• Archives of Plastic Surgery
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• 제45권6호
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• pp.578-582
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• 2018

Teratomas can occur in almost any region of the body and are the most common extragonadal germ cell childhood tumors. However, craniofacial teratomas are rare. Craniofacial teratomas can present unique features and cause significant functional and aesthetic concerns. There are complex lesions that can have components intra-cranially and extra-cranially. Therefore, their management requires significant multi-stage multidisciplinary surgical procedures. Herein, we present a case of craniofacial teratoma in a child with the phenotype of proboscis lateralis that highlights some of the pertinent point of the diagnosis and management of congenital neonatal teratomas.

• Advances in pediatric surgery
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• 제6권2호
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• pp.160-165
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• 2000

저자들은 확정되지 않은 악성의 가능성을 가진 부신 피질 방추세포 종양 (adrenal cortical spindle cell neoplasm of indeterminate malignant potential)을 동반한 Beckwith-Wiedemann 증후군을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• 제27권5호
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• pp.386-389
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• 1994

신생아에서 심실중격결손을 동반하지 않은 대혈관전위증 (Transposition of th egreat arteries with intact ventricular septum)의 해부학적교정은 저체중등의 외과적 위험인자에도 불구하고 심방내 교정보다 생리적이다는 점에서 선호되고 있고 조기수술성적이 향상되었다고는 하나 아직도 국내사망율은 높은 편이다. 저자들은 최근 3.5Kg(생후 19일), 3.6 Kg(생후 16일)된 신생아에서 Jatene operation 2례를 치험하였기에 보고하는 바이다.

• Archives of Plastic Surgery
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• 제48권6호
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• pp.641-645
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• 2021

A 3,480 g male neonate showed tachypnea symptom with a serum ionized calcium level of 0.66 mmol/L by routine clinical analysis. He was injected calcium gluconate intravenously through femoral vein catheter to treat the hypocalcemia. On second day after the injection, he started to show erythema in the flank area. The lesion became firm and changed into whitish crust consist of small crystals. Abdominal X-ray and ultrasonography showed the accumulation of calcium deposit in the subcutaneous layer of the lesion. Surgical debridement was performed to remove the crust with calcium deposit and acellular fish skin graft rich in omega-3 (Kerecis) was applied to the defect site for secondary intention of the defect wound. After 2 months, the skin and soft tissue defect were fully covered with healthy normal skin without depression or contracture. This report is a first case of iatrogenic calcinosis cutis without extravasation symptom.

• Neonatal Medicine
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• 제25권3호
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• pp.131-135
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• 2018

Hyponatremia is defined as a plasma sodium concentration of <135 mEq/L. It is a common electrolyte imbalance in newborns. We report the case of a term neonate with cleft lip, cleft palate, imperforate anus, normal male karyotype, and chronic hyponatremia. On the 4th day of life, he showed hyponatremia (plasma sodium concentration 130 mEq/L) with low serum osmolality (275 mOsm/kg), high urine sodium (116.7 mEq/L), and high urine osmolality (412 mOsm/kg). His thyroid and adrenal functions were normal. Despite intravenous and oral sodium supplementation and hydrocortisone treatment, hyponatremia persisted. Brain magnetic resonance imaging showed normal results. He was diagnosed as having reset osmostat, a rare subtype of the syndrome of inappropriate secretion of antidiuretic hormone characterized by a subnormal threshold for antidiuretic hormone secretion, with hypotonic hyponatremia.

• Advances in pediatric surgery
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• 제20권2호
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• pp.53-57
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• 2014

Purpose: Neonatal neuroblastoma (NBL) is the most common malignant tumor in neonates, but there have been few studies about it. The purpose of this study was to investigate the clinical features of NBL and to compare prenatal and postnatal diagnosed groups. Methods: Nineteen patients who were diagnosed with NBL prenatally or within 28 days after birth from February 1986 to February 2013 in Seoul National University Hospital were enrolled in the study. The patients were categorized according to the International Neuroblastoma Staging System (INSS) and Children's Oncology Group (COG). Retrospective medical-record reviews were performed on these patients. The operative date, complication, pathological stage, and overall survival of the prenatally diagnosed group and the postpartum diagnosed group were compared. Results: Tumor was detected via prenatal ultrasonography in 8 patients (42.1%), and 11 patients (57.9%) were diagnosed within 28 days after birth. Based on INSS, the patients were divided into the stage I (n=8), stage II (n=1), stage III (n=3), stage IV (n=4), and stage IVs (n=3) groups, respectively. Based on COG, on the other hand, the patients were divided into the low-risk (n=8), intermediate-risk (n=8), and high-risk (n=3) groups. The postoperative complication rate was 29%. One patient died from complications from chemotherapy. The other 18 patients' mean follow-up period was 77.7 months. The differences between the postoperative complication rate, proportion of early-stage tumor, and overall survival of the prenatal and postnatal groups were not statistically significant (p=0.446, p=0.607, p=0.414). Conclusion: NBL showed favorable outcomes but relatively higher postoperative complications. There seem to be no significant statistical differences in the postoperative complications, proportion of early-stage tumor, and overall survival between the prenatally diagnosed group and the postpartum diagnosed group.

• Clinical and Experimental Pediatrics
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• 제53권6호
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• pp.705-710
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• 2010

Purpose: To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit. Methods: A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007. Results: The rate of prenatal diagnosis was 12%. The average gestational age and birth weight were $37^{+2}$ weeks and $2.5{\pm}0.7kg$, respectively. Thirty-one infants were born prematurely (32%). Type C was the most common. The mean gap between the proximal and distal esophagus was 2 cm. Esophago-esophagostomy was performed in 72 patients at a mean age of 4 days after birth; gastrostomy or duodenostomy were performed in 8 patients. Forty patients exhibited vertebral, anorectal, cardiac, tracheoesophageal, renal, limb (VACTERL) association with at least 2 combined anomalies, and cardiac anomaly was the most common. The most common post-operative complications were esophageal stricture followed by gastroesophageal reflux. Balloon dilatation was performed for 1.3 times in 26 patients at a mean age of 3 months. The mortality and morbidity rates were 24% and 67%, respectively, and the most common cause of death was sepsis. The weight of approximately 40% patients was below the 10th percentile at 2 years of age. Conclusion: Mortality and morbidity rates of patients with TEF and EA are high as compared to those of infants with other neonatal surgical diseases. Further efforts must be taken to reduce mortality and morbidity and improve growth retardation.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제7권2호
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• pp.179-185
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• 2004

목적: 대구 부산 지역에서 신생아 시기에 수술이 시행된 소화기 질환의 최근 현황과 이와 관련된 임상적 소견들을 살펴보고 현재 소아 소화기 질환의 술 후 합병증과 사망률 등의 치료성과에 대해 살펴보았다. 방법: 1996년 1월부터 2003년 7월까지 부산대학교, 경북대학교, 영남대학교 및 대구가톨릭대학교 병원에서 생후 1개월 이내에 소화기 질환으로 수술을 시행받은 202명을 대상으로 하여 임상적 소견을 분석하였다. 결과: 1) 항문 직장 기형이 48명(23.8%), 선천성 중장 폐쇄가 27명(13.4%), 비후성 유문 협착증이 27명(13.4%), 선천성 거대 결장이 22명(10.9%), 십이지장 폐쇄가 15명(7.4%) 등의 빈도순을 보였다. 2) 남녀비는 2.8 : 1로 남아가 우세하였고, 비후성 유문 협착증과 장 회전 이상에서는 남아가 10배 이상 많았다. 3) 동반 기형의 빈도는 선천성 심장 기형, 잠복 고환, 수신증 등의 순서로 많았으며, 제대 기저부 탈장에서는 50%에서 동반 기형이 있었다. 4) 20명(9.9%)의 환아가 산전에 진단되었는데, 선천성 장폐쇄, 십이지장 폐쇄, 복벽 개열증, 제대 기저부 탈장과 같이 장 내 공기 패턴이 특이 소견을 보이는 경우였다. 5) 평균 입원 기간은 22.8일이었고, 신생아 괴사성 장염이 44.7일로 가장 길었다. 6) 술 후 합병증은 주로 창상 감염 또는 문합부 누출 등 사소한 합병증으로 18례(8.9%)에서 발생하였다. 7) 사망률은 횡격막 탈장증(37.5%), 식도 폐쇄(28.6%), 제대 기저부 탈장(20.0%) 순으로 많았으며, 12명이 사망하여 5.9%의 사망률을 보였다. 결론: 대구 부산 지역에서 신생아 시기에 소화기 질환으로 수술을 받았던 환자들의 질병 종류가 다양해졌고, 술 후 합병증과 사망률이 현저히 감소함을 알 수 있었으며, 이것은 관련 분야의 발전에 기인한 것으로 판단된다.

• Advances in pediatric surgery
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• 제14권2호
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• pp.173-177
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• 2008

Sialoblastoma is a rare tumor of salivary gland origin, developing in the fetal or neonatal period. Most tumors arise in parotid gland, and rarely in submandibular gland. Because of its rare incidence, diagnosis of the sialoblastoma in head and neck tumors of children is not easy. The case of a congenital submandibular gland sialoblastoma is presented. A neonate was transferred right after birth due to a submandibular mass. CT scan showed a lobulating mass located posterior to the left submandibular gland, suggesting neurogenic tumor or myofibroma. The tumor was excised easily after division of a duct-like structure connecting with the submandibular gland. The microscopic findings showed the basaloid cells and ductules forming cellular nests, separated by thin fibrous tissue. Immunohistochemical staining was positive for cytokeratin, vimentin and Ki-67, which was consistent with sialoblastoma.

• Advances in pediatric surgery
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• 제8권2호
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• pp.156-160
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• 2002

When jaundice persists for more than 14 days postnatally, the early diagnosis of surgical jaundice is important for the prognosis in extrahepatic biliary atresia after draining procedure. The role of diagnostic laparoscopy to differenctiate medical causes of jaundice from biliary atresia is evaluated in this report. Four patients with prolonged jaundice have been included in this study. When the gallbladder was not visualized we proceeded to laparotomy. In patients with enlarged gallbladder visualized at laparoscopy, laparoscopic guided cholangiogram was performed, and laparoscopic liver biopsy was done for those who had a patent biliary tree. Two patients had small atretic gallbladder and underwent a Kasai hepato-portoenterostomy. One patients showed a patent gallbladder and common bile duct with atresia of the common hepatic and intrahepatic ducts, and they underwent a Kasai hepatic-portoenterostomy. One patient showed an enlarged gallbladder and laparoscopic-guided cholangiogram were normal. Laparoscopic liver biopsy was performed. There were no complications. Laparoscopy with laparoscopic-guided cholangiogram may be a valuable method in accurate and earlier diagnosis in an infant with prolonged jaundice.