Zinner syndrome in pediatric patients: rare disease leading to challenging management.

Introduction: Zinner syndrome (ZS) is the association of seminal vesicle cysts, ipsilateral ejaculatory duct obstruction, and ipsilateral renal agenesis. This condition is very rare in children and both diagnosis and treatment may be challenging. We reviewed the clinical presentation and treatment describing our experience with a series of three patients. Methods: From January 2016 to January 2021, three patients (patients 1, 2, and 3) with symptomatic ZS, aged 2, 15, and 17 years, respectively, were diagnosed and treated. All three patients were symptomatic, manifesting pelvic pain and dysuria. The diagnosis was made by physical examination, ultrasonography, and abdominopelvic MRI. Patient 1 underwent open surgery, while for patients 2 and 3, laparoscopic excision was performed. Results: The renal agenesis regarded the left side in patients 1 and 3, and the right side in patient 2. In all cases, the cystic complex was excised. The mean operating time was 4 h and the mean hospitalization time was 5 days (range 4-6 days). The mean follow-up period was 5 years (range 2-5 years). Patients 1 and 3 showed a complete resolution of the symptoms during postoperative follow-up. In patient 2, clinical symptoms relapsed because of the persistence of a 9 mm cyst requiring a redo laparoscopic excision. Conclusions: Seminal vesicle cyst with ipsilateral renal agenesis, even if rare in pediatric age, should be suspected in young male patients presenting with pelvic cystic masses, pelvic pain, dysuria, and ipsilateral renal absence. Conservative management should be reversed to asymptomatic patients. Surgical treatment is mandatory in symptomatic cases and the preferred approach is minimally invasive surgery to magnify the operating field to spare anatomical structures, primarily the contralateral vas deferens. Radicality is crucial to avoid the persistence of symptoms and the need for reintervention.

Pediatric liver cirrhosis interventional procedures: from biopsy to transjugular intrahepatic portosystemic shunt.

Cirrhosis is a complex diffuse process whereby the architecture of the liver is replaced by abnormal nodules because of the presence of fibrosis. Several pediatric diseases such as extrahepatic portal vein obstruction, biliary atresia, alpha-1-antitrypsin deficit and autoimmune hepatitis can lead to cirrhosis and portal hypertension in children. In this article the authors describe interventional radiology procedures that can facilitate the diagnosis and treatment of diseases associated with liver cirrhosis and portal hypertension in the pediatric population. These procedures include image-guided liver biopsy, mesenteric-intrahepatic left portal vein shunts, balloon-occluded retrograde transvenous obliteration, transjugular intrahepatic portosystemic shunts and splenic embolization.

640-Slice CT Measurement of Superior Orbital Fissure as Gateway for Light into the Brain: Statistical Evaluation of Area and Distance.

OBJECTIVE: Our aim was to provide normative data concerning superior orbital fissure area (SOFA), ocular skin and the substantia nigra (D-SS) and orbital fissure and the substantia nigra (D-SOF-S) distances by CT scan in adult Caucasian population. METHODS: The area of the superior orbital fissure (SOF), the distance between the ocular skin and the substantia nigra and the distance between the superior orbital fissure and the substantia nigra using CT and 3D-CT images. RESULTS: Normative data stratified for age and gender were obtained. The data here reported show that some degree of variability in SOFA, D-SS and D-SOF-S measurements can be observed healthy Caucasian subjects. Gender stratified prediction intervals (mean +/- 2 Standard Deviations) for SOFA and D-SOF-S were 69.2 (+/-15.8) and 38.4 (+/-7.6) for male and 56.8 (+/-11.9) and 36.5 (+/-6.1) for female, respectively. Age and gender significantly impacted on D-SS values and normative data were constructed generating data stratified for these two variables. D-SS was 89.4 (+/-10.3) and 86.4 (+/-9.7) for male and female, respectively. CONCLUSIONS: Here we provide adjunctive anatomical information on specific anatomical cerebral zones. Our data may have implications for surgeons actively committed to treat pathological conditions involving these cerebral areas. Additionally, the anatomical variability found with respect to SOF and the potential different exposure of the substanzia nigra to the bright light could play a role in Parkinson's disease as already speculated in literature.

Spine epidural and sacroiliac joints injections--when and how to perform.

OBJECTIVES: To review the state-of-the-art of image-guided techniques used to treat painful syndromes of the lower back, their indications, how they should be performed, their related risks and the expected results. METHODS: We describe the actual standards about image-guided infiltrative therapies both on spine and on sacroiliac joints. RESULTS: Both spinal epidural and sacroiliac injections appear useful in a large percentage of treated patients to get control of the perceived pain. Performing these therapies under CT or fluoroscopic guidance is the best and safest way to obtain satisfactory results because it is possible to target the use of drugs directly to the involved painful structures. CONCLUSIONS: Image-guided injections of the epidural space and of the sacroiliac joints are effective techniques for the treatment of pain; their effectiveness is sometimes not lasting for long periods of time but considering the low associated risk when performed by trained personnel, they can be easily repeated.

Critical role of HIFU in musculoskeletal interventions.

Magnetic resonance-guided focused ultrasound surgery (MRgFUS) is a totally noninvasive ablation technique that in the last years had an important development in a large number of applications: in particular gynaecological disorders, bone lesions, prostate, breast, brain and other organs. This review of MRgFUS is focused on the technical aspects and the current clinical application in musculoskeletal interventions. More precisely, this paper aims to review the relatively scarce literature on this topic also in comparison with our 3-year experience in the use of this technique in the field of musculoskeletal interventions.