• Archives of Plastic Surgery
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• v.33 no.2
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• pp.237-240
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• 2006

The simultaneous correction of the hypertelorism and exophthalmos combined with craniosynostosis is very rarely performed operative procedures in the world. The craniosynostosis is the congenital anomaly that designates premature fusion of one or more sutures in either cranial vault or cranial base. Hypertelorism is not a distinct clinical syndrome in itself, but is a physical finding secondary to facial and cranial maldevelopment and it is defined as a increase in the distance between the medial orbital walls. Exophthalmos can occur following the decrease in the size of the orbit in patients with developmental skeletal disorders such as craniofacial synostosis. The authors experienced 9-year-old male patient, who has complex cranio-facial abnormality. The craniosynostosis was oxycephaly type and primary fronto-orbital advancement surgery had been performed in other hospital. The abnormal cranial vault combined with hypertelorism and exophthalmos due to maldeveloped both orbital walls. Surgical correction was obtained by various cranio-fronto-orbital remodeling technique such as calvarial bone craniotomy, fronto-orbital advancement, paramedian resection, medial canthopexy, Tessier-Wolfe three wall orbital expansions. We achieved a quite satisfactory result both functionally and aesthetically in a complex cranio-facial deformity patient by combination and modification of previously developed various cranio-facial plasty technique and hereby report the case with brief discussion and review of literature.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.222-225
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• 2005

Meckel's diverticulum is generally acknowledged to be the most prevalent congenital anomaly of the gastrointestinal tract. The preoperative diagnosis of Meckel's diverticulum is difficult, especially in neonates, because of the lesions ability to masquerade as one of a variety of much more common abdominal pathologies. Recently we experienced a case of perforated Meckel's diverticulum with mild inflammatory reaction, intestinal adhesion, and small bowel obstruction in a previous healthy 9-day-old neonate. The spontaneous perforation of Meckel's diverticulum in neonate is very rare but serious entity. The course was rapid and progressed to sepsis. So despite its varied presentation, Meckel's diverticulum should be kept in mind as a cause of acute abdomen in neonates.

• Journal of Chest Surgery
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• v.37 no.10
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• pp.856-860
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• 2004

Interrupted aortic arch with concomitant intracardiac defects is a rare congenital anomaly that has an unfavorable natural course. We report a successful staged operation of interrupted aortic arch with apical muscular ventricular septal defect associating esophageal atresia with tracheoesophageal fistula in a 3-day-old neonate weighing 2.6 kg. We repaired esophageal atresia through the right thoracotomy and subsequently performed extended end-to-end anastomosis of the aortic arch with pulmonary artery banding through the left thoracotomy at same operation. The apical muscular VSD was repaired 87 day after first operation. The patient required multiple additional interventions before closure of the apical muscular ventricular septal defect, such as pyloromyotomy for idiopathic hypertrophic pyloric stenosis, anterior aortopexy for airway obstruction, and balloon aortoplasty for residual coarctation. She is now doing well.

• Journal of Chest Surgery
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• v.41 no.4
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• pp.512-515
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• 2008

An anomalous systemic arterial supply to the left basal segments without sequestration is a rare congenital abnormality within the spectrum of pulmonary sequestration. But this is rather different from the definition of pulmonary sequestration in that it has normal bronchial connections. We describe here our experience with surgical treatments for an anomalous systemic arterial supply to the left basal segments without sequestration, and this condition was confirmed preoperatively.

• Advances in pediatric surgery
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• v.17 no.1
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• pp.35-44
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• 2011

Thyroglossal duct cysts (TGDC) are the most common type of congenital developmental anomaly encountered in the anterior midline of the neck in childhood. The aim of the study was to evaluate the clinical characteristics of TGDC and identify any factors that could be related to recurrence after surgery. This study consisted of a retrospective chart review of 45 patients treated at Kyungpook National University Hospital for TGDC between 1990 and 2008. All records were reviewed for age and sex, length of history, presentation, diagnostic methods, sizes and locations of cyst, surgical management, histopathology of the lesion and recurrences. The statistical analysis of risk factors for recurrence was made using the Fisher's exact test with a significance level of p (0.05. The male to female ratio was 2.2:1 with a male preponderance. The mean age at operation was 5 years and 2 months (4 months - 17 years). The most common presenting symptom was a nontender cervical mass (78 %). Most TGDC were found in the midline position. Twenty four were infrahyold, 17 were hyoid, and 4 were suprahyoid level. Forty one (91 %) patients received the Sistrunk operation, and 4(9 %) patients received Cyst excision. Postoperative a seroma developed in six patients in the early postoperative days. There were a total of 3(6.6 %) recurrences, 2 in patients who had excision only and in one patient who had the Sistrunk operation. Univariate analysis for risk factors with recurrence showed that there was no statistical relationship between the presence of preoperative infection and the development of recurrence. The removal of hyoid bone along with TGDC was a statistically significant risk factor for recurrent disease. This study suggests that the Sistrunk operation Is the treatment of choice for TGDC in order to reduce recurrence.

• The Korean Journal of Nuclear Medicine
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• v.14 no.1
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• pp.37-44
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• 1980

The usefulness of single, random measurements of serum FSH and LH in the diagnosis of primary amenorrhea by radioimmunoassay was investigated. The 16 patients were divided into 3 groups by the level of serum FSH and LH. The first group with increased level of serum FSH and LH is five patients, all of these are related to the acquired or congenital abnormality of the ovary. Further studies indicated include buccal smear, chromosome analysis, gynecography and laparosocopy. The second group with normal serum FSH and LH is nine patients, four patients of these are related to the developmental anomaly of the Mullerian duct and five patients are undo etermined origin. Further studies indicated include laparoscopy and gynecography. The third group with decreased serum FSH and normal or decreased serum LH is two patients, one of these is related to the pituitary function, isolated FSH deficiency, the other is undetermined origin. Further studies indicated include the pituitary function test, LH-RH stimulation test, skull radiography. Determination of serum FSH and LH levels does not permit a specific etiologic diagnosis of primary amenorrhea. However the serum levels of FSH and LH can be used to differentiate the principal area of the investigation and can be of assistance in choosing more specific testing procedures.

• Journal of Yeungnam Medical Science
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• v.21 no.1
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• pp.67-73
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• 2004

Background: A ureteropelvic junction (UPJ) obstruction is a congenital anomaly commonly afflicting the pediatric population. However, it occurs more frequently in adults than is generally appreciated. To assess their characteristics, we have here compared and analyzed the clinical manifestations, causes, and outcomes of UPJ obstruction found in children and adults. Materials and Methods: 102 patients (118 renal units) out of 182 patients (218 renal units), with diagnosed UPJ obstruction, were available for follow up. The follow-ups were retrospectively reviewed to determine the presenting symptoms, treatments, and clinical outcomes. Patients who were younger than 18 years of age were assigned to the pediatric group (44 patients), others the adult group (58 patents). Results: The majority of the patients presented with flank pain (pediatric group: 68%, adult group: 76%). The majority of patients underwent a pyeloplasty (pediatric group: 50.9%, adult group: 62.3%). And the major cause of the UPJ obstruction was an intrinsic stenosis (pediatric group: 53%, adult group: 56%). Conclusion: The prevalence of bilateral UPJ obstruction in adults is evidently less prevalent than in pediatrics, and despite the late diagnosis, the surgical outcome is similar. A further investigation of UPJ obstruction will lead us to a more comprehensive understanding of the disease.

• Journal of Yeungnam Medical Science
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• v.32 no.2
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• pp.118-121
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• 2015

Cardiogenic embolic stroke accounts for approximately 20% of ischemic strokes and the likelihood of its recurrence is high. Paradoxical embolism may be an important cause of cardioembolic stroke, which can be evaluated through multiple diagnostic modalities including transesophageal echocardiography (TTE) or transcranial Doppler. A persistent left superior vena cava (PLSVC) is a rare congenital vascular anomaly, which mainly drains to the right atrium via the coronary sinus. Although rare, PLSVC draining into the left heart predisposes the patient to paradoxical embolism through a right-to-left shunt. We report on a 78-year-old female patient with an ischemic stroke associated with PLSVC draining into the left atrium through the pulmonary vein, which was investigated via TTE with an agitated saline test and computed tomography.

• Clinical and Experimental Pediatrics
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• v.60 no.11
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• pp.353-358
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• 2017

Purpose: To develop and evaluate a simple screening tool to assess hearing loss in newborns. A derived score was compared with the standard clinical practice tool. Methods: This cohort study was designed to screen the hearing of newborns using transiently evoked otoacoustic emission and auditory brain stem response, and to determine the risk factors associated with hearing loss of newborns in 3 tertiary hospitals in Northern Thailand. Data were prospectively collected from November 1, 2010 to May 31, 2012. To develop the risk score, clinical-risk indicators were measured by Poisson risk regression. The regression coefficients were transformed into item scores dividing each regression-coefficient with the smallest coefficient in the model, rounding the number to its nearest integer, and adding up to a total score. Results: Five clinical risk factors (Craniofacial anomaly, Ototoxicity, Birth weight, family history [Relative] of congenital sensorineural hearing loss, and Apgar score) were included in our COBRA score. The screening tool detected, by area under the receiver operating characteristic curve, more than 80% of existing hearing loss. The positive-likelihood ratio of hearing loss in patients with scores of 4, 6, and 8 were 25.21 (95% confidence interval [CI], 14.69-43.26), 58.52 (95% CI, 36.26-94.44), and 51.56 (95% CI, 33.74-78.82), respectively. This result was similar to the standard tool (The Joint Committee on Infant Hearing) of 26.72 (95% CI, 20.59-34.66). Conclusion: A simple screening tool of five predictors provides good prediction indices for newborn hearing loss, which may motivate parents to bring children for further appropriate testing and investigations.

• Archives of Plastic Surgery
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• v.34 no.3
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• pp.346-351
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• 2007

Purpose: As a rare congenital anomaly, Poland's syndrome has been known to show hypoplasia in breast and nipple, absence of pectoralis major muscle, and aplasia or deformity of rib or costal cartilage which has been reported to be more common in male. However, most patients who are seeking operation are female patients having one-side deformity. In the field of plastic surgery, the major surgical indications could be asymmetric chest wall depression in man or breast hypoplasia in woman. There are many reconstruction options according to the degree of patient's deformity: a prosthetic implant, breast implant with or without tissue expander, latissimus dorsi musculocutaneous pedicled flap with or without implant and/or tissue expander, and free tissue transfer with or without tissue expander. Methods: The authors have treated 4 patients(2 male, 2 female) who had a diagnosis of Poland's syndrome. According to the degree of patient's deformity, all patients underwent correction of breast asymmetry and unilateral anterior thoracic hypoplasia with one-staged or two-staged reconstruction. Results: All patents were satisfied with the results and there occurred no specific complications. Conclusion: The authors propose the treatment plan for patient with Poland's syndrome, according to the degree of patient's deformity. In case of male patient with mild deformity, the prosthetic implant or latissimus dorsi musculocutaneous pedicled flap will simulate the missing pectoralis and improve the contour deformity. In case of female patient with moderate to severe breast asymmetry and upward displaced nipple areolar complex (NAC), NAC can be lowered with tissue expander, breast can be enlarged with autologous free flaps or latissimus dorsi musculocutaneous pedicled flap with implant.