The Currarino triad is a unique complex of congenital caudal anomalies, including anorectal malformation, sacral bony defect and presacral mass. This triad may be associated with Mullerian duct anomalies, such as duplication of the vagina and uterus. Each of these diseases has a familial tendency and sometimes coexist within families. But, when coexisting in familial cases, nearly all reported cases revealed mutations of the motor neuron and pancreas homeobox 1 (MNX1) gene. Familial cases of Currarino triad combined with Mullerian duct anomaly without MNX1 gene mutation are very rare. Here we report cases of mother and daughter, who had Currarino triad and Mullerian duct anomaly without MNX1 gene mutation, along with a brief literature review.
Genes, Homeobox ; Humans ; Mothers* ; Motor Neurons ; Mullerian Ducts ; Nuclear Family* ; Pancreas ; Uterus ; Vagina

PURPOSE: The availability of intraoperative parathyroid hormone (IOPTH) monitoring allows successful focused parathyroidectomy for primary hyperparathyroidism (pHPT). The objective of this study was to report our initial experience in IOPTH monitoring during parathyroid surgery for primary hyperparathyroidsim. METHODS: Between May 2011 and February 2013, 37 patients underwent focused parathyroidectomy due to pHPT. IOPTH monitoring based on Miami criteria was used to confirm complete excision of hyperfunctioning parathyroid gland during surgery. Medical records of patients were reviewed retrospectively. RESULTS: Preoperative mean maximal calcium level was 11.7 ± 0.9 mg/dL. Preoperative technetium (99mTc) sestamibi scan and ultrasonography identified 32 of 37 (86.5%) and 29 of 37 (78.4%) of abnormal parathyroid glands, retrospectively. Results of the 2 imaging modalities were discordant for 8 cases (21.6%). The mean pre-excision PTH level was 147.2 ± 201.5 pg/mL. At 5- and 10-minute post tumor resection, PTH levels were 65.3 ± 25.4 pg/mL and 47.5 ± 24.3 pg/mL, respectively. In all cases, IOPTH levels fell by at least 50% after removing all suspected abnormal glands. All patients had a successful return to normocalcemia after surgery (mean follow-up period: 60.2 ± 15.4 months). CONCLUSION: Surgeon could confirm complete excision of abnormal hyperfunctioning parathyroid glands by IOPTH monitoring during surgery for pHPT. IOPTH monitoring can maximize performance of successful focused para thyroidectomy for pHPT, especially when preoperative imaging results are discordant.
Calcium ; Follow-Up Studies ; Humans ; Hyperparathyroidism, Primary ; Medical Records ; Parathyroid Glands ; Parathyroid Hormone ; Parathyroidectomy ; Retrospective Studies ; Technetium ; Thyroidectomy ; Ultrasonography

PURPOSE: Nipple-sparing mastectomy (NSM) has become increasingly popular due to improved cosmesis without compromising oncologic safety. Radial and inframammary incisions are usually used to achieve NSM, with periareolar incisions usually being avoided because of the risk to nipple-areola complex viability. In an attempt to maximize esthetic effects, we performed NSM through periareolar incision with immediate reconstruction. We report our initial experience. METHODS: This case series consisted of all consecutive patients (n = 34) who underwent NSM through a periareolar incision in our institution between August 2017 and December 2018. All patients underwent NSM through periareolar incision followed by immediate reconstruction with an implant or deep inferior epigastric perforator flap. Patient demographics, tumor and treatment characteristics, and short-term postoperative outcomes were reviewed. RESULTS: The mean patient age was 46.74 ± 6.69 years (range, 38–62 years), and the mean operation time was 96.68 ± 28.00 minutes. Indications included in situ cancer in 12 cases and invasive cancer in 22 cases. There was 1 major complication (postoperative hematoma) requiring operative reintervention. No other complications including fistula, implant exposure, or reconstruction failure was observed. At the time of writing, no case of local recurrence has been observed. CONCLUSION Our initial report shows that NSM with immediate reconstruction may successfully be performed through periareolar incision. This method maximizes esthetic effects and may be an appropriate surgical option for NSM.

PURPOSE: Endoscopic thyroidectomy using a cervico-axillary approach (CAA) provides optimal visualization with a smaller dissection plane. Despite the excellent cosmetic results and high patient satisfaction, the surgical and oncologic safety of CAA endoscopic surgery has not been fully established. The present study evaluated the feasibility, safety, and surgical outcomes of CAA endoscopic thyroidectomy. METHODS: From October 2009 to April 2012, 100 patients with papillary thyroid cancer underwent CAA endoscopic thyroidectomy. Patient demographics, pathologic features, and surgical outcomes including complications and recurrence were collected. RESULTS: CAA endoscopic thyroidectomy was successful in all patients, and none required conversion to open thyroidectomy. All patients underwent ipsilateral thyroid lobectomy with or without central compartment neck dissection. The mean tumor size was 1.0±0.6 cm (range, 0.5~1.6), and 35.0% of tumors showed extrathyroidal extension. The mean number of harvested lymph nodes was 4.1±4.4, and metastasis was found in 12.0% of patients. The mean surgical time was 175.2±50.4 min, mean intraoperative blood loss was 42.5±69.2 ml, and the mean hospital stay was 3.3±0.6 days. There were five cases of postoperative transient hypocalcemia and eight cases of vocal cord palsy. No permanent complication or postoperative bleeding was observed. Patients continued to be seen for a median period of 63.7 months, and no recurrence of thyroid cancer was seen. CONCLUSION: CAA endoscopic thyroidectomy is a feasible and safe procedure for low-risk thyroid cancer, with excellent cosmesis. It can be recommended as an alternative option for selected patients with low-risk thyroid cancer.
Demography ; Hemorrhage ; Humans ; Hypocalcemia ; Length of Stay ; Lymph Nodes ; Neck Dissection ; Neoplasm Metastasis ; Operative Time ; Patient Satisfaction ; Recurrence ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy* ; Vocal Cord Paralysis

PURPOSE: A number of researchers have suggested the use of sodium hyaluronate carboxymethyl cellulose (HA-CMC) membrane for preventing postoperative adhesion. This study evaluated the antiadhesive effect and safety of HA-CMC membrane in thyroidectomy for papillary thyroid cancer. METHODS: One hundred sixty-two patients who underwent thyroidectomy were prospectively randomized. In the study group of 80 patients, the 7.5 cm x 13 cm HA-CMC membrane was applied to the operative field after thyroidectomy. The subjects were asked about complications including adhesive symptoms using an 8-item questionnaire at 2 weeks, 3 months, and 6 months after surgery. In addition, items on the appearance of neck wrinkles and scars were evaluated by a physician who had no information about the patient's allocation. RESULTS: There were no significant differences in complications such as swallowing difficulty, and wrinkles between study and control groups. Both groups presented significantly decreased scores over time in swallowing difficulty, and wrinkles. There were no complications regarding the HA-CMC membrane. CONCLUSION: The antiadhesive effect of HA-CMC membrane in thyroid surgery is still uncertain, although it is biologically safe. Further investigation is needed to confirm the antiadhesive effect of HA-CMC membrane in thyroid surgery.
Adhesives ; Carboxymethylcellulose Sodium ; Cellulose* ; Cicatrix ; Deglutition ; Humans ; Hyaluronic Acid ; Membranes* ; Neck ; Prospective Studies ; Surveys and Questionnaires ; Sodium* ; Thyroid Gland* ; Thyroidectomy

We recently established a novel disease entity presented as progressive respiratory failure associated with the inhalation of humidifier disinfectants. In April 2011, we encountered a series of peripartum patients with complaints of respiratory distress of unknown etiology, which was an uncommon phenomenon. Accordingly, we created a multidisciplinary team comprising intensivists, radiologists, pathologists, epidemiologists, and the Korea Centers for Disease Control and Prevention (KCDC). Further, we defined the disease entity and performed a case-control study, epidemiologic investigation, and animal study to determine the etiology. The study findings indicated that the lung injury outbreak was related to the inhalation of humidifier disinfectants and showed that household chemical inhalation can cause severe respiratory failure. Following the withdrawal of humidifier disinfectants from the Korean market in 2012, no such cases were reported. This tragic event is a warning that appropriate safety regulations and monitoring for potential toxic household chemicals are critical to protect public health.
Adult* ; Animals ; Case-Control Studies ; Centers for Disease Control and Prevention (U.S.) ; Disinfectants* ; Family Characteristics ; Humans ; Humidifiers* ; Inhalation* ; Korea ; Lung Injury* ; Lung* ; Peripartum Period ; Public Health ; Respiratory Insufficiency ; Social Control, Formal

PURPOSE: Various methods of sentinel lymph node (SLN) biopsy in thyroid cancer have been introduced. Tc-99m phytate as a radiotracer has been successfully utilized for SLN biopsy in breast, cervix, and endometrial cancer. We assessed the feasibility of SLN dissection using Tc-99m phytate in papillary thyroid carcinoma (PTC). METHODS: Seventeen patients with PTC were prospectively enrolled. Ultrasound-guided peritumoral injection of 55.5 MBq Tc-99m phytate in 0.25-mL normal saline was performed. Preoperative single-photon emission-computed tomography (SPECT) and intraoperative gamma-probe were used for SLN detection during operation. RESULTS: Identification rate of SLNs was 70.6% (12 of 17) with SPECT, and 88.2% (15 of 17) with gamma-probe. Combined SPECT and gamma-probe had identification rates of 88.2% (15 of 17). Identification rates of SLNs in central LN compartments were 82.4% (14 of 17) and 41.2% (7 of 17) in lateral LN compartments. Overall sensitivity, specificity, positive predictive value, and negative predictive value of the results of SLN biopsy were 91.6%, 100%, 88.4%, and 100%, respectively. Eight patients (47.1%) had metastasis in SLNs; all patients had SLN metastasis in the central compartment and 2 patients had SLN metastasis in both the central and lateral compartments. CONCLUSION: Combined SPECT and gamma-probe could detect SLNs with an 88.2% identification rate in PTC. SLN biopsy using Tc-99m phytate is technically feasible. Further investigation is warranted for clinical application of Tc-99m phytate in PTC.
Biopsy ; Breast ; Cervix Uteri ; Endometrial Neoplasms ; Female ; Humans ; Lymph Node Excision* ; Lymph Nodes* ; Neoplasm Metastasis ; Phytic Acid* ; Prospective Studies ; Sensitivity and Specificity ; Sentinel Lymph Node Biopsy ; Thyroid Gland* ; Thyroid Neoplasms* ; Tomography, Emission-Computed, Single-Photon

The Korean Society for Electrolyte and Blood Pressure Research, in collaboration with the Korean Society of Nephrology, has published a clinical practice guideline (CPG) document for hyponatremia treatment. The document is based on an extensive evidence-based review of the diagnosis, evaluation, and treatment of hyponatremia with the multidisciplinary participation of representative experts in hyponatremia with methodologist support for guideline development. This CPG consists of 12 recommendations (two for diagnosis, eight for treatment, and two for special situations) based on eight detailed topics and nine key questions. Each recommendation begins with statements graded by the strength of the recommendations and the quality of the evidence. Each statement is followed by rationale supporting the recommendations. The committee issued conditional recommendations in favor of rapid intermittent bolus administration of hypertonic saline in severe hyponatremia, the use of vasopressin receptor antagonists in heart failure with hypervolemic hyponatremia, and syndrome of inappropriate antidiuresis with moderate to severe hyponatremia, the individualization of desmopressin use, and strong recommendation on the administration of isotonic fluids as maintenance fluid therapy in hospitalized pediatric patients. We hope that this CPG will provide useful recommendations in practice, with the aim of providing clinical support for shared decision-making to improve patient outcomes.

The Korean Society for Electrolyte and Blood Pressure Research, in collaboration with the Korean Society of Nephrology, has published a clinical practice guideline (CPG) document for hyponatremia treatment. The document is based on an extensive evidence-based review of the diagnosis, evaluation, and treatment of hyponatremia with the multidisciplinary participation of representative experts in hyponatremia with methodologist support for guideline development. This CPG consists of 12 recommendations (two for diagnosis, eight for treatment, and two for special situations) based on eight detailed topics and nine key questions. Each recommendation begins with statements graded by the strength of the recommendations and the quality of the evidence. Each statement is followed by rationale supporting the recommendations. The committee issued conditional recommendations in favor of rapid intermittent bolus administration of hypertonic saline in severe hyponatremia, the use of vasopressin receptor antagonists in heart failure with hypervolemic hyponatremia, and syndrome of inappropriate antidiuresis with moderate to severe hyponatremia, the individualization of desmopressin use, and strong recommendation on the administration of isotonic fluids as maintenance fluid therapy in hospitalized pediatric patients. We hope that this CPG will provide useful recommendations in practice, with the aim of providing clinical support for shared decision-making to improve patient outcomes.

Background: Postoperative thyroid stimulating hormone (TSH) suppression therapy is recommended for patients with intermediate- and high-risk differentiated thyroid cancer to prevent the recurrence of thyroid cancer. With the recent increase in small thyroid cancer cases, the extent of resection during surgery has generally decreased. Therefore, questions have been raised about the efficacy and long-term side effects of TSH suppression therapy in patients who have undergone a lobectomy. Methods: This is a multicenter, prospective, randomized, controlled clinical trial in which 2,986 patients with papillary thyroid cancer are randomized into a high-TSH group (intervention) and a low-TSH group (control) after having undergone a lobectomy. The principle of treatment includes a TSH-lowering regimen aimed at TSH levels between 0.3 and 1.99 μIU/mL in the low-TSH group. The high-TSH group targets TSH levels between 2.0 and 7.99 μIU/mL. The dose of levothyroxine will be adjusted at each visit to maintain the target TSH level. The primary outcome is recurrence-free survival, as assessed by neck ultrasound every 6 to 12 months. Secondary endpoints include disease-free survival, overall survival, success rate in reaching the TSH target range, the proportion of patients with major cardiovascular diseases or bone metabolic disease, the quality of life, and medical costs. The follow-up period is 5 years. Conclusion The results of this trial will contribute to establishing the optimal indication for TSH suppression therapy in low-risk papillary thyroid cancer patients by evaluating the benefit and harm of lowering TSH levels in terms of recurrence, metabolic complications, costs, and quality of life.