Giant Left Atrium as Cause of Left Pulmonary Artery Obstruction

A case of a giant left atrium with compression of the left pulmonary artery and left main bronchus is described. The surgical approach was through a left thoracotomy, and the correction included mitral valve replacement, reduction atrioplasty, and dissection and release of the left pulmonary artery.     

Hepatic Hydatid Cyst: A Rare Cause of Recurrent Pancreatitis

A case of pancreatitis secondary to a hepatic hydatid cyst is illustrated together with its preoperative imaging and intraoperative appearance. Cystobiliary communication is a common complication of large hydatid cysts, and episodes of recurrent pancreatitis resulting from passage of cyst contents down the biliary tract are rarely described. The clinical manifestations, diagnostic workup, and surgical management options of echinococcal-related pancreatitis are discussed, and a review of the literature is provided.     

A Rare Cause of Chronic Abdominal Pain: Recurrent Sub-torsions of an Accessory Spleen

Accessory spleen is defined as one, two, or three nodules of additional ectopic splenic parenchyma hung by a vascular pedicle generally near the spleen. Despite a relatively high frequency (from 10 to 30 % of the population based on autopsy studies), most accessory spleens are asymptomatic. Although cases of accessory spleen were clearly described in the literature, this perplexing diagnosis is often delayed and rarely made preoperatively. We repot episodic recurrences of abdominal pain in a 66-year-old man attributed to iterative sub-torsions of an accessory spleen, as well as a comprehensive review of the literature.     

Abdominal Cocoon Syndrome: a Rare Cause for Recurrent Abdominal Pain

Abdominal cocoon syndrome, or sclerosing peritonitis, is a rare condition characterized by encasement of small bowel loops by a thick fibrous scar. It most commonly presents as nonspecific vague chronic abdominal pain and weight loss, and is difficult to recognize clinically until the patient develops symptoms of bowel obstruction. We present a case of abdominal cocoon syndrome in a 65-year-old female and describe its clinical, imaging, and pathologic features.     

Osteogenic Sarcoma of the Left Atrium

A primary cardiac osteogenic sarcoma is described in a patient presenting with a left atrial obstructive lesion. Wide surgical excision was possible with left atrial reconstruction and mitral valve replacement. The patient survived the operation and was symptom free for 1 year, finally dying at 18 months of cerebral metastases.     

Bronchogenic Cysts: A Rare Congenital Cystic Malformation of the Lung

Purpose Bronchogenic cysts are rare congenital cystic malformations of the lung. We retrospectively analyzed ten cases of bronchogenic cyst (BC) to reinforce the importance of recognizing this malformation.Methods Between 1985 and 2000, ten pediatric patients with BC were treated surgically in our department. Their clinical presentation, radiological, operative, and pathological findings were analyzed retrospectively.Results There were five boys and five girls, ranging in age from 16 days to 6 years (mean 6.5 months). The clinical signs and symptoms included respiratory distress in seven patients (70%), cyanosis in four (40%), chronic cough and fever in five (50%), and dysphasia in two (20%). Routine chest X-ray revealed a pulmonary air-filled cyst in six patients (60%) and a pulmonary nodular opacity in four (40%). The diagnosis was supported by computed tomography in four patients and by ultrasonography in two. Eight of the patients were treated by cyst excision and two by lobectomy. The pathological diagnosis made from all specimens was bronchogenic cyst, with squamous metaplasia in two.Conclusion In newborns, infants, and even children, the development of dyspnea, cyanosis, chronic cough, and fever should alert us to the suspicion of a cystic malformation in the lung, such as a bronchogenic cyst, especially if an air-filled cyst is seen on a plain chest X-ray.     

Urethral Venous Malformation: An Unusual Cause of Recurrent Post-Coital Gross Haematuria in Association with Haematospermia

Three cases of recurrent post-coital haematuria are described. Extensive protracted investigations pinpointed urethral varicosities as the likely cause. All patients were successfully treated with diathermy fulguration.     

Recurrent 2,8‐Dihydroxyadenine Nephropathy: A Rare but Preventable Cause of Renal Allograft Failure

Adenine phosphoribosyltransferase (APRT) deficiency is a rare autosomal recessive enzyme defect of purine metabolism that usually manifests as 2,8‐dihydroxyadenine (2,8‐DHA) nephrolithiasis and more rarely chronic kidney disease. The disease is most often misdiagnosed and can recur in the renal allograft. We analyzed nine patients with recurrent 2,8‐DHA crystalline nephropathy, in all of whom the diagnosis had been missed prior to renal transplantation. The diagnosis was established at a median of 5 (range 1.5–312) weeks following the transplant procedure. Patients had delayed graft function (n = 2), acute‐on‐chronic (n = 5) or acute (n = 1) allograft dysfunction, whereas one patient had normal graft function at the time of diagnosis. Analysis of allograft biopsies showed birefringent 2,8‐DHA crystals in renal tubular lumens, within tubular epithelial cells and interstitium. Fourier transformed infrared microscopy confirmed the diagnosis in all cases, which was further supported by 2,8‐DHA crystalluria, undetectable erythrocyte APRT enzyme activity, and genetic testing. With allopurinol therapy, the allograft function improved (n = 7), remained stable (n = 1) or worsened (n = 1). At last follow‐up, two patients had experienced allograft loss and five had persistent chronic allograft dysfunction. 2,8‐DHA nephropathy is a rare but underdiagnosed and preventable disorder that can recur in the renal allograft and may lead to allograft loss.