Background: Posterior reversible encephalopathy syndrome (PRES) is a rare complication of lung transplantation with poorly understood risk factors and clinical characteristics. This study aimed to examine the occurrence, risk factors, and clinical data of patients who developed PRES following lung transplantation. Methods: A retrospective analysis was conducted on 147 patients who underwent lung transplantation between February 2013 and December 2023. The patients were diagnosed with PRES based on the clinical symptoms and radiological findings. We compared the baseline characteristics and clinical information, including primary lung diseases and immunosuppressive therapy related to lung transplantation operations, between the PRES and non-PRES groups. Results: PRES manifested in 7.5% (n=11) of the patients who underwent lung transplantation, with a median onset of 15 days after operation. Seizures were identified as the predominant clinical manifestation (81.8%, n=9) in the group diagnosed with PRES. All patients diagnosed with PRES recovered fully. Patients with PRES were significantly associated with connective tissue disease-associated interstitial lung disease (45.5% vs. 18.4%, P=0.019, odds ratio=9.808; 95% CI, 1.064–90.386; P=0.044). Nonetheless, no significant variance was observed in the type of immunotherapy, such as the use of calcineurin inhibitors, blood pressure, or acute renal failure subsequent to lung transplantation. Conclusions PRES typically manifests shortly after lung transplantation, with seizures being the predominant initial symptom. The presence of preexisting connective tissue disease as the primary lung disease represents a significant risk factor for PRES following lung transplantation.

Background: In addition to vascular endothelial cells, vascular smooth muscle cells (VSMCs) are subject to continuous shear stress because of blood circulation. The angiogenic properties of VSMCs in extracranial arteriovenous malformations (AVMs) may exceed those of normal blood vessels if the body responds more sensitively to mechanical stimuli. This study was performed to investigate the hypothesis that rapid angiogenesis may be achieved by mechanical shear stress. Methods: VSMCs were obtained from six patients who had AVMs and six normal controls. The target genes were set to angiopoietin-2 (AGP2), aquaporin-1 (AQP1), and transforming growth factor-beta receptor 1 (TGFBR1). Reverse-transcriptase polymerase chain reaction (RT-PCR) and real-time PCR were implemented to identify the expression levels for target genes. Immunofluorescence was also conducted. Results: Under the shear stress condition, mean relative quantity values of AGP2, AQP1, and TGFBR1 in AVM tissues were 1.927±0.528, 1.291±0.031, and 2.284±1.461 when compared with neutral conditions. The expression levels of all three genes in AVMs were higher than those in normal tissue except for AQP1 under shear stress conditions. Immunofluorescence also revealed increased staining of shear stress-induced genes in the normal tissue and in AVM tissue. Conclusions Shear stress made the VSMCs of AVMs more sensitive. Although the pathogenesis of AVMs remains unclear, our study showed that biomechanical stimulation imposed by shear stress may aggravate angiogenesis in AVMs.

Background: Arteriovenous malformation (AVM) which is a high-blood-flow lesion with connections between arteries and veins without an intervening capillary bed, is difficult to manage. The ear is the second most common site of extracranial AVM. However, studies regarding the management of this condition remain lacking. The purpose of this study was to share managing experiences in our center and to investigate the treatment effect through a retrospective analysis of cases. Methods: Among 265 patients with AVM treated in our vascular anomalies center between January 2008 and January 2021, 10 patients with auricular AVM were included in the study to investigate the lesion distribution, clinical stage, and treatment methods by performing a retrospective evaluation. Results: Among 10 patients, five patients had AVMs distributed in the upper half of the ear, one patient in the lower half of the ear, and four patients in whole ear, respectively. Seven patients had Schobinger stage II, and three had stage III. One patient received surgical treatment only, four patients received sclerotherapy only, and five patients received both surgical treatment and sclerotherapy. The posttreatment status was checked as controlled in two patients, improved in seven patients, persistent in one patient. There were no worsening patients. Conclusion Auricular AVM is a disease that is difficult to manage by one specific department, thus requiring a collaborative management effort from multidisciplinary team.

Background: Oral propranolol has recently been introduced as a successful treatment for infantile hemangioma (IH).Though, there are limited reports on this treatment including large number of Korean patients with IH covering a long-term powder and solution formulation period. Objective: We investigated the effectiveness and side effects of two different formulations of oral propranolol treatment in patients with IH at a Korean tertiary university hospital. Methods: From June 2011 to October 2019, 375 patients were treated with powder- or solution-type oral propranolol starting at 1 mg/kg/day and increasing up to 3 mg/kg/day. Drug effectiveness was evaluated on four scales through sequential photographs by two dermatologists. Side effects were recorded on a medical chart. Results: Overall, the mean improvement scale was 2.61±0.73 at 3 months after treatment initiation. The scale was higher for solution-type than for powder-type oral propranolol at the 3-month follow-up (2.71±0.79 vs. 2.54±0.67, p＜0.05). The patients’ mean duration of treatment was 8.56±5.85 months, which was shorter for solution-type than for powder-type oral propranolol (6.0 vs. 10.69 months, p＜0.05). Among the total number of patients, 22 reported mild side effects, including loose stools and noticeable sleep disturbance, and few serious side effects such as grunting, while two patients required medical intervention. Conclusion The patients in our study were effectively treated for IH with oral propranolol without significant side effects and had a shorter treatment duration with solution-type oral propranolol than with powder-type oral propranolol.

Background: Arteriovenous malformation (AVM) which is a high-blood-flow lesion with connections between arteries and veins without an intervening capillary bed, is difficult to manage. The ear is the second most common site of extracranial AVM. However, studies regarding the management of this condition remain lacking. The purpose of this study was to share managing experiences in our center and to investigate the treatment effect through a retrospective analysis of cases. Methods: Among 265 patients with AVM treated in our vascular anomalies center between January 2008 and January 2021, 10 patients with auricular AVM were included in the study to investigate the lesion distribution, clinical stage, and treatment methods by performing a retrospective evaluation. Results: Among 10 patients, five patients had AVMs distributed in the upper half of the ear, one patient in the lower half of the ear, and four patients in whole ear, respectively. Seven patients had Schobinger stage II, and three had stage III. One patient received surgical treatment only, four patients received sclerotherapy only, and five patients received both surgical treatment and sclerotherapy. The posttreatment status was checked as controlled in two patients, improved in seven patients, persistent in one patient. There were no worsening patients. Conclusion Auricular AVM is a disease that is difficult to manage by one specific department, thus requiring a collaborative management effort from multidisciplinary team.

Background: Oral propranolol has recently been introduced as a successful treatment for infantile hemangioma (IH).Though, there are limited reports on this treatment including large number of Korean patients with IH covering a long-term powder and solution formulation period. Objective: We investigated the effectiveness and side effects of two different formulations of oral propranolol treatment in patients with IH at a Korean tertiary university hospital. Methods: From June 2011 to October 2019, 375 patients were treated with powder- or solution-type oral propranolol starting at 1 mg/kg/day and increasing up to 3 mg/kg/day. Drug effectiveness was evaluated on four scales through sequential photographs by two dermatologists. Side effects were recorded on a medical chart. Results: Overall, the mean improvement scale was 2.61±0.73 at 3 months after treatment initiation. The scale was higher for solution-type than for powder-type oral propranolol at the 3-month follow-up (2.71±0.79 vs. 2.54±0.67, p＜0.05). The patients’ mean duration of treatment was 8.56±5.85 months, which was shorter for solution-type than for powder-type oral propranolol (6.0 vs. 10.69 months, p＜0.05). Among the total number of patients, 22 reported mild side effects, including loose stools and noticeable sleep disturbance, and few serious side effects such as grunting, while two patients required medical intervention. Conclusion The patients in our study were effectively treated for IH with oral propranolol without significant side effects and had a shorter treatment duration with solution-type oral propranolol than with powder-type oral propranolol.

Background: Venous malformations (VMs) are the most common type of vascular malformations. Intramuscular venous malformations (IMVMs) are lesions involving the muscles, excluding intramuscular hemangiomas. The purpose of this study was to compare clinical outcomes between patients with IMVMs who were treated with sclerotherapy and those who were treated with surgical excision. Methods: Of 492 patients with VMs treated between July 2011 and August 2020 at a single medical center for vascular anomalies, 63 patients diagnosed with IMVM were retrospectively reviewed. Pain, movement limitations, swelling, and quality of life (QOL) were evaluated subjectively, while radiological outcomes were assessed by qualified radiologists at the center. Complication rates were also evaluated, and radiological and clinical examinations were used to determine which treatment group (sclerotherapy or surgical excision) exhibited greater improvement. Results: Although there were no significant differences in pain (P=0.471), swelling (P=0.322), or the occurrence of complications (P=0.206) between the two treatment groups, the surgical treatment group exhibited significantly better outcomes with regard to movement limitations (P=0.010), QOL (P=0.013), and radiological outcomes (P=0.017). Moreover, both duplex ultrasonography and magnetic resonance imaging showed greater improvements in clinical outcomes in the surgical excision group than in the sclerotherapy group. Conclusions Although several studies have examined IMVM treatment methods, no clear guidelines for treatment selection have been developed. Based on the results of this study, surgical excision is strongly encouraged for the treatment of IMVMs.

Sinus pericranii is a rare vascular anomaly characterized by abnormal venous communication betweenthe inner and outer regions of the cranial cavity. Here, we report a case of sinus pericraniiand venous malformations in the right periorbital region of a 2-year-old girl. Radiologic findingsshowed venous malformations in the right parietal region communicating with the superior sagittalsinus in the intracranial region. There were notable improvements following surgical resectionfor the abnormal venous lesions and several sclerotherapies. Presence of a bluish and pulsatingmass on the scalp, which showed bruit on auscultation, may indicate sinus pericranii, whichshould be included in the differential diagnosis.

BACKGROUND: Treatment for venous malformations of the head and neck includes sclerotherapy, surgical resection, or a combination of both. Surgical resection can remove or reduce the volume of vascular lesions; however, surgery can cause postoperative scarring and potential surgical complications. This study sought to determine the effectiveness of surgery for the treatment of venous malformations of the head and neck. METHODS: A retrospective review of the medical records of patients who received surgeries for venous malformations of the head and neck from January 2011 to July 2019 was performed. Using clinical photographs, preoperative and postoperative Doppler ultrasonography, outpatient clinic records, and operation records, the postoperative result and complications were evaluated for each case. RESULTS: Among patients who visited our vascular anomalies clinic, 43 patients (ratio of male to female= 24:19) received surgeries for venous malformations of the head and neck. Twenty-nine patients had undergone surgery only, five patients received sclerotherapy after surgery, and nine patients received surgery after preoperative sclerotherapy. In postoperative evaluations, the result was excellent in 24 patients, good in 18 patients, and poor in one patient. Four patients experienced a recurrence of lesions with lagophthalmos, drooping of the corner of the mouth, partial wound necrosis, and scar widening found in one patient each. CONCLUSION: Because the head and neck region is the most exposed area in the body, more active implementation of surgical treatments with or without sclerotherapy is essential to reduce the functional and cosmetic impairments associated with venous malformations.
Ambulatory Care Facilities ; Cicatrix ; Head ; Humans ; Male ; Medical Records ; Mouth ; Neck ; Necrosis ; Recurrence ; Retrospective Studies ; Sclerotherapy ; Surgery, Plastic ; Ultrasonography, Doppler ; Vascular Malformations ; Wounds and Injuries

PURPOSE: Poloxamer 407 (P407) thermo-sensitive hydrogel formulations were developed to enhance the retention time in the urinary bladder after intravesical instillation. MATERIALS AND METHODS: P407 hydrogels (P407Gels) containing 0.2 w/w% fluorescein isothiocyanate dextran (FD, MW 4 kDa) as a fluorescent probe were prepared by the cold method with different concentrations of the polymer (20, 25, and 30 w/w%). The gel-forming capacities were characterized in terms of gelation temperature (G-Temp), gelation time (G-Time), and gel duration (G-Dur). Homogenous dispersion of the probe throughout the hydrogel was observed by using fluorescence microscopy. The in vitro bladder simulation model was established to evaluate the retention and drug release properties. P407Gels in the solution state were administered to nude mice via urinary instillation, and the in vivo retention behavior of P407Gels was visualized by using an in vivo imaging system (IVIS). RESULTS: P407Gels showed a thermo-reversible phase transition at 4℃ (refrigerated; sol) and 37℃ (body temperature; gel). The G-Temp, G-Time, and G-Dur of FD-free P407Gels were approximately 10℃–20℃, 12–30 seconds, and 12–35 hours, respectively, and were not altered by the addition of FD. Fluorescence imaging showed that FD was spread homogenously in the gelled P407 solution. In a bladder simulation model, even after repeated periodic filling-emptying cycles, the hydrogel formulation displayed excellent retention with continuous release of the probe over 8 hours. The FD release from P407Gels and the erosion of the gel, both of which followed zero-order kinetics, had a linear relationship (r²=0.988). IVIS demonstrated that the intravesical retention time of P407Gels was over 4 hours, which was longer than that of the FD solution ( < 1 hour), even though periodic urination occurred in the mice. CONCLUSIONS: FD release from P407Gels was erosion-controlled. P407Gels represent a promising system to enhance intravesical retention with extended drug delivery.
Administration, Intravesical* ; Animals ; Dextrans ; Drug Liberation ; Fluorescein ; Hydrogel* ; Hydrogels* ; In Vitro Techniques ; Kinetics ; Methods ; Mice* ; Mice, Nude ; Microscopy, Fluorescence ; Optical Imaging ; Phase Transition ; Poloxamer* ; Polymers ; Urinary Bladder* ; Urination