Kristina Potočki

Spinal cord compression caused by multiple spinal osteochondromas

A 15-year-old boy with positive family history of osteochondromatosis presented with a three-month history of progressive paraparesis and a month history of urinary incontinence. The patient underwent irradiation of the whole neuroaxis for meduloblastoma at the age of 18 months. The CT and MRI showed multilevel osteochondromas of the spine with irregularly narrowed spinal canal to the level of complete obliteration, and spinal cord compression and displacement (Fig. 1A and 1B). MRI delineated the extent of the osteochondromas, particularly the cartilage cap, more accurately than CT. MRI was also superior in visualizing spinal cord compression. CT, however, was performing better in analysis of the bony structures of the tumors, calcifications, and in assessment of the spinal canal deformity (Fig. 1C). After laminectomy and partial resection of the tumor masses, neurological deficits remained, despite achieved decompression of the neural structures. Postoperative CT and MRI of the spine revealed residual exostosis that caused displacement of the spinal cord and postischemic changes of the spinal cord at the level Th2 (Fig. 2). Histological analysis of the removed specimens confirmed the benign nature of the tumors. Osteochondroma is a cartilaginous tumor, the most common benign tumor of the bone. It may be solitary, or multiple (osteochondromatosis, usually with autosomal dominant inheritance) [1, 2]. We believe that the osteochondromatosis in our patient was hereditary with possible acceleration of progression of the tumor growth caused by irradiation since it may be induced by radiation exposure [3].

Thigh abscess as an extension of psoas abscess: the first manifestation of perforated appendiceal adenocarcinoma: case report

SummaryA 65-year-old woman presented with a painful, swollen, red right thigh and the mild pain in the right abdomen without nausea, vomiting or diarrhoea that lasted for 1 week. Laboratory findings revealed elevated inflammatory markers. Computed tomography of the right thigh, abdomen and pelvis showed an abscess formation in the adductor muscles draining from the abscess that completely occupied the right retroperitoneum up to the diaphragm, dissecting downward through the inguinal canal. Appendix was enlarged with an appendicolith. Emergent exploratory laparotomy revealed a perforated appendix with psoas abscess. Pathohistological diagnosis revealed adenocarcinoma of the appendix. Thigh abscess is an uncommon condition with insidious clinical presentation. Therefore, early recognition and setting of the correct diagnosis enables adequate treatment avoiding additional complications and in some cases potential life-threatening conditions. When upper leg abscess is suspected or proven abdominal examination is mandatory.ZusammenfassungEine 65-jährige Frau präsentierte sich mit einer seit einer Woche schmerzhaften, geschwollenen rechten Hüfte und geringen Schmerzen im rechten Abdomen ohne Nausea, Erbrechen oder Diarrhoe. Im Labor waren die Entzündungsmarker erhöht. Die Computertomographie der rechten Hüfte, des Abdomens und Beckens zeigte eine Abszessbildung in den Adduktoren als Drainage von einem Abszess, der das rechte Retroperitoneum bis zum Diaphragma ausfüllte und hinunter durch den Inguinalkanal dissezierte. Der Appendix war vergrößert mit einem Appendikolithen. Die explorative Not-Laparatomie ergab einen perforierten Appendix mit einem Psoasabszess. Pathohistologisch wurde ein Adenokarzinom des Appendix befundet. Hüftabszesse sind selten und präsentieren sich klinisch schleichend. Deshalb sind das frühe Erkennen und die frühe Erstellung der korrekten Diagnose wichtig. Nur so kann eine adäquate Therapie unter Vermeidung von zusätzlichen Komplikationen und manchmal sogar lebensbedrohenden Zuständen ermöglicht werden. Wenn der Verdacht eines Abszesses im Oberschenkel besteht oder dieser bestätigt ist, muss unbedingt eine Untersuchung des Abdomens durchgeführt werden.

Akute Schulterschmerzen beidseits als Anfangssymptom einer Ochronose: Fallbericht und Literaturübersicht

Ochronosis is a rare genetic metabolic disorder resulting from a constitutional lack of homogentisic acid oxidase and subsequent accumulation of its substrate causing destruction of connective tissues with various systemic abnormalities. Typical musculoskeletal symptoms are arthropathy and low back pain and stiffness. Arthropathy primarily affects knee and hip joints, but shoulders are less frequently involved. We describe a case of ochronosis initially presented as acute bilateral shoulder pain. Review of literature with respect to the etiology, natural course of the disease, differential diagnosis and management was made.ZusammenfassungOchronose ist eine seltene, genetisch bedingte Störung des Metabolismus, die durch den Mangel an Homogentisinsäureoxidase entsteht – mit daraus folgender Anhäufung ihres Substrats, was die Destruktion des Bindegewebes und Störungen verschiedener Systeme verursacht. Arthropathie und Kreuzschmerzen sind typische muskuloskelettale Manifestationen. Von der Arthropathie sind in erster Linie Knie und Hüften betroffen. Selten ist sie in den Schultern manifest. Wir stellen einen Fall von Ochronose dar, dessen Anfangsphase von akuten Schmerzen in beiden Schultern gekennzeichnet war. Die Literatur wurde im Hinblick auf die Ätiologie, den natürlichen Krankheitsverlauf, die Differenzialdiagnose und die Behandlung herangezogen.AbstractOchronosis is a rare genetic metabolic disorder resulting from a constitutional lack of homogentisic acid oxidase and subsequent accumulation of its substrate causing destruction of connective tissues with various systemic abnormalities. Typical musculoskeletal symptoms are arthropathy and low back pain and stiffness. Arthropathy primarily affects knee and hip joints, but shoulders are less frequently involved. We describe a case of ochronosis initially presented as acute bilateral shoulder pain. Review of literature with respect to the etiology, natural course of the disease, differential diagnosis and management was made.

Breast carcinoma metastasis in retrosternal fat tissue.

A 58-year-old woman presented at her oncology specialist’s office with the symptoms of dyspnea, difficulty in breathing and a vague feeling of retrosternal unease. Eight years before, she was diagnosed with a right breast cancer, an invasive lobular carcinoma (ILC), and underwent surgery (right breast quadrantectomy). She received four courses of adjuvant chemotherapy (cyclophosphamide/doxorubicin), four cycles of docetaxel, local-regional radiotherapy, and tamoxifen hormonal therapy. Following mentioned symptoms, chest x-ray and radiography of sternum were taken which revealed no cause for patient’s symptoms. Mammography and breast ultrasound showed normal findings. Subsequently, a breast dynamic magnetic resonance imaging was performed, which showed status after right quadrantectomy without signs of recidivism or residual tumor tissue but also revealed ill-defined retrosternal lesion in the fat tissue of the anterior mediastinum, 11 9 8 cm in size and spreading to the left with inhomogeneous postcontrast enhancement (Fig. 1). A multislice computed tomography of the sternum and retrosternal space showed inhomogeneous thickening in the retrosternal soft tissues at the level of sternal body, with absorption coefficients of solid tissue along with areas of dense liquid content, posteriorly insepa-

Familial antiphospholipid syndrome presenting as bivessel arterial occlusion in a 17-year-old girl.

This article presents a case of a 17-year-old girl with primary antiphospholipid syndrome developing subacute signs of hand and leg ischaemia caused by radiologically verified radial and popliteal artery occlusion. She is successfully treated with a thrombolytic agent (alteplase) and recovers completely. Her laboratory results came positive for all three subtypes of antiphospholipid antibodies. This kind of antiphospholipid syndrome presentation is a very rare entity in itself. Shortly afterwards her mother is diagnosed with primary antiphospholipid syndrome as well. A familial form of antiphospholipid syndrome is suspected. Combination of a familial antiphospholipid syndrome presenting as bivessel arterial thrombosis is a unique case, to the best of our knowledge, never described in the literature before.

Imunological and clinical characteristics in children with polyarteritis nodosa: a retrospective study over the last 20 years

Results PAN was diagnosed in 12 patients (6 girls and 6 boys). The share of PAN amongst all vasculitides was 4%. The mean age at disease onset was (±SD) 11.33±3,08 years. Systemic PAN was diagnosed in 7 children (58%), microscopic polyangiitis in 3 (25%), cutaneus PAN in 2 (17%) and classic PAN in 0 (0%). The most consistent symptoms were skin involvement (90%) and arthritis/ arthralgia (60%). The CNS was affected in 40% of patients. ESR and CRP were elevated in all patients. Antineutrophil cytoplasmic antibodies were elevated in 3 patients (25%). Antistreptolysin O was elevated in 4 patients (25%). The relation between the severity of skin involvement and involvement of other organs was not found. Therapy mode for all patients was corticosteroids. Immunosuppressive drugs and Rituximab (antiCD20) were used as additional therapy for patients with severe symptoms. Two patients with microscopic polyangiitis died due to chronic renal and pulmonary failure during the follow-up. Conclusion In comparison to available studies, we found a difference in distribution of childhood polyarteritis nodosa as well as some clinical characteristics (e.g. higher prevalence of neurological symptoms), while other researched features, laboratory and treatment, were similar.

Multidetector computed tomography of iatrogenic urinary trauma – pictorial review

Abdomino-pelvic interventional and surgical procedures can result in iatrogenic urinary tract injury. Radiological diagnosis can be difficult to establish because findings are caused by iatrogenic trauma, and by underlying disease and therapeutical procedures. We performed multidetector computed tomography (MDCT) in patients with suspected urinary tract trauma with scanning protocol selected according to the suspected type of injury: non-enhanced scanning for detection of fluid collections, MDCT angiography for renal parenchymal and vascular changes, MDCT urography for pelvicalyceal and ureteric injuries, and MDCT cystography for bladder and urethral lesions. The injuries were found after urologic, general surgical and gynaecological/obstetric procedures. Renal parenchymal injuries were caused by blunt (extracorporeal shock-wave lithotripsy) or penetrating trauma (renal biopsy), with formation of subcapsular, perinephric and/or paranephric haematomas. Renal vascular pedicle changes after kidney transplantation were precisely defined using MDCT angiography. MDCT urography depicted leakage of contrast material from the pelvicalyceal system after nephron-sparing surgery, from the ureter after kidney transplantation, and from bladder tear after caesarean section, while urethral injury after bladder catheterization was diagnosed using MDCT cystography. MDCT angiography and/or MDCT urography present methods for evaluation of iatrogenic urinary tract trauma because they enable fast and accurate diagnosis and planning of surgical and interventional procedures.