• Clinical and Experimental Pediatrics
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• 제54권5호
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• pp.219-223
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• 2011

McKusick-Kaufman syndrome (MKS) is an autosomal recessive multiple malformation syndrome characterized by hydrometrocolpos (HMC) and postaxial polydactyly (PAP). We report a case of a female child with MKS who was transferred to the neonatal intensive care unit of Seoul National University Children's Hospital on her 15th day of life for further evaluation and management of an abdominal cystic mass. She underwent abdominal sonography, magnetic resonance imaging, genitography and cystoscopy which confirmed HMC with a transverse vaginal septum. X-rays of the hand and foot showed bony fusion of the left third and fourth metacarpal bones, right fourth dysplastic metacarpal bone and phalanx, right PAP and hypoplastic left foot with left fourth and fifth dysplastic metatarsal bones. In addition, she had soft palate cleft, mild hydronephroses of both kidneys, hypoplastic right kidney with ectopic location and mild rotation, uterine didelphys with transverse vaginal septum and low-type imperforated anus. She was temporarily treated with ultrasound-guided transurethral aspiration of the HMC. Our patient with HMC and PAP was diagnosed with MKS because she has two typical abnormality of MKS and she has no definite complications of retinal disease, learning disability, obesity and renal failure that develop in Bardet-Biedl syndrome, but not in MKS until 33 months of age. Here, we describe a case of a Korean patient with MKS.

• 대한소아치과학회지
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• 제21권2호
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• pp.599-604
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• 1994

Very few reports are available on the occurrence of abnormality of both enamel and dentin. This case has some characteristiced of both amelogenesis imperfecta and dentinogenesis imperfecta. Clinically, the enamel of primary dentition was completely absent and when the permanent teeth came to the pediatric dept. of Dankook University Dental Hopital for treatment. Fixed-removable type resin plate was delivered to increase vertical dimension and to solve esthetic and functional defects. Hypoplastic teeth were restored either stainless steel crown or composite resin restoration or both. The periodic recall check and oral hygiene education are recommended.

• Journal of Korean Neurosurgical Society
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• 제49권4호
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• pp.226-228
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• 2011

We report a case of spontaneous right carotid-cavernous fistula (CCF) in a proximal segment of persistent primitive trigeminal artery (PPTA) and combined vascular anomalies such as left duplicated hypoplastic proximal posterior cerebral arteries and a variation of anterior choroidal artery supplying temporal and occipital lobe. A 45-year-old male presented with progressive right exophthalmos, diplopia, and ocular pain. With manual compression of the internal carotid artery, a cerebral angiography revealed a right CCF from a PPTA. Treatment involved the placement of detachable non-fibered and fibered coils, and use of a hyperglide balloon to protect against coil herniation into the internal carotid artery. A final angiograph revealed complete occlusion of PPTA resulted in no contrast filling of CCF.

• Journal of Chest Surgery
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• 제17권3호
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• pp.448-455
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• 1984

Coarctation of the Aorta is a congenital constriction of aorta of varying degree, usually located at or near the aortic ismuth with frequent associations of other cardiac anomalies. Various modes of surgical corrections, such as resection and end-to-end anastomosis, graft interposition, angioplasty using prosthetic patch or subclavian flap have been used according to the status of coarctation and age of the patient. We have experienced two cases of surgically treated coarctation of the aorta, one of which was preductal coarctation with hypoplastic aortic arch and ventricular septal defect in a 4 year old boy, and the other case was juxtaductal type with aortic regurgitation. Subclavian flap angioplasty with additional pulmonary artery banding procedure was done in the first case and wedge resection with end-to-end anastomosis and aortic valve replacement [St. Jude valve, 23mm] 20 days later of first operation in the other case. The first case developed massive tarry stool on 3rd POD, probably due to mesenteric arteritis with resultant bowl ecrosis, and expired the next day. Recovery was uneventful with the second case.

• Archives of Plastic Surgery
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• 제49권2호
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• pp.253-257
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• 2022

The free fibula flap (FFF) is based on the peroneal artery (PA) system, and it is well known that several anatomical variations of the lower limb vascular system exist, including peronea arteria magna (PAM). PAM is a rare congenital variation in which both anterior tibial artery and posterior tibial artery are either aplastic or hypoplastic, and as a result, PA will be the dominant blood supply to the foot. This variation was described as type III-C in Kim-Lippert's Classification of the Infra-Popliteal Arterial Branching Variations. The awareness of its existence is crucial as it often precludes FFF from being harvested due to the risk of significant limb ischemia and limb loss. Despite some literature reporting donor site complications and impending limb loss following FFF harvest in PAM, preoperative vascular mapping before FFF transfer remains controversial among the microsurgeons. We present a case with an incidental intraoperative finding of PAM that had a successful FFF harvest by luck, without preoperative vascular mapping.

• Journal of Chest Surgery
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• 제9권2호
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• pp.276-286
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• 1976

Coarctation of the aorta is an important congenital cardiovascular defect,. which occurs in a significant number of persons. The basic anatomic defect is a localized deformity of the media, manifested by two types of strictures in the aorta: "true" coarctation and tubular hypoplasia. The zone of coarctation is characteristically located distal to the origin of left subclavian artery at or just beyond the insertion of the ligamentum arteriosum. It shortens life if untreated, but it can be corrected to render the patient functionally normal. Here we have a 2-year operative experiences with 3 cases of the aortic coarctation, two of hypoplastic type and one of postductal type, at age of 17 to 19 year old Korean, operated in 1965 and l968 at the National Medical Center. The purposes of this report are to describe the immediate and late effect of surgery, histopathologic bases and the rarity of this lesions in Korea.

• Journal of Korean Neurosurgical Society
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• 제53권5호
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• pp.293-296
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• 2013

The aneurysm arising from fenestrated proximal anterior cerebral artery (ACA) is considered to be unique. The authors report a case of a 59-year-old woman who presented with a subarachnoid hemorrhage (SAH) secondary to a ruptured aneurysm originating from the fenestrated A1 segment of right ACA. The patient had another unruptured aneurysm which was located at the right middle cerebral artery bifurcation. She was successfully treated with surgical clipping for both aneurysms. From the previously existing literatures, we found 18 more cases (1983-2011) of aneurysms associated with fenestrated A1 segment. All cases represented saccular type of aneurysms, and 79% of the patients had SAH. There were three subtypes of the fenestrated A1 aneurysms depending on the anatomical location, relative to the fenestrated segment. The most common type was the aneurysms located on the proximal end of fenestrated artery (82%). Azygos ACA and hypoplastic A1 were frequently accompanied by the aneurysm (33% and 31%, respectively), and multiple aneurysms were shown in three cases (16%). Considering that fenestrated A1 segment is likely to develop an aneurysm, which has high risk of rupture, early management may benefit patients with aneurysms accompanied by fenestrated proximal ACA.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제11권1호
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• pp.56-59
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• 2008

저자들은 간의 거대 종괴와 심방 중격 결손으로 수술을 받았던 환아에서 복부 CT와 간 조직 검사를 통해 확인된 Abernethy 기형 2형 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Investigative Magnetic Resonance Imaging
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• 제17권1호
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• pp.55-58
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• 2013

전환추동맥은 드물게 보고된 태생기 경-기저동맥문합 잔류동맥의 하나이다. 태생 3주경에 발생하는 경-기저동맥문합은 삼차동맥, 귀동맥, 설하동맥, 그리고 전환추동맥으로 이루어지며, 정상인에서는 태생 6주경 전환추동맥을 마지막으로 모두 퇴화된다. 일반적으로 전환추동맥은 주로 내경동맥에서 기원하며 경추 가로돌기구멍을 통과하지 않는 제1형과, 외경동맥에서 기원하여 첫째 경추 가로돌기구멍을 통과하는 제2형으로 나뉘며, 임상적인 증상을 일으키는 경우는 드물다. 그러나 비교적 잘 동반되는 척추동맥의 무형성을 포함한 두개강내 동맥기형이 있을 수 있으며, 이는 경동맥 내막 절제술 및 외경동맥 색전술 등의 시술시 의의가 있어 심도 있는 이해가 필요하다. 이에 저자들은 자기공명혈관조영술로 진단된 제2형 전환추동맥 증례보고와 문헌고찰을 하고자 한다.

• Journal of Chest Surgery
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• 제17권4호
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• pp.729-734
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• 1984

Bochdalek hernia is a type of congenital diaphragmatic defect in the posterolateral portion of the diaphragm. The defect is usually Lt. sided due to protective effect of liver on right. Sex distribution is male preponderance [2:1] and it is diagnosed during neonate, mostly first 24 hours, due to severe respiratory distress. We experienced a rare case of old aged female patient with congenital Bochdalek hernia on Rt. side which was found incidentally during treatment of spontaneous pneumothorax of Rt. side. 17 year old female patient was admitted to CS department for chest discomfort on right and mild dyspnea with duration of 20 days. Under the diagnosis of spontaneous pneumothorax, Rt. closed thoracostomy and underwater sealed drainage with continuous suction was applied. On follow-up chest x-ray, poorly defined hazy increased density with multiple air-fluid levels in Rt. lower lung field and Lt. subphrenic free air were noted. So, Barium enema was done under the impression of Rt. diaphragmatic hernia, and nearly entire colon proximal to sigmoid was demonstrated in the Rt. hemithorax. Operation was done-for surgical repair of defected diaphragm through Rt. posterolateral thoracotomy. Operative findings were as follows; 1.Hypoplastic Rt. lung, esp. RML & RLL. 2.Nearly entirely intestines were herniated. 3.Diaphragmatic defect was located on posterolateral portion of the diaphragm, about 10x3cm in size with blunt smooth margin. 4.A large bleb on apex of RUL of lung. Herniated intestines were repaired into abdominal cavity manually and defect of diaphragm was repaired with No. I black silk interrupted sutures directly, and bleb was resected. Postoperative courses were uneventful and the patient was discharged with good condition on POD 14th.