BackgroundFibrodysplasia ossificans progressiva is an ultrarare autosomal dominant disorder and disabling syndrome characterized by postnatal progressive heterotopic ossification of the connective tissue and congenital malformation of the big toes. Fibrodysplasia ossificans progressiva has worldwide prevalence of about 1 in 2 million births. Nearly 90% of patients with fibrodysplasia ossificans progressiva are misdiagnosed and mismanaged and thus undergo unnecessarily interventions. So far, the number of reported existing cases worldwide is about 700. Clinical examination, radiological evaluation, and genetic analysis for mutation of the ACVR1 gene are considered confirmatory tools for early diagnosis of the disease. Association of fibrodysplasia ossificans progressiva with heterotopic ossification is well documented; however, postsurgical exaggerated response has never been reported previously, to the best of our knowledge.Case presentationWe report a case of a 10-year-old Pakistani boy brought by his parents to our institution. He had clinical and radiological features of fibrodysplasia ossificans progressive and presented with multiple painful lumps on his back due to hard masses and stiffness of his shoulders, neck, and left hip. He underwent surgical excision of left hip ossification followed by an exaggerated response in ossification with early disability. Radiological examination revealed widespread heterotopic ossification. All of his laboratory blood test results were normal.ConclusionFibrodysplasia ossificans progressiva is a very rare and disabling disorder that, if misdiagnosed, can lead to unnecessary surgical intervention and disastrous results of early disability. We need to spread knowledge to physicians and patients’ family members about the disease, as well as its features for early diagnosis and how to prevent flare-up of the disease to promote better quality of life in these patients.

BackgroundSplenic artery aneurysms are the commonest visceral and third most common abdominal artery aneurysms, having a strong association with both pregnancy and multiparity. Here we report possibly the first case of a giant splenic artery aneurysm in association with a smaller portal vein aneurysm, in a woman who had never conceived, leading to non-cirrhotic portal hypertension.Case presentationA 40-year-old Pakistani Asian woman who had no evidence of liver cirrhosis presented in April 2016 for a diagnostic workup of ascites, massive splenomegaly, and pancytopenia. An abdominal ultrasound followed by computed tomography angiography showed a giant aneurysm in her splenic artery and another smaller one in her portal vein.She underwent splenectomy and excision of the splenic artery aneurysm. Surgical findings included a giant splenic artery aneurysm pressing on her portal vein and causing its aneurysmal dilatation. On her first review in July 2016, she was generally in good health, ascites had subsided, and her full blood count was normal. Her portal vein aneurysmal dilatation, which was presumed to be secondary to the pressure effect from the splenic artery aneurysm, had shrunken remarkably in size.ConclusionA giant splenic artery aneurysm can cause non-cirrhotic portal hypertension and should be treated with splenectomy and aneurysmectomy.

A previously well, 45-year-old Pakistani lady was admitted to the medical unit on-call of Khyber Teaching Hospital (KTH) Peshawar with a 5-day history of fever, productive cough with copious mucoid sputum, dyspnea, and pleuritic chest pain. She also complained of dry eyes, mouth, and vagina. Her chest X-ray showed diffuse alveolar shadowing and arterial gas analysis confirmed type 1 respiratory failure. Over the next few days, she deteriorated rapidly making an urgent transfer to the medical intensive care unit (MICU) necessary, where she was mechanically ventilated. An HRCT followed by bronchoscopic biopsies made a diagnosis of acute interstitial pneumonitis (AIP), formerly known as Hamman-Rich syndrome. She also turned out to be positive for both anti-SS-A/Ro and anti-SS-B/La antibodies along with a positive Schirmer's test and lower lip biopsy. She received intravenous steroids and supportive care. The patient had a complete recovery after approximately three weeks' stay in the hospital with lung function returning back to normal. This is most probably the first ever case of primary Sjogren syndrome (pSjS) presenting as AIP, recovering completely in less than a month time.

Objective: To compare outcome of patients with supracondylar fractures of humerus treated by cross k wires and lateralentry k wires in children. Study Design: Randomized controlled trial study. Place and duration of study: January 2012 to December2012 at Department of Orthopedics, Nawaz Sharif Social Security hospital, Lahore. Subjects and Methods: Two hundred patients,meeting the selection criteria were identified. These patients were divided into group A and group B randomly. In group A cross k wirefixation and in group B lateral entry k wire fixation was performed. Loss of reduction was assessed and recorded in the immediatepostoperative period and three weeks later at the time of removal of k wires. Range of motion of the elbow, in the form of excellent outcomewas assessed at the end of 12 weeks postoperatively. Results: A total of 200 patients were included in the study. Loss of reduction wasfound in thirty patients (30%) in group A and in forty one patients (41%) in group B. In group A, seventy two patients (72%) had excellentoutcome while twenty eight patients (28%) did not gain the desired range of motion at elbow. In group B, sixty five patients (65%) hadexcellent outcome while in thirty five patients (35%), the desired range of motion at elbow was not achieved. No neurological injuryoccurred with both configurations. Conclusions: Lateral entry k wire fixation is as effective as cross k wire in the treatment of displacedsupracondyler fracture of humerus in children.