonary vein close to the heart. It is often associated with additional congenital or acquired heart disease. A 49 year old male patient was admitted with the chief complaints of anginal pain and exertional dyspnea for 9 months. He was diagnosed as the right coronary arterial fistula combined with right coronary arteriosclerotic stenosis and old inferior myocardial infarction by cardiac evaluation. The right coronary arterial fistula was communicated between the just distal portion of acute marginal branch and coronary sinus. The operative procedure was as followings; after suture ligation of fistula opening in the coronary sinus under beating heart, coronary arterial bypass grafting with saphenous vein was performed at the just proximal portion of the posterior descending branch under cardiopulmonary bypass. The postoperative course was uneventful and he was discharged without anginal pain at the 8th postoperative day.

• Journal of Korean Neurosurgical Society
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• v.57 no.3
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• pp.225-228
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• 2015

Congenital dermal sinus (CDS) is a type of occult spinal dysraphism characterized by a midline skin dimple. A 12-month-old girl presented with fever and ascending quadriparesis. She had a midline skin dimple in the upper sacral area that had been discovered in her neonatal period. Imaging studies revealed a holocord intramedullary abscess and CDS. Overlooking CDS or misdiagnosing it as benign sacrococcygeal dimple may lead to catastrophic infection and cause serious neurological deficits. Therefore, further imaging work-up or consultation with a pediatric neurosurgeon is recommended following discovery of any atypical-looking dimples in the midline.

• Advances in pediatric surgery
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• v.7 no.1
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• pp.7-14
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• 2001

Congenital anomalies of the head and neck region such as preauricular sinus and skin tag, thyroglossal duct cyst, branchial anomaly, cystic hygroma and dermoid cyst are common in pediatric population. It is important for pediatricians and pediatric surgeons to be familiar with the embryology and the anatomical characteristic of these lesions in order to diagnose and treat them properly. Three hundred and nineteen patients with congenital head and neck anomalies treated at Hanyang University Hospital between 1980 and 1999 were reviewed to determine the relative frequency of the anomalies and to analyze the method of management. Eight-four (25.1 %) of 335 lesions were preauricular sinus and skin tag, 81 (24.2 %) were thyroglossal duct cyst, 81 (24.2 %) branchial anomaly, 58 (17.3 %) cystic hygroma and 31 were (9.2 %) dermoid cyst. The male-to-female ratio was 1.4:1. Thyroglossal duct cyst most commonly present at 3-5years, however branchial anomalies commonly are diagnosed in children younger than 1 year. Preauricular sinus showed familial tendency in three patients and was bilateral is 33.8 %. Most head and neck anomalies in children have specific clinical and anatomical characterics. A careful history and physical examination is very useful for diagnosis and proper management. Experienced pediatric surgeons should do the initial surgery since the recurrence rate after incomplete surgical excision can be high.