General Surgery in Patients With a Bleeding Diathesis: How We Do It

Background  

This study was undertaken to assess perioperative management, postoperative complications, and the adequacy of perioperative plasma factor levels in a regional hemophilia center.     

Outcome of hepaticojejunostomy without access loop for repair of iatrogenic bile duct injury at laparoscopic cholecystectomy

Background Purpose Roux-en-Y hepaticojejunostomy is the accepted treatment for transectional biliary injury at cholecystectomy. Many authors advocate leaving a long redundant jejunal access loop to facilitate subsequent access. Reasoning that percutaneous access can be achieved transhepatically in patients with stenosis, this study reports the outcome of a policy of biliary repair without the use of a jejunal access loop. Methods Eleven patients undergoing biliary reconstruction over a 5-year period constituted the study population. Three (27%) were male, and the median (range) age at injury was 53 (26–75) years. Median delay from injury to repair was 2 (1–48) months. Bismuth stage was: stage I, 4; stage II, 5; and stage III, 2. Four patients had concomitant arterial injury. All underwent surgical repair by Roux-en-Y hepaticojejunostomy without an access loop. Results The median follow-up was 13 (1–64) months. The principal postoperative complication was a hepatic abscess in one patient. There was one death during follow-up, from acute myeloid leukemia. One patient (9%) with a type III injury presented with a symptomatic recurrent biliary stricture 6 months after repair, and was successfully managed by percutaneous biliary dilatation, using a combination of transhepatic and jejunal loop puncture. Conclusions Successful biliary reconstruction can be performed without a routine jejunal access loop.     

Contemporary management of infected necrosis complicating severe acute pancreatitis

Pancreatic necrosis complicating severe acute pancreatitis is a challenging scenario in contemporary critical care practice; it requires multidisciplinary care in a setting where there is a relatively limited evidence base to support decision making. This commentary provides a concise overview of current management of patients with infected necrosis, focusing on detection, the role of pharmacologic intervention, and the timing and nature of surgical interventions. Fine-needle aspiration of necrosis remains the mainstay for establishment of infection. Pharmacological intervention includes antibiotic therapy as an adjunct to surgical debridement/drainage and, more recently, drotrecogin alfa. Specific concerns remain regarding the suitability of drotrecogin alfa in this setting. Early surgical intervention is unhelpful; surgery is indicated when there is strong evidence for infection of necrotic tissue, with the current trend being toward 'less drastic' surgical interventions.     

Sudden infant death in a patient with FGFR3 P250R mutation

P250R mutation in the FGFR3 gene also known as Muenke syndrome is associated with coronal craniosynostosis, sensorineural deafness, craniofacial, and digital abnormalities. We report a family with this mutation associated with sudden death in an affected newborn, most probably due to upper airway obstruction.     

Relative frequency of odontogenic tumors in Sri Lanka: Analysis of 1677 cases

Odontogenic tumors (OTs) constitute a heterogeneous group of lesions with diverse histopathological features and clinical manifestations. The present study is to determine the frequency of odontogenic tumors (OTs) in Sri Lankan population. A total of 1677 cases of OTs were retrieved and analyzed for age, gender and primary site of the tumors. Cases were re-classified according to the 2005 WHO classification of OTs. The relative frequency of different types of tumors was also analyzed and compared with the literature. OTs represent 3.75% of all cases received during a period of 30 years. Ninety-eight percent of these tumors were benign and the rest malignant. Mandible to maxilla ratio is 2.8:1. The posterior part, the molar region, is the most frequently affected site for the mandible whilst it is the anterior region for the maxilla. The age ranges from 1 to 80 years, with a mean age of 30.6 years. Ameloblastoma of solid/multicystic and unicystic types showed a high preponderance for the mandible (>90%) with a ratio of 12.9:1 and 10.8:1, respectively. Out of 1677 cases, 48.7% were ameloblastoma, and other tumors, such as keratocystic odontogenic tumor (KCOT) and odontoma, were 25.7% and 10.1%, respectively. There is a significant change in the frequency of OTs after the inclusion of odontogenic keratocyst as a tumor. Although odontoma is said to be the commonest in western countries, our results showed ameloblastoma as the commonest followed by KCOT, and the relative frequencies of different tumors have changed significantly as a result of inclusion of KCOT in the new classification.     

Arginase I deficiency: severe infantile presentation with hyperammonemia: more common than reported?

Enzyme defects of the urea cycle typically present with significant hyperammonemia and its associated toxicity, in the first few months of life. However, arginase I (ARG1) deficiency, a rare autosomal recessive disorder, has classically been the exception. ARG1 deficiency usually presents later in life with spasticity, seizures, failure to thrive and developmental regression. Neonatal and early infantile presentation of ARG1 deficiency with severe hyperammonemia remains rare and only six such cases have been described. We report a severely affected infant with ARG1 deficiency who presented at 6 weeks of age with lethargy, poor feeding and severe encephalopathy caused by hyperammonemia. The clinical and biochemical features of the proband and six other previously reported cases with neonatal or infantile-onset presentation of ARG1 deficiency with hyperammonemia are reviewed. In addition, the clinical spectrum of seven previously unpublished patients with later onset ARG1 deficiency, who also experienced recurrent hyperammonemia, is presented. Several biochemical abnormalities have been postulated to play a role in the pathogenesis of the neurological changes in ARG1 deficiency including hyperargininemia, elevated guanidino compounds and elevated glutamine levels, as well as the hyperammonemia. The index case demonstrated many of these. The cases reviewed here suggest a genotype/phenotype correlation and advocate for the addition of arginine as a primary target in newborn screening programs.