Robot-Assisted Laparoscopic Adenomyomectomy for Patients Who Want to Preserve Fertility

An adenomyomectomy is a conservative-surgical option for preserving fertility. Conventional laparoscopic adenomyomectomies present difficulties in adenomyoma removal and suturing of the remaining myometrium. Robot-assisted laparoscopic surgery could overcome the limitations of conventional laparoscopic surgery. Four patients with severe secondary dysmenorrhea and pelvic pain visited Seoul St. Mary's Hospital and were diagnosed with adenomyosis by pelvic ultrasonography and pelvic magnetic resonance imaging (MRI). The four patients were unmarried, nulliparous women, who desired a fertility-preserving treatment. We performed robot-assisted laparoscopic adenomyomectomies. The dysmenorrhea and pelvic pain of the patients nearly disappeared after surgery. No residual adenomyosis was observed on the follow-up pelvic MRI. A robot-assisted laparoscopic adenomyomectomy was feasible, and could be a minimally invasive surgical option for fertility-sparing treatment in patients with adenomyosis.

The Recent Review of the Genitourinary Syndrome of Menopause

The genitourinary syndrome of menopause (GSM) is a new term that describes various menopausal symptoms and signs including not only genital symptoms (dryness, burning, and irritation), and sexual symptoms (lack of lubrication, discomfort or pain, and impaired function, but also urinary symptoms (urgency, dysuria, and recurrent urinary tract infections). The terms vulvovaginal atrophy and atrophic vaginitis, which were generally used until recently, had a limitation because they did not cover the full spectrum of symptoms and did not imply that the symptoms are related to a decreased estrogen level in menopause. Since the GSM may have a profound negative impact on the quality of life of postmenopausal women, women should be made aware of these problems and treated with an appropriate effective therapy. Thus, in this review we introduce new terminology and discuss the importance of comprehension of GSM and the necessity of active treatment of this syndrome in postmenopausal women.

Cardiac Resynchronization Therapy in Infant with Dilated Cardiomyopathy during Extracorporeal Membrane Oxygenator

Although heart transplantation is a final therapeutic option in pediatric patients with dilated cardiomyopathy (DCMP), the shortage of pediatric heart donors is a major obstacle. In adults with DCMP characterized by cardiac dyssynchrony, cardiac resynchronization therapy (CRT) is known to be an effective treatment option. However, there is a lack of evidence on the effectiveness of CRT in infants with DCMP. Several studies have reported improvement in hemodynamics and cardiac performance following CRT in infants with DCMP. Here, we report CRT in an infant with DCMP during extracorporeal membrane oxygenation with 5 months of follow-up.

Small Left Atrial Size Complicating Percutaneous Transcatheter Device Closure of Secundum Atrial Septal Defect with Conventional Approach

BACKGROUND AND OBJECTIVES: Transcatheter device closure becomes the first option for treating secundum atrial septal defect (ASD), but the conventional method is sometimes unsuccessful even when the defect size indicates the closure to be feasible. To increase the success rate, modified methods have been introduced and used. This study aimed to find predictors for using the modified methods in the device closure of secundum ASDs. SUBJECTS AND METHODS: Between October 2010 and December 2012, 92 patients with ASDs underwent the transcatheter device closure. We analyzed the sizes of the defect, the surrounding rims, and the ratios of the left atrium (LA) dimensions to the device size in the patients who underwent the procedure either using the conventional or modified methods. RESULTS: Among the 88 successful cases (95.7%), 22 patients (25%) required modified methods (12 using pulmonary vein and 10 using balloon). The modified method group had the larger size of ASDs and smaller posterosuperior rim. The mean ratios of the LA anteroposterior diameter, width, and length to the device size were all significantly smaller in the modified methods group than in the conventional group (1.20 vs. 1.56, 1.32 vs. 1.71, and 1.61 vs. 2.07, respectively). We found that the risk factors for the modified methods were smaller retroaortic rim, larger ASD, and smaller LA dimension/device size. CONCLUSION: In addition to larger defects and smaller retroaortic rim, the smaller ratios of the LA dimensions to the device size influenced the need for the application of modified methods in the transcatheter device closure of ASDs.

Diagnostic characteristics of supplemental laboratory criteria for incomplete Kawasaki disease in children with complete Kawasaki disease / 소아과

PURPOSE: In 2004, the American Heart Association (AHA) had published an algorithm for the diagnosis of incomplete Kawasaki disease (KD). The aim of the present study was to investigate characteristics of supplemental laboratory criteria in this algorithm. METHODS: We retrospectively examined the medical records of 355 patients with KD who were treated with intravenous immunoglobulin (IVIG) during the acute phase of the disease. Laboratory data were obtained before the initial IVIG administration and up to 10 days after fever onset. In 106 patients, laboratory testing was performed more than twice. RESULTS: The AHA supplemental laboratory criteria were fulfilled in 90 patients (25.4%), and the frequency of laboratory examination (odds ratio [OR], 1.981; 95% confidence interval [CI], 1.391-2.821; P<0.001) was a significant predictor of it. The fulfillment of AHA supplemental laboratory criteria was significantly associated with refractoriness to the initial IVIG administration (OR, 2.388; 95% CI, 1.182-4.826; P=0.013) and dilatation of coronary arteries (OR, 2.776; 95% CI, 1.519-5.074; P=0.001). CONCLUSION: Repeated laboratory testing increased the rate of fulfillment of the AHA supplemental laboratory criteria in children with KD.

Three cases of complications after high-intensity focused ultrasound treatment in unmarried women

High-intensity focused ultrasound (HIFU) has been regarded as a non-surgical, minimally invasive therapeutic option for patients who prioritize uterus-conservation. Although many studies have shown that HIFU therapy is a safe and effective treatment of uterine fibroid, not all fibroids are suitable for HIFU due to risks of serious complications. We experienced three cases of complications after the HIFU ablation for huge uterine fibroids, including two cases of rapid myoma enlargement and one case of heavy vaginal bleeding.

Erratum to: Small Left Atrial Size Complicating Percutaneous Transcatheter Device Closure of Secundum Atrial Septal Defect with Conventional Approach

In this article, on page 218, Fig. 2B and D have errors.

Segmental Analysis of Right Ventricular Longitudinal Deformation in Children before and after Percutaneous Closure of Atrial Septal Defect

BACKGROUND: The aim of study is to identify the dependence of right ventricular (RV) free wall longitudinal deformation on ventricular loading through segmental approach in relatively large number of patients with atrial septal defect (ASD). METHODS: Patients with ASD (n = 114) and age matched healthy children (n = 60) were echocardiographically examined the day before percutaneous device closure and within 24 hours afterwards. RV free wall deformation parameters, strain (small je, Ukrainian) and strain rate (SR), were analyzed in the apical (small je, Ukrainian(A), SR(A)) and basal (small je, Ukrainian(B), SR(B)) segments. Measured deformation parameters were adjusted for RV size (small je, Ukrainian(AL), SR(AL), small je, Ukrainian(BL), SR(BL)) by multiplying by body surface area indexed RV longitudinal dimension. Regression analyses determined the relationships of these deformation parameters with RV loading parameters that were measured by catheterization. RESULTS: small je, Ukrainian(BL) and SR(BL) were not different between pre-closure patients and controls (p = 0.245, p = 0.866), and were decreased post-closure (p = 0.001, p = 0.018). Post-closure small je, Ukrainian(BL) was lower than in controls (p = 0.001). Pre-closure small je, Ukrainian(AL) and SR(AL) were higher than in controls (p = 0.001, p < 0.001), but decreased after closure (all p < 0.001). The pulmonary to systemic flow ratio was related to procedural differences of small je, Ukrainian(BL) (p = 0.017) and of SR(BL) (p = 0.019). RV end diastolic pressure was negatively related to post-closure small je, Ukrainian(BL) (p = 0.020) and post-closure SR(BL) (p = 0.012), and the procedural SR(BL) difference (p = 0.027). CONCLUSION: The longitudinal deformation of the RV basal segment is dependent and its remodeling is also dependent on volume loading in children with ASD.

A Case of 17q22 with Interstitial Deletion

Cases of interstitial deletions of the long arm of chromosome 17 are very rare, with only nine cases ever reported worldwide. We describe a 12-year-old boy with profound developmental delay, microcephaly, facial dysmorphism, contracture of the large joints and bilateral hearing loss. A chromosomal study using a peripheral blood sampled revealed 46,XY,del(17)(q22q23). To our knowledge, he is the first case of interstitial deletion of the long arm of chromosome 17 ever reported in Korea.

Clinical Manifestations of Leukodystrophies: A Single Center Study / 대한소아신경학회지

PURPOSE: Leukodystrophies have been defined as inherited metabolic disorders of myelin resulting in abnormal development or progressive destruction of the white matter. This study was performed to investigate the clinical manifestations and treatments of leukodystrophies in a single Korean tertiary center. METHODS: We retrospectively analysed the medical records of patients who had been diagnosed with leukodystrophy from May 1995 to May 2010 at the Asan Medical Center. RESULTS: During the 15-year study period, 36 cases of leukodystrophies were diagnosed with an verage age at symptom presentation of 49 months. Prominent symptoms at presentation were developmental delay (41%) and seizure (25%); however, nystagmus, developmental regression, hearing loss, gait disturbance, visual disturbance, attention deficit, hypotonia, hyperpigmentation, and hemiparesis were also observed. On MRI, periventricular involvement was noted frequently. The most common diagnoses were adrenoleukodystophy (25%), metachromatic leukodystrophy (11%), Krabbe disease (11%), and Pelizaeus-Merzbacher disease (8.3%). No final diagnosis was made in 14 cases (41%). Bone marrow transplantation was performed in 4 patients and showed favorable prognoses. CONCLUSION: Clinical features of leukodystrophies are not specific to diagnosis and most leukodystrophies remain undiagnosed; however, a logical algorithm based on prevalence could aid the laboratory testing. Because early detection and diagnosis is crucial for treatment and prognosis, it is important to have a high index of suspicion and watchful screening of familial history.