National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma.

A consensus meeting of national experts from all major national hepatobiliary centres in the country was held on May 26, 2023, at the Pakistan Kidney and Liver Institute & Research Centre (PKLI & RC) after initial consultations with the experts. The Pakistan Society for the Study of Liver Diseases (PSSLD) and PKLI & RC jointly organised this meeting. This effort was based on a comprehensive literature review to establish national practice guidelines for hilar cholangiocarcinoma (hCCA). The consensus was that hCCA is a complex disease and requires a multidisciplinary team approach to best manage these patients. This coordinated effort can minimise delays and give patients a chance for curative treatment and effective palliation. The diagnostic and staging workup includes high-quality computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography. Brush cytology or biopsy utilizing endoscopic retrograde cholangiopancreatography is a mainstay for diagnosis. However, histopathologic confirmation is not always required before resection. Endoscopic ultrasound with fine needle aspiration of regional lymph nodes and positron emission tomography scan are valuable adjuncts for staging. The only curative treatment is the surgical resection of the biliary tree based on the Bismuth-Corlette classification. Selected patients with unresectable hCCA can be considered for liver transplantation. Adjuvant chemotherapy should be offered to patients with a high risk of recurrence. The use of preoperative biliary drainage and the need for portal vein embolisation should be based on local multidisciplinary discussions. Patients with acute cholangitis can be drained with endoscopic or percutaneous biliary drainage. Palliative chemotherapy with cisplatin and gemcitabine has shown improved survival in patients with irresectable and recurrent hCCA.

Impact of fixed dental prosthesis on neuroimaging: assessment of artefacts.

Objectives: To determine the frequency and extent of artefacts in magnetic resonance imaging and/or computed tomography scans of head caused by fixed dental prosthesis. METHODS: The retrospective study was conducted at Aga Khan University Hospital from July to December 2021, and comprised magnetic resonance imaging and/or computed tomography scans from January 2015 to December 2020 of the head of individuals with existing fixed dental prosthetic work at the time of exposure. They were analysed for the presence of artefacts. The association between artefacts and the presence of fixed dental prosthesis was explored. Data was analysed using SPSS 23. RESULTS: Of the 297 images evaluated, 173 (58%) were magnetic resonance imaging scans, and 124(42%) were computed tomography scans. The most common artefacts was grade I 148(49.8%), followed by grade II 140(47.1%) and grade III 9(3%). There was no significant association between fixed dental prosthesis and the artefacts (p>0.05). Conclusion: There should be no reservations in placing fixed metal prosthesis in individuals on account of future brain scans.

Acquired Uterine Vascular Anomaly: Experience from a tertiary care centre in Pakistan.

Objectives: This study aimed to retrospectively review imaging findings and the outcomes of uterine artery embolisation (UAE) in symptomatic uterine vascular anomalies (UVA). Methods: This study included a total of 15 patients with acquired UVA admitted to the Aga Khan University Hospital in Karachi, Pakistan, from 2010 to 2020. These patients were evaluated using ultrasound, computed tomography and magnetic resonance imaging, either alone or in combination. All patients had a history of dilatation and curettage or uterine instrumentation and underwent angiography and embolisation of the uterine arteries. The primary outcome post embolisation was assessed clinically and/or in combination with ultrasound. Post-procedure pregnancies were also recorded. Results: Non-invasive imaging was abnormal in all patients; however, this pre-intervention imaging was unable to accurately classify the type of vascular anomaly, except in the case of a pseudoaneurysm. Conventional angiography showed uterine artery hyperaemia in six patients, arteriovenous malformation in seven patients and pseudoaneurysm in two patients. The technical success rate was 100% and no repeat embolisation was needed. The follow-up ultrasound in 12 patients revealed a resolution of the abnormal findings, while the remaining three were found to be normal on clinical follow-up. Seven patients (46.7%) had a normal pregnancy 15.7 months after the procedure (range: 4-28 months). Conclusions: UAE is a safe and effective management option for intractable severe bleeding in patients with UVA post instrumentation and it was found that the procedure does not impair future pregnancy.

Percutaneous endovascular removal of a broken port-a-catheter from right atrium using manual made snare.

Fracture and migration of port-a-catheter, following long term access into the central venous vasculature is a rare clinical scenario. The consequences of fracture and migration includes fragmented device relocating into the right atrium or ventricle, eventually causing life threatening complications such as arrhythmias, pseudoaneurysms, perforations or very rarely embolization. We report a case of a 67-year-old female with a broken port-a-catheter which had been placed initially for chemotherapy for bilateral breast cancer. Chest radiograph showed the fragmented catheter had migrated to the right atrium; which was successfully removed via percutaneous radiological endovascular intervention. No immediate post procedure complication was noted.

Assessment of the normal pediatric pineal gland - MR findings of a single centre; A retrospective study.

OBJECTIVE: To analyse the size and morphology of the normal pineal gland in the paediatric age group using magnetic resonance imaging. METHODS: The retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised data of patients aged <18 years who had magnetic resonance imaging done between 2007 and 2017. Pineal size was estimated measuring its largest anteroposterior and supero-inferior diameters on sagittal T2-weighted sequences on any slice with maximal diameter. The width of pineal gland was measured on axial or coronal T2 sequences. The volume, morphology and enhancement patterns of the gland were also assessed. Data was analysed using SPSS 21. RESULTS: Of the 200 patients, 116(58%) were males and 84(42%) were females. The overall mean age was 7.8±5.5 years. The mean anteroposterior dimension was 4.85±1.46mm, height 3.31±0.95mm, width 4.16±1.19, and volume 39.54±38.12 mm3. Mean size of cyst was 2.77±1.4mm (range: 1.2-7.5mm). No significant differences in the pattern of enhancement were found in different age groups (p>0.05). CONCLUSIONS: Comprehensive knowledge of the size of the typical pineal organ is useful for radiologists in the identification of pineal gland anomalies and the exclusion of neoplastic lesions.

Angioembolisation for the Treatment of Pseudo-aneurysm of Internal Maxillary Artery.

Pseudo-aneurysm of internal maxillary artery, following a road traffic accident, is a rare clinical scenario. The consequence of pseudo-aneurysm may be spontaneous rupture of the arterial wall, which may eventually lead to life-threatening hemorrhage. We report a case of a 20-year male who presented with recurrent epistaxis. CT scan was performed, which revealed a pseudo-aneurysm of the internal maxillary artery; this was successfully treated by angioembolisation. Similar cases of traumatic pseudo-aneurysms have been reported, however, none presented with recurrent epistaxis after management of pan-facial fractures. Key Words: Epistaxis, Pseudo-aneurysm, Angioembolisation, Pan-facial fracture.

To determine the efficacy of bilateral inferior petrosal sinus sampling in differentiating Cushing disease from ectopic Cushing syndrome.

Management of endogenous Cushing syndrome is based on its aetiology. Increased Adrenocorticotropic Hormone (ACTH) levels are the most common cause of this disorder and, therefore, it is critical to determine the source of ACTH before further management. Dynamic post contrast MRI is currently the most common investigation implied to diagnose pituitary adenoma; however, it comes with the drawback of low specificity and high false positive results. Inferior petrosal sinus sampling (IPSS) is an established invasive procedure performed to differentiate central versus peripheral source of ACTH which, in turn, results in hypercortesolaemia. This is a series of 14 patients who underwent IPSS at the Department of Radiology, Aga Khan University Hospital, Karachi, from January 2006 to December 2018. The case series emphasises the role of IPSS in the management of ACTH-dependent Cushing syndrome and combined efficacy of Dynamic post-contrast MRI and the procedure under focus.

Angiography and Therapeutic Embolisation of Bleeding Aberrant Iliac Artery Branches.

A 78-year female presented with the complain of per rectal fresh bleeding for 4 days. She was known to have diabetes and hypertension, 3 weeks back. She had an episode of left middle cerebral artery (MCA) stroke. After stroke, she suffered from upper limb weakness and aphasia. At the time of presentation, her vitals showed blood pressure of 118/52 mmHg, O2 saturation of 98%, temperature: 37°C, respiratory rate (RR) of 20/min, and heart rate (HR) of 90 bpm. After achieving hemodynamic stability, she was transferred to radiology department. Her presenting complain of active rectal bleeding was managed by interventional radiologist using angiographic embolisation. In this patient, it was found pooling of blood in a retrograde fashion in the sigmoid colon. Bleeding was initially believed to be coming from sigmoid arteries seen on images of CT scan and colonoscopy. However, arteriography showed that source of bleeding was from middle and inferior rectal arteries that originated from left internal iliac artery. The intervention radiology (IR) team had to put in extensive effort to locate and perform therapeutic embolisation.

Challenges in diagnosis of disease reported 100 years back: Cushing syndrome; recent advances.

Treatment of Cushing syndrome depends on diagnosis of etiology responsible for hypercortisolism in the body, which sometimes presents with a challenge. Inferior petrosal sinus sampling for ACTH levels, followed by peripheral venous sampling is a proven tool to be a gold standard for differentiating between peripheral and central cause of ACTH dependent Cushing syndrome. This case report is of an elderly female who presented as an outpatient in the endocrinology clinic of Aga Khan university hospital on 22/6/2017 with clinical features of hypercortisolism. After workup she was found to have cushing syndrome secondary to ACTH secreting bronchial carcinoid tumour.

Neuroimaging Findings in Tuberculosis: A Single-Center Experience in 559 Cases.

BACKGROUND AND PURPOSE: There is a paucity of literature related to the neuroimaging of CNS tuberculosis (TB) and largely covers pediatric CNS TB. The objective of this study was to determine the frequency of different forms of CNS TB and its associated complications and to study longitudinal disease course using computed tomography (CT) and MRI. METHODS: Retrospective chart and imaging review of patients diagnosed with CNS TB in a tertiary care hospital in Pakistan over a 10-year period. A total of 452 initial brain MRI and 209 CT scans were reviewed by an expert radiologist specialized in neuroimaging. This was followed by review of 53 MRI/52 CT and 7 MRI/14 CT first and second follow-up scans, respectively. RESULTS: Note that 559 patients, 296 males and 263 females were included in the study. On the initial CT scans, tuberculomas were found in 25 (12%), infarction in 54 (25%), basal meningeal enhancement in 29 (14%), and hydrocephalus in 84 (40%). On initial MRI, tuberculomas were found in 182 (40%), infarction in 120 (27%), basal meningeal enhancement in 184 (41%), and hydrocephalus in 116 (26%). On review of follow-up CT scans, 13 (25%) showed new or worsening hydrocephalus, 8 (15%) showed new infarcts, 1 exhibited new tuberculoma, and 5 showed worsening cerebral edema. On review of follow-up MRI scans, new or worsening hydrocephalus was seen in 3 (6%), new infarcts in 3 (6%), new tuberculoma in 10 (19%), worsening cerebral edema in 7 (13%), and TB myelitis in 4 (8%) patients. CONCLUSIONS: Tuberculoma, hydrocephalus, and cerebral infarcts are the most prominent findings in CNS tuberculosis. Our study showed development of new lesions on subsequent neuroimaging suggesting a dynamic and progressive nature of the disease process in some individuals.

Role of ADC values and ratios of MRI scan in differentiating typical from atypical/anaplastic meningiomas.

Atypical/anaplastic meningiomas are prone to aggressive behaviour which affects treatment planning and prognostication. Our aim was to assess the role of Apparent Diffusion Coefficient (ADC) values of MRI brain in differentiating typical from atypical/anaplastic meningioma. We reviewed 84 typical and 37 atypical/anaplastic meningiomas and compared mean ADC values and ADC ratios of their preoperative MRI brain. At 3 Tesla, mean ADC value for typical meningioma was1.03±0.10x10-3 and 0.63±0.05x10-3 for atypical/anaplastic meningioma. At 1.5 Tesla, mean ADC value for typical meningioma was 1.05±0.11x10-3 and atypical/ anaplastic meningioma was 0.70 ± 0.04x10-3. The mean ADC ratios were 1.08 ± 0.17 and 0.85 ± 0.15 for typical and atypical/anaplastic meningomas respectively. Mean ADC ratios and the mean ADC values of typical and atypical/anaplastic meningiomas were significantly different (p< 0.001). ADC values and ADC ratios have important role in differentiating typical from atypical/anaplastic meningioma and it must be part of the routine preoperative MRI reporting.

Prevalence of subarachnoid haemorrhage among patients with cranial venous sinus thrombosis in the presence and absence of venous infarcts.

Introduction In patients with cranial venous sinus thrombosis, the occurrence of subarachnoid haemorrhage in association with haemorrhagic venous infarcts is a well described phenomenon. However, the presence of subarachnoid haemorrhage in patients with cranial venous sinus thrombosis in the absence of a haemorrhagic venous infarct is exceedingly rare. Methods We retrospectively reviewed charts and scans of all patients who had cranial venous sinus thrombosis confirmed by magnetic resonance venography at our hospital between September 2004 and May 2015. The presence of subarachnoid haemorrhage was ascertained on fluid-attenuated inversion recovery, susceptibility-weighted imaging and/or unenhanced computed tomography scans by a single experienced neuroradiologist. Statistical analysis was performed using the Statistical Package for Social Sciences version 20. Differences in the proportion of haemorrhagic venous infarcts among patients with subarachnoid haemorrhage versus those without subarachnoid haemorrhage were compared using the chi-square test. A P value of less than 0.05 was considered significant. Results A total of 138 patients who had cranial venous sinus thrombosis were included in the study. Seventy-three (52.9%) were women and the median age of subjects was 35 (interquartile range 22-47) years. Venous infarcts and haemorrhagic venous infarcts were noted in 20/138 (14.5%) and 62/138 (44.9%) cases, respectively. Subarachnoid haemorrhage was present in 15/138 (10.9%) cases and, in three cases, subarachnoid haemorrhage occurred in the absence of a venous infarct. Haemorrhagic venous infarcts were more prevalent ( P = 0.021) among patients with subarachnoid haemorrhage (11/15) than in those without subarachnoid haemorrhage (51/123). Conclusion In patients with cranial venous sinus thrombosis, subarachnoid haemorrhage can occur even in the absence of a haemorrhagic venous infarct. The recognition of cranial venous sinus thrombosis as the underlying cause of subarachnoid haemorrhage is important to avoid misdiagnosis and inappropriate management.

Pituitary stalk interruption syndrome presenting in a euthyroid adult with short stature.

Pituitary stalk interruption syndrome (PSIS) is a distinct and rare clinical entity responsible for congenital hypopituitarism resulting in deficiency of pituitary hormones with deficiency of the growth hormone (100%) and gonadotropins (97.2%) being its most common presentation at the time of hospital encounter (Wang et al., 2015). Isolated sparing of thyroid-stimulating hormone (TSH) with deficiency of the remaining anterior pituitary hormones may be present in PSIS, as is true in our case. Therefore, it should be kept in mind at the time of examination in suspected cases of PSIS.

Percutaneous Management of Biliary Enteric Anastomotic Strictures: An Institutional Review.

Purpose Stricture formation at the biliary enteric anastomotic site is a common complication due to fibrotic healing. Few therapeutic options are available for biliary-enteric anastomotic site stricture (BES) including new surgical reconstruction or percutaneous transhepatic biliary drainage followed by balloon dilation of BES or stent placement. The purpose of this study is to assess the technical success, complications and reintervention rate of percutaneous transhepatic balloon dilatation (PTBD) of BES after iatrogenic bile duct injuries (BDI). Methods A retrospective review of patients who underwent PTBD for benign resistant BES, previously treated for iatrogenic BDI, from December 2004 to January 2016 was performed. Diagnostic transhepatic cholangiogram was performed to assess the level of obstruction. BES was dilated using 8-12 mm diameter balloons followed by placement of eight to ten Fr internal-external drainage catheters, which were removed after three to six weeks post-PTBD cholangiogram. Follow-up by clinical assessment, liver function tests, and ultrasound was done. Fischer exact test was used to determine if there was a significant association between PTBD sessions and recurrent strictures. Results In total, 37 patients underwent 66 sessions of PTBD, including 10 (27%) males and 27 (73%) females. The mean age was 41.3 years (range 23-70 years). Out of these, 29 (78%) were treated with choledochojejunostomy and eight (22%) with hepaticojejunostomy. 100% technical success was achieved in all the PTBD sessions. Nineteen (51.3%) patients were treated with a single PTBD session. Mean follow-up time was 36 months (range 1-75 months). Eighteen (48.7%) patients needed reintervention, out of these, 11 (29.7%) were symptom-free after second session on three-year follow-up, three (8%) were symptom-free after the third session of PTBD. No significant difference was observed in risk of recurrent strictures after first and second PTBD sessions [18 (48%) vs. 7 (39%); p-value 0.495]. In four (11%) patients, the symptoms persisted and BES recurred even after third session and those were treated by placing metallic stent. In total, three (8.1%) patients got complicated with the stone formation; in two (5%) patients stone was successfully removed percutaneously and in one (3%) patient percutaneous attempt failed so it was followed by surgical removal. Conclusion PTBD is a safe and useful treatment option for benign BES for long-term symptom-free time-period. However, there is no significant difference in developing recurrent BES after PTBD sessions. Few patients with resistant strictures might require stent placement.

Fibrolipomatous Hamartoma of the Median Nerve with Macrodystrophia Lipomatosa.

Fibrolipomatous hamartoma (FLH) is a rare congenital condition that presents with a benign overgrowth of the bone and fibroadipose tissue termed as macrodystrophia lipomatosa (MDL). Although commonly seen in the median nerve, other peripheral nerves can be involved. Diagnosis can be made on magnetic resonance imaging (MRI) due to the characteristic coaxial cable appearance on axial images and the spaghetti appearance on sagittal images. Histology shows mature adipose and fibrous tissue infiltrating the epineural and perineural compartments. Multiple or debulking surgeries are often needed, with an emphasis on cosmetic aspects. We present one such case in which wide margin excision and sural nerve graft were carried out.

Institutional Review of Hemorrhagic Pelvic Emergencies Effectively Managed with Percutaneous Arterial Embolization.

OBJECTIVE: Our aim was to review the results of pelvic arterial embolization (PAE) performed in the interventional radiology suite. METHOD: The data of all patients in whom pelvic angioembolization was performed was collected from July 2011 to June 2017. Procedures were performed by an experienced interventional radiologist. The clinical and laboratory data, as well as the outcome data, were obtained from the medical records of our hospital. The following parameters were collected for each patient, including the age, gender, presenting symptoms, site of bleeding, catheters used for embolization, material used for embolization, previous computed tomography (CT) scan and/or focused assessment with sonography for trauma (FAST) ultrasound, average hemoglobin before the procedure, and patient clinical status on discharge. RESULT: A total of 37 patients underwent pelvic angiography for acute hemorrhage at our institution. They had contrast blush, active extravasation, or abnormal vascularity from the branches of the internal iliac artery and underwent therapeutic transcatheter embolization. A total of 29 patients (78.3%) were male and 8 (21.7%) were female. The average age was 30.0 years (range: 6-90 year). Of these, 16 patients (43.2%) presented with road traffic accidents (RTAs), six with gunshot injuries (16.2%), six with iatrogenic injuries (16.2%), four with a history of a fall (10.8%), two with bomb blast injuries (5.4%), one with a history of a glass injury (2.7%), one had a history of a roof falling on her during an earthquake, and one patient had a pelvic pseudoaneurysm secondary to an abscess. The type of embolic material used for embolization included coils in 16 patients, polyvinyl alcohol (PVA) particles were used in eight patients, both PVA particles and coils were used in 11 patients, and glue was used in one patient. All were successfully embolized. Thirty-four were discharged while three patients expired during the course of hospital stay due to other coexisting morbidities. CONCLUSION: The management of pelvic injuries has always been a topic of debate, with multiple methods reported to date but growing evidence supports the use of pelvic arterial embolization in hemorrhagic pelvic injuries. The formulation of a standardized protocol is the need of the day.

Diagnostic Accuracy of Pelvic MRI for Determination of the Cervical Involvement in Endometrial Cancer.

OBJECTIVE: To determine the diagnostic accuracy of pelvic MRI for assessment of the cervical involvement in endometrial cancer. STUDY DESIGN: Cross-sectional analytical study. PLACE AND DURATION OF STUDY: Radiology Department of the Aga Khan University Hospital, Karachi from January 2014 to December 2015. METHODOLOGY: Patients with biopsy-proven endometrial cancer were included, who had both their MRI and histopathological diagnosis performed at our institution. Those patients treated with chemo/radiotherapy or had incomplete medical records, were excluded. The extent of cervical involvement by endometrial carcinoma was seen on T2WI images, and findings were correlated after surgery taking histopathology as the gold standard. The sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy were calculated. RESULTS: The mean age of the 56 patients was 60.87 ±8.80 years (range 37-84 years). The most common clinical indication was post-menopausal bleeding (n=37, 66.1%). The most common histological subtype was endometrioid adenocarcinoma (n=50, 89.3%). The sensitivity, specificity, diagnostic accuracy, positive and negative predictive values of MRI in the detection of cervical invasion were 92.85%, 88.09%, 89.28%, 72.22% and 97.36%, respectively. CONCLUSION: MRI is a highly sensitive and specific imaging modality for detection of cervical invasion in endometrial carcinoma.

Non-ionic iodinated contrast-induced sialadenitis with parotid gland sparing in patient of hepatocellular carcinoma.

61-years-old male patient presented with complain of neck swelling and soreness following contrast-enhanced (CE) CT examination with resolution of symptoms in 36-48 hours. He is diagnosed with hepatitis C virus and hepatocellular carcinoma (HCC); already treated with radiofrequency ablation for HCC. He had already undergone two CECT examinations before he was referred to our institution for disease staging/treatment. He also underwent three triphasic CT scan examinations at our institution during the course of treatment for treatment response and staging. Patient remained undiagnosed up to his fourth CT scan due to inability to relate symptoms with contrast administration. The patient was offered close monitoring on fifth CT exam and ultrasound of neck revealed enlarged and echogenic bilateral submandibular glands, however, thyroid and bilateral parotid glands appear unremarkable. This represents transient iodinated contrast induced sialadenitis with sparing of parotid glands.

Varied percutaneous and endovascular management of large iatrogenic Profunda Femoris artery pseudoaneurysm.

The case of an elderly male diagnosed with a large iatrogenic pseudoaneurysm arising from the profunda femoris artery on ultrasound examination is presented. The immediate postoperative period was uneventful; however later he developed leg swelling and mild oozing at the incision site. In view of deranged renal function CT angiogram was not done and decision was made to treat the pseudoaneurysm with percutaneous thrombin injection. Almost complete thrombosis was achieved however a small residual portion remained patent near the neck. Later limited contrast angiography was done which re-demonstrated a small anneurysm. Instead of embolizing the vessel completely repeat prolonged balloon inflation was done which later showed no further filling of the pseudoaneurysm.

A large choledochocystolithiasis mimicking Mirizzi syndrome.

An 18-year-old man presented with spontaneous severe epigastric pain, progressing and radiating to back since 3 days. It was associated with epigastric tenderness, bilious vomiting and jaundice. He had been intermittently experiencing these symptoms for the last 1 year. No known comorbid. Ultrasound showed a poorly visualised heterogeneous focus at porta hepatis; considering poor visualisation, this might represent an enlarged calcified lymph node or cystic duct calculus causing extrinsic compression or a large sludge ball within the common bile duct (CBD), leading to dilatation of common hepatic duct and intrahepatic biliary system. Subsequent magnetic resonance cholangiopancreatography revealed a focal saccular dilatation of middle part of CBD, a type I-B choledochal cyst, large heterogeneous focus seen within it representing choledochocystolithiasis. Later, CT was performed for further characterisation of surrounding anatomy and pathology, which confused the appearance of choledochocystolithiasis for Mirizzi syndrome. Later, surgery and histopathology confirmed type I-B choledochocystolithiasis and chronic cholecystitis.