Uterine lymphangioleiomyomatosis in a premenopausal woman with tuberous sclerosis: A case report.

Lymphangioleiomyomatosis is a rare disease characterized by abnormal smooth muscle cell growth. It primarily occurs in the lungs but can also rarely occur in other organs, in which case it is classified as extrapulmonary lymphangioleiomyomatosis. It often accompanies tuberous sclerosis complex. This report concerns a case of uterine lymphangioleiomyomatosis with spontaneous uterine rupture in a young woman with tuberous sclerosis complex. A 27-year-old nulligravida patient presented to the emergency room with vaginal bleeding. She had a history of clinical diagnosis of tuberous sclerosis complex and pulmonary lymphangioleiomyomatosis. Initially, abdominopelvic computed tomography and magnetic resonance imaging suggested a hemorrhagic necrosis and rupture of degenerated uterine myoma. She underwent emergency exploratory laparotomy. The right side of her normal-sized uterus were ruptured without any specific mass. Active bleeding and hematoma from the ruptured uterus and partially ruptured right ovary were noted. The procedure included total hysterectomy and right salpingo-oophorectomy. Pathological analysis confirmed lymphangioleiomyomatosis in the uterine serosa and myometrium. Lymphangioleiomyomatosis mainly occurs in women of reproductive age and worsens with estrogen. Early diagnosis and careful follow-up are necessary due to the risk of worsening gynecological symptoms or even uterine rupture during pregnancy. This case enhances our understanding of extrapulmonary lymphangioleiomyomatosis and highlights the importance of comprehensive evaluation in complex clinical scenarios.

Surgical technique of two-step pelvic and para-aortic sentinel lymph node mapping in early-stage endometrial cancer: laparoscopic, robotic, and open method.

Since sentinel lymph node mapping in endometrial cancer is becoming more widely used, the need of standardizing surgical technique is growing [1, 2]. The objective of this surgical video is to describe the procedure of two-step pelvic and para-aortic sentinel lymph node mapping using indocyanine green and fluorescent camera in endometrial cancer, in three versions of surgical modality of laparoscopic, robotic, and open laparotomy. The patients in the surgical video are diagnosed with biopsy-proven endometrial cancer in its early stage determined by the preoperative imaging study. After collecting washing cytology, bilateral salpinges were clamped with Endo Clip™ to minimize tumor spillage. Gauze packing in posterior cul-de-sac was done to minimize the spillage of indocyanine green dye during paraaortic sentinel lymph node mapping. Indocyanine green dye was injected in bilateral uterine fundus, to detect isolated paraaortic sentinel lymph node pathway. After bilateral paraaortic sentinel lymph node was sampled, cervical injection of Indocyanine green dye was done in 3 o'clock and 9 o'clock directions, both superficially and deeply, 2 mL in each side. After dissecting off the obliterated umbilical ligament, para-vesical and para-rectal spaces were developed. The ureter, uterine artery, and internal and external iliac vessels were identified before bilateral pelvic sentinel lymph nodes were sampled. Asan Medical Center's Institutional Review Board exempted this project. Sentinel paraaortic and pelvic lymph nodes were successfully harvested by two-step method of sentinel lymph node mapping through laparoscopic, robotic, and open laparotomy methods. This surgical video provides specific steps of pelvic and para-aortic sentinel lymph node mapping.

Comparison of the Prognostic Factors of Fetuses With Congenital Pulmonary Airway Malformations According to Type.

OBJECTIVES: To compare the prognostic factors of fetuses with microcystic and macrocystic congenital pulmonary airway malformations (CPAMs). METHODS: We retrospectively evaluated fetuses with CPAMs at Asan Medical Center. The CPAM size, mass effect, and maximum cyst size in macrocystic CPAMs were evaluated prenatally. The adverse postnatal outcomes, including respiratory symptoms, mechanical ventilation, and surgery, were evaluated. RESULTS: In 118 cases, 2 fetal deaths and 1 neonatal death occurred. All cases of fetal hydrops and complete regression after birth were in the macrocystic and microcystic CPAM groups, respectively. Twenty-four neonates (20.7%) had respiratory symptoms, and 18 (15.5%) required mechanical ventilation. Sixty-three neonates (54.3%) underwent surgery, of whom 21 (33.3%) required surgery in the neonatal period. The maximum congenital pulmonary airway malformation volume ratio was significantly associated with all postnatal outcomes (P < .05), and the optimal cutoff values were lower for respiratory symptoms, mechanical ventilation, and neonatal surgery in the macrocystic CPAMs. The maximum cyst size was also associated with all postnatal outcomes in macrocystic CPAMs (P < .05). CONCLUSIONS: Different cutoff values for the maximum congenital pulmonary airway malformation volume ratio should be applied according to the CPAM type for the prediction of postnatal outcomes. The maximum cyst size can also be a useful prognostic factor in macrocystic CPAMs.