Clival Chordoma in an Adolescent: A Perspective from Primary Care / 가정의학회지

Clival chordoma is a rare malignant tumor of the brain that typically occurs in older adults. It has a high local recurrence rate and is hence associated with poor prognosis. Here, we report a case of an adolescent who presented with a 1-month history of worsening headache and blurring of vision, as well as a 6-month history of left-sided facial and body numbness. Clinical findings were consistent with left upper motor neuron lesion of the seventh cranial nerve with involvement of the fifth cranial nerve. He was also found to have a sixth cranial nerve palsy demonstrated by diplopia upon lateral gaze with no evidence of papilledema. Magnetic resonance imaging of the brain suggested clival chordoma. He was subsequently referred to the neurosurgical team, and he successfully underwent an endoscopic trans-sphenoidal surgery to excise the lesion. He recovered well, continued his follow-ups with the neurosurgical team, and showed good progress. He also attended regular follow-ups with his primary care physician to ensure ongoing psychosocial support and monitoring of his overall health status. This case demonstrates the importance of prompt identification and treatment of clival chordoma in an adolescent. Long-term follow-ups and shared care between primary and secondary care physicians are essential to monitor recurrence of tumor and to provide psychosocial support.

Pleuritic chest pain and fever: An unusual presentation of aortic dissection

@#It remains a challenge to diagnose aortic dissection in primary care, as classic clinical features are not always present. This case describes an atypical presentation of aortic dissection, in which the patient walked in with pleuritic central chest pain associated with a fever and elevated C-reactive protein. Classic features of tearing pain, pulse differentials, and a widened mediastinum on chest X-ray were absent. This unusual presentation highlights the need for a heightened level of clinical suspicion for aortic dissection in the absence of classic features. The case is discussed with reference to the literature on the sensitivity and specificity of the classic signs and symptoms of aortic dissection. A combination of the aortic dissection detection risk score (ADD-RS) and D-dimer test is helpful in ruling out this frequently lethal condition.

Onyx In Brain Arteriovenous Malformation Embolization

Introduction: Embolisation has long been used as an adjunct to surgical resection in the treatment of brain arteriovenous malformation (bAVM). The most commonly used embolic material, n-butylcyanoacrylate glue, requires experience and skill to handle its quick and unpredictable flow and polymerisation. A new liquid embolic agent, ethylene vinyl alcohol copolymer (Onyx), is less adhesive and polymerises slowly, which provides better control for radiologists performing embolisation. Objective: To report our experience in embolisation using Onyx alone or in combination with histoacryl for bAVM embolisation in our tertiary referral centre. Methods: We retrospectively reviewed the anatomy, technical conditions, complications and clinical outcome of all bAVM patients embolised at our centre using Onyx alone or in combination with n-butylcyanoacrylate glue. Results: Between 2010 and 2013, 13 patients [6 (46.2%) male; 7 (53.8%) female; aged, 14– 57 years] were included, and a total of 31 embolisations were performed. Clinical presentation included hemorrhage [9 (69.2%)], seizures [2 (15.4%)], and headache [2 (15.4%)]. Most AVMs were located in the brain hemispheres [12 (92.3%)] and measured <3 cm [7 (53.8%]. Complete occlusion of the AVM was obtained in 2 (15.4%) patients; 11 (84.6%) patients had partial occlusion [6 (54.5%) had <50% nidus occlusion]. Complications occurred in four procedures involving 3 patients (morbidity, 23.1%). This resulted in the death of 1 patient (mortality, 7.7%) and complete recovery with no disability in 2 patients. Conclusion: The total nidal occlusion achieved herein is comparable to other similar studies. Our morbidity and mortality were higher compared to other studies which may be attributed to the small number of patients. More data is being collected which may better reflect on our experience.

Atypical Clival Chordoma in an Adolescent without Imaging Evidence of Bone Involvement

Clival chordoma is a rare primary bone tumour that arises from the remnant of the notochord and typically occurs in older adults. Upon imaging, the tumour can be seen arising from the clivus and causes clival destruction. This usually provides insight for a diagnosis. Here we present a case of a non-enhancing, pre-pontine mass that was hypointense on T1W and hyperintense on T2W in an adolescent. No clival bone erosion was observed. Based on the age group, imaging findings, and lack of clival erosion, a provisional diagnosis of epidermoid cyst was made and the tumour was resected. This patient was eventually diagnosed with a clival chordoma based on histopathological examination.

Evaluation of time-dependent pathways in an acute ischemic stroke protocol that incorporates CT perfusion: A tertiary referral center experience

Background and Objective: Intravenous thrombolysis service for stroke was introduced at the Universiti Kebangsaan Malaysia Medical Centre (UKMMC) in 2009, based on the recommendations of a multidisciplinary team of clinicians. We report the experience at our center in establishing a stroke protocol incorporating computed tomography perfusion (CTP) of the brain, to assess the feasibility of incorporating CTP in the stroke protocol. Methods: A retrospective review of all patients who had a CTP between January 2010 and December 2011 was performed. Results: Of 272 patients who were admitted with acute ischemic stroke, 44 (16.2%) arrived within 4.5 hours from symptom onset and had a CTP performed with the intention to treat. The median time for symptom-to-door, symptom-to-scan and door-to-scan was 90.0 minutes (62.5 – 146.3), 211.0 minutes (165.5 – 273.5) and 85.0 minutes (48.0 – 144.8) respectively. Eight patients (2.9%) were thrombolysed of whom five received IV thrombolysis and three underwent mechanical thrombolysis. The median symptom-to-needle and door-to-needle times were 290.5 minutes (261.3 – 405.0) and 225.0 minutes (172.5 – 316.8) respectively. Four patients were thrombolysed despite being outside the window of treatment based on the CTP findings. Six of the thrombolysed patients had a Modified Rankin Score (MRS) of 1-2 at 5 months post procedure. Conclusions: CTP provides a benefit to management decisions and subsequent patient outcome. It is feasible to incorporate CTP as a standard imaging modality in a stroke protocol. The delays in the time-dependent pathways are due to our work flow and organisational process rather than performing the CTP per se.

Uterine Arteriovenous Malformation

Uterine arteriovenous malformation (AVM) is a rare condition, with fewer than 100 cases reported in the literature. Despite it being rare, it is a potentially life-threatening condition. This case report describes a 33-year-old woman who presented with secondary post-partum hemorrhage. Transabdominal ultrasound (US) of the pelvis showed increased vascularity with multidirectional flow of the uterus and a prominent vessel, located on the left lateral wall. She also had retained product of conception, which complicated the diagnosis. A uterine artery angiogram confirmed an AVM in the fundal region with an early draining vein. Embolisation of the AVM was performed successfully.