Foetal Akinesia Deformation Sequence: A Rare Lethal Entity.

Foetal akinesia deformation sequence (FADS) represents a group of disorders resulting from absent or diminished in utero foetal mobility. The aetiology is multifactorial, including genetic, environmental, maternal, and foetal causes. The absence of foetal movements leading to multiple joint contractures, pulmonary hypoplasia, and intrauterine growth restriction are the key features of foetal akinesia deformation sequence. Herein we describe the case of a 30-year-old gravida 4 (para 2+1) who came for foetal ultrasound at 28 weeks of gestation due to decreased foetal movements. Ultrasound showed features of FADS with fixed flexed position of foetal limbs, pulmonary hypoplasia, polyhydramnios, and intrauterine growth restriction. The timely use of ultrasound enables early detection of these cases and aids in appropriate counselling and management.

Wandering spleen with acute torsion mimicking an adnexal mass.

Wandering spleen manifests when the splenic ligaments are underdeveloped, or become lax, thereby allowing the spleen to relocate from its anatomical site to more distant areas. During such movements, torsion of the long splenic peduncle is common, which can lead to symptoms of acute abdomen and further complications such as infarction. It is typically seen in children and young females. Our report presents a case of a 22-year-old female presenting to the ER with complaints of severe pain in the abdominal region. On ultrasound, there was suspicion of an adnexal mass, which was later confirmed to be a misplaced spleen in the lower abdomen, with torsion, fat stranding, and splenic vein thrombosis, as revealed by enhanced CT abdomen and pelvic MRI. It was followed by an emergency splenectomy. As wandering spleen presents nonspecifically and is a rare condition, it is important to consider wandering spleen when patients present similarly to this case, to prevent misdiagnosis and to deliver surgical treatment quickly to preserve the spleen.

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.

Metallic implant-related osteosarcoma.

Orthopedic metallic prostheses are commonly used in the current practice of orthopedic surgery. Although, biomaterials used in these implants are generally considered to be biologically inert, there have been consequences of foreign body reactions and potential carcinogenesis. Majority of implant-related malignancies are high grade, and develop in bone or soft tissue around the implant site. No clear association has been identified between the biomaterial implanted and the type of sarcoma. We report the case of a 36-year-old male who underwent intramedullary nailing of femur for mid-shaft fracture secondary to trauma and presented with osteogenic sarcoma of femur 14 months later.

Necrotizing Infection of the Breast: A Case Report on a Rare Presentation of Breast Carcinoma.

Necrotizing infection (NI) of the breast associated with underlying malignancy is a rare phenomenon characterized by necrosis of breast parenchyma, causing a delay in diagnosis and even leading to sepsis. We present a case of a 42-year-old female with NI of the right breast while on homeopathic treatment for a right breast lump for six months. Tissue culture showed a polymicrobial infection and histopathology established the diagnosis of breast carcinoma. After treating the NI, her breast cancer was managed as per standard guidelines.

Aspirated Denture: A Rare Cause of Empyema in an Asian Male.

Here, we report the case of an undiagnosed foreign body aspiration (FBA) in a 50-year-old male who presented with fever, productive cough, and shortness of breath suggestive of pneumonia. The patient reported a history of empyema for which he underwent left-sided video-assisted thoracoscopic surgery and decortication at another facility. Careful evaluation of prior chest imaging revealed a radio-opaque linear density projecting along the left of his spine suspicious for a foreign body in the airway which was missed on radiographic evaluation at the time of empyema. On flexible fiberoptic bronchoscopy, an irretrievable foreign body was visualized in the patient's left mainstem bronchus, which was removed via rigid bronchoscopy. This is a rare case of an aspirated denture manifesting as empyema and subsequently as pneumonia. We suggest that in patients with a recurrent chest infection, the possibility of FBA must be ruled out by detailed history and careful evaluation of imaging.

Mammographic Breast Density in Pakistani Women, Factors Affecting It, and Inter-Observer Variability in Assessment.

Introduction Breast density on mammography can affect the sensitivity of breast cancer detection and is an independent risk factor for breast cancer. The incidence of breast cancer in Pakistani women is reported to be the highest among women in Asia. No published data is describing the patterns of mammographic breast density in this population. We undertook this study to assess the Breast Imaging Reporting and Data System (BI-RADS) patterns of breast density on mammography, factors that affect breast density, and inter-observer variability in breast density assessment. Methods Bilateral breast mammograms were retrospectively reviewed for breast density by two separate readers (resident and attending radiologist). Breast density was categorized into four types according to the BI-RADS lexicon. Types 1 and 2 were grouped into non-dense and types 3 and 4 into dense breasts. The association of patient factors with breast density was assessed, with p < 0.05 considered statistically significant. The inter-observer variability in breast density assessment between the two readers was calculated using Cohen's κ coefficient. Results A total of 612 women underwent mammography in the study period. Type 3 (heterogeneously dense breast parenchyma) was the most frequent pattern (51.6%) followed by type 2 (scattered fibroglandular) pattern (38.9%). Fatty parenchyma (type 1) and extremely dense parenchyma (type 4) were the least common. Breast density was inversely related to age (p < 0.001) and parity (p <0.002). Breast density was also lower in postmenopausal women (p < 0.001). There was no statistically significant difference in mean age at menarche, age at first delivery, family history of breast cancer, or presence of cancer among women with dense and non-dense breasts. The inter-observer agreement was almost perfect (κ = 0.86).  Conclusion The majority of women in our population (56.9%) had dense breasts (BI-RADS type 3 and 4) which decrease the sensitivity of breast cancer detection on mammography suggesting it may be insufficient as the sole screening/diagnostic tool in this population. Lower breast density was associated with increasing age, parity, and post-menopausal status. Breast density assessment was almost perfect among the resident and attending radiologist.

Breast Imaging Chameleon: Pseudoangiomatous Stromal Hyperplasia Presenting as Breast Malignancy.

Pseudoangiomatous stromal hyperplasia (PASH) is a benign mesenchymal proliferative lesion of the breast, often an incidental finding on breast biopsy specimens and rarely presents as a palpable lump. The case being reported is interesting as a lactating female presented with gross left breast enlargement due to a huge firm mass with skin thickening and palpable left axillary lymph nodes. A provisional diagnosis of left breast malignancy was made and the patient extensively worked up with ultrasound, CT scan, bone scan and core biopsy. The histopathology, however, revealed PASH of the breast. There was no invasive or in situ malignancy. The patient was successfully managed conservatively.

Outcome of Non-Malignant Papillary Lesions of the Breast on Core Biopsy: An Experience from a Tertiary Care Center in Pakistan.

Background Papillary lesions of the breast constitute a heterogeneous group ranging from non-malignant papillomas to papillary carcinoma. While surgical excision is recommended for atypical papilloma or papillary DCIS/ carcinoma on core biopsy, controversy persists in the management of benign papillomas which are diagnosed with core needle biopsy (CNB) since there are variable reported rates for tumor upgrade. The purpose of this study was to determine the outcome of papillary lesions of the breast diagnosed at image-guided CNB, after surgical excision or follow-up, and to identify potential predictors of high-risk lesions/malignancy on imaging. Materials and methods We retrospectively identified 52 non-malignant papillary lesions on core biopsy between January 2012 and June 2018. The outcome of surgical excision, as well as clinical and imaging features of these lesions, were assessed. The final histologic upgrade was recorded, and variables were compared between benign and atypical lesions on core biopsy as well as between upgraded and non-upgraded lesions after surgical excision.  Results Thirty-six lesions out of 52 lesions were benign papillomas on core biopsy, while 16 were papillary lesions with ADH/DCIS. All of these lesions except four benign papillomas were excised. Of the 32 benign papillomas excised, 7 were upgraded to papilloma with ADH/DCIS and one to DCIS with the focus of invasion. Among the 16 atypical lesions excised, one was upgraded to papillary DCIS with a final upgrade rate of 17.3%. There was no statistically significant clinical or imaging feature among those that were upgraded on excision from those that were not upgraded.  Conclusion Non-malignant papillary lesions have a significant upgrade rate. There are no reliable clinical or imaging features that can pre-surgically predict upgrade. Therefore, surgical excision of all papillary lesions is recommended for definitive diagnosis.

Antenatal Ultrasound Diagnosis of Congenital High Airway Obstruction Syndrome: A Case Report and Review of Literature.

Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening fetal condition resulting from obstruction of the upper fetal airway which may be partial or complete. Prenatal diagnosis is crucial as it usually results in stillbirth or death after delivery if unrecognized. We report a case of CHAOS that was diagnosed prenatally due to characteristic ultrasound features. We also briefly review literature in light of current management options.

Tuberculosis of Male Breast: A Rare Benign Entity.

Granulomatous mastitis is a rare benign condition often seen in young lactating females of reproductive age group. Prepubescent males or elderly women may also be the victim of this infectious disease. Primary infection of the breast may occur through skin abrasions, open wounds or through the lactiferous ducts while secondary spread occurs from an infective focus elsewhere in the body via lymphatic or hematogenous routes. We present a case of breast tuberculosis diagnosed in a 62-year-old man at our institution. The patient presented with a palpable painful mass in the left breast with chronic sinus formation and pus discharge for a month with loss of appetite and weight loss. The etiology was unknown. The imaging features were suggestive of tuberculosis. Histopathology was concordant with imaging and showed chronic granulomatous inflammation with necrosis. The patient received oral anti-tuberculosis therapy for six months with no side effects or any further complications. Breast tuberculosis is a rare entity especially in male breast mimicking carcinoma. The mainstay of treatment is antitubercular therapy if imaging and histopathology confirms the diagnosis. Clinical awareness is necessary during diagnostic workup for establishing the correct diagnosis and treatment.

Intraabdominal Gossypibomas with variable CT appearance: A case report.

A retained surgical sponge or gossypiboma is a rare and an underreported complication occurring most commonly after abdominal surgeries. The clinical presentation as well as the time of presentation is variable with about one third of patients being asymptomatic. The diagnosis is challenging because of marked variation in the presentation and imaging plays a crucial role in diagnosis. We report a 30-year old Asian woman with prior history of Caesarean section who presented with persistent abdominal pain since surgery and underwent imaging in December 2012. The case is interesting as she had two intraabdominal gossypibomas with different appearances on computerized tomography. One was suggested to be a retained foreign body while the other was initially misinterpreted as a solid ovarian mass. However, on ultrasound, both lesions showed similar appearance and the left lower abdominal solid mass was also suggested to be a retained foreign body which was then confirmed on laparotomy.

Probably Benign Solid Breast Lesions on Ultrasound: Need for Biopsy Reassessed

Objective: To determine the negative predictive value of ultrasound for breast masses with probably benign morphology, and to assess whether follow-up may be an acceptable alternative to biopsy. Methods: After Institutional Review Board approval, all solid breast masses categorized as probably benign (American College of Radiology Breast Imaging Reporting and Data System [BI-RADS] 3) on ultrasound from January 2014 to December 2015, and having either tissue diagnosis or imaging stability for 24 months, or downgrading to BIRADS 2 during imaging surveillance were included. Result: A total of 157 lesions in 40 patients constituted the study population. The mean patient age was 31.3 years (range, 20-56 years). Seventeen of these 157 lesions underwent tissue diagnosis with no invasive breast cancer. Out of the remaining 140 lesions, 115 were stable on imaging for 24 months or more. The rest 25 were deemed benign because of decrease in size on follow up (n=1), non-recommendation of further imaging by the second radiologist on follow up ultrasound (n= 13) or presence of benign tissue diagnosis in the largest lesion (n=11). Conclusion: Ultrasound has 100% negative predictive value for breast lesions with probably benign morphology, whether palpable or not. Follow up is an appropriate option to immediate biopsy of such lesions keeping in mind that noncompliance with surveillance may be a potential problem.

Scar endometriosis: an entity not to be forgotten.

Scar endometriosis is an uncommon but well-described condition. It is caused by the dissemination of endometrial tissue in the wound at the time of surgery. The deposits can involve uterine scar, abdominal musculature or subcutaneous tissue, with the latter being the most common. It usually presents as a palpable mass at the scar site with or without cyclical pain. We report three cases of scar endometriosis which presented with cyclical pain and swelling at the abdominal wall scar following uterine surgery. The patients underwent imaging which revealed abnormal findings at the scar site suggesting scar endometriosis. In the presence of strong clinical suspicion and supportive imaging, all three of them underwent local excision of the lesion. The diagnosis of endometriosis was confirmed on histopathology.

Case series and literature review of multiple nodular sarcoidosis.

BACKGROUND: Nodular lung disease is a rare presentation of sarcoidosis. Radiologically it can present as multiple pulmonary masses or solitary lung nodule. CASE PRESENTATION: We report three cases of nodular sarcoidosis in young females of Asian origin who had initially presented with dry cough and worsening dyspnea non-responsive to initially administered antibiotics. Pulmonary nodules were discovered upon radiographic imaging in all three cases which raised concern for the possibility of neoplastic processes. Subsequent biopsies revealed granulomatous inflammation indicative of sarcoidosis. All cases responded very well to systemic corticosteroids. CONCLUSION: Sarcoidosis may present as nodular infiltrates which alerts the treating physician to other neoplastic and infectious diseases of the lungs. Appropriate workup may reveal the true nature of this disease and hence, simplify treatment.

Osseous spinal sarcoidosis: an unusual but important entity to remember.

Sarcoidosis is a multi-system disorder of unknown aetiology characterised by non-caseating granulomatous inflammation with varying presentation and prognosis. Bone involvement is uncommon and vertebral involvement is rare. We report a case of vertebral osseous sarcoidosis which presented with pulmonary symptoms mimicking tuberculosis and later developed vertebral involvement despite administration of oral corticosteroids.

Clinical characteristics, management and outcome of major pulmonary embolism: an experience from a tertiary care center in Pakistan.

OBJECTIVE: To evaluate the clinical characteristics, risk factors, management and outcome of major pulmonary embolism (PE) in a tertiary care center of Karachi. METHODS: Medical records of all patients who underwent a spiral CT scan of the chest for suspected pulmonary embolism were reviewed between January 2000 and June 2007 at the Aga Khan University Hospital, Karachi. Patients having evidence of major pulmonary embolism on spiral CT scan were selected. RESULTS: A total of 30 patients (10 males, 20 females) with mean age 52 +/- 14.59 years were identified who fulfilled our predefined criteria for major pulmonary embolism. Risk factors for thromboembolism were identified in 22 (73%) patients, prolonged immobilization in 8 (27%) and recent surgery in 8 (27%) patients being the commonest. All patients were symptomatic on presentation. Tachypnea and tachycardia were present in 27 (90%) patients. Refractory hypoxia was present in 18 (60%) patients and 3 (10%) were hypotensive on presentation. On spiral CT scan, 8 (27%) patients had embolus in the main pulmonary trunk, 26 (87%) patients in main right pulmonary artery and 20 (67%) patients had left main pulmonary artery embolus. Echocardiography was done in 22 (73%) patients with the findings of right ventricular dysfunction in all of them. All patients except one were treated with anticoagulation with either heparin infusion or low molecular weight heparin. In addition, thrombolytics were given in 7 (23%) patients and five (17%) underwent surgical embolectomy. Four (13%) patients died during hospitalization with a total of 26 (87%) surviving till hospital discharge. CONCLUSION: Major pulmonary embolism is an uncommon but potentially life threatening entity. Early diagnosis and aggressive therapy improves the clinical outcome.