Focal nodular hyperplasia: a review of current indications for and outcomes of hepatic resection

Background

Focal nodular hyperplasia (FNH) is a common benign disease of the liver with no recognized potential for malignant transformation. The term describes an entity of lobular proliferation of normally differentiated hepatocytes, frequently around a central fibrous scar. Two key issues influence surgical decision making in FNH: diagnostic certainty, and symptomatic assessment.

Methods

A systematic review of studies reporting hepatic resections of FNH was performed. Indications and outcomes in adult populations were examined with a focus on diagnostic workup, patient selection and operative mortality and morbidity.

Results

Diagnostic modalities in the majority of studies involved ultrasound and computed tomography. Fewer than half employed magnetic resonance imaging (MRI). In instances in which MRI was not available, diagnostic accuracy was inferior.

Conclusions

Percutaneous biopsy should be avoided to prevent the risk for tumour seeding. Patients presenting with asymptomatic definitive FNH can be safely managed conservatively. In symptomatic patients surgical resection is a safe and effective treatment for which acceptable rates of morbidity (14%) and zero mortality are reported. However, evidence of symptom resolution is reported with conservative strategies. Diagnostic uncertainty remains the principal valid indication for FNH resection, but only in patients in whom contrast-enhanced MRI forms part of preoperative assessment.     

The risks of red cell transfusion for hip fracture surgery in the elderly

Background and Objectives The benefits and indications for blood transfusion among surgical patients are controversial. There is evidence which suggests that blood transfusion is associated with poor clinical outcomes and risks of infection, but there are few data in the elderly population. Materials and Methods Data were collected on haemoglobin concentrations and transfusions in 919 patients undergoing hip fracture repair at a university hospital over a 2‐year period. 28‐day and 180‐day mortality were specified as primary outcomes. A composite infection outcome (chest infections, urinary tract infections and wound infections) was the main secondary outcome. Preoperative, operative and/or postoperative transfusions were the main exposure variable. Regression analyses were used to explore the associations between transfusion and outcomes, adjusting for pre‐defined preoperative variables. Results 300 patients (32·6%) were transfused at least once during their admission. There was no evidence of a significant difference in either 28‐day survival or 180‐day survival between transfused and non‐transfused hip fracture patients. The transfused group had higher adjusted composite infection rate (HR, 1·91; 95% CI, 1·41–2·59, P < 0·001) and prolonged length of stay in hospital than the non‐transfused group (HR, 1·15; 95% CI, 1·07, 1·23, P < 0·001). Anaemia at the time of admission, extra capsular fracture and using walking aids in an indoor setting were preoperative variables, which predicted the need for transfusion. Conclusion Among an elderly population with hip fracture, blood transfusion was not associated with changes in mortality, but was associated with an increased rate of postoperative infection. These data add to the wider literature about adverse clinical outcomes in patients receiving blood transfusions and emphasises the need for prospective trials to evaluate the role of transfusion in the elderly.     

The use of music to engage children with autism in a receptive labelling task

Children with autism are less engaged with social and non-social objects and activities than their typically developing peers, resulting in reduced learning opportunities. There is some support for the use of music to enhance the engagement of children with autism but there has been little research investigating the use of music to engage children in language learning tasks and determining if this impacts on learning outcomes. This study investigated the use of music to engage children with autism in a receptive labelling intervention. Twenty two children (mean age 5.9 yrs) participated in a randomised controlled cross-over design comparing sung and spoken conditions embedded into a computer based intervention. Child performance and observational data were analysed to determine relationships between music, engagement and learning outcomes. The findings from this research showed children with autism were more engaged in the sung condition compared to the spoken condition although there was considerable variability in levels of engagement between participants. Furthermore, a correlation between engagement and learning was found. Implications of these findings are discussed and recommendations made for future research.     

MCPH1 deletion in a newborn with severe microcephaly and premature chromosome condensation

A newborn with severe microcephaly and a history of parental consanguinity was referred for cytogenetic analysis and subsequently for genetic evaluation. While a 46,XY karyotype was eventually obtained, premature chromosome condensation was observed. A head MRI confirmed primary microcephaly. This combination of features focused clinical interest on the MCPH1 gene and directed genetic testing by sequence analysis and duplication/deletion studies disclosed a homozygous deletion of exons 1–11 of the MCPH1 gene. This case illustrates a strength of standard cytogenetic evaluation in directing molecular testing to a single target gene in this disorder, allowing much more rapid diagnosis at a substantial cost savings for this family.     

A case of an atypically large proximal 15q deletion as cause for Prader–Willi syndrome arising from a de novo unbalanced translocation

We describe an 11 month old female with Prader–Willi syndrome (PWS) resulting from an atypically large deletion of proximal 15q due to a de novo 3;15 unbalanced translocation. The 10.6 Mb deletion extends from the chromosome 15 short arm and is not situated in a region previously reported as a common distal breakpoint for unbalanced translocations. There was no deletion of the reciprocal chromosome 3q subtelomeric region detected by either chromosomal microarray or FISH. The patient has hypotonia, failure to thrive, and typical dysmorphic facial features for PWS. The patient also has profound global developmental delay consistent with an expanded, more severe, phenotype.     

Toward magnetic resonance-guided electroanatomical voltage mapping for catheter ablation of scar-related ventricular tachycardia: a comparison of registration methods

Toward MR‐Guided Electroanatomical Voltage Mapping for Catheter Ablation. Introduction: Integration of preprocedural delayed enhanced magnetic resonance imaging (DE‐MRI) with electroanatomical voltage mapping (EAVM) may provide additional high‐resolution substrate information for catheter ablation of scar‐related ventricular tachycardias (VT). Accurate and fast image integration of DE‐MRI with EAVM is desirable for MR‐guided ablation. Methods and Results: Twenty‐six VT patients with large transmural scar underwent catheter ablation and preprocedural DE‐MRI. With different registration models and EAVM input, 3 image integration methods were evaluated and compared to the commercial registration module CartoMerge. The performance was evaluated both in terms of distance measure that describes surface matching, and correlation measure that describes actual scar correspondence. Compared to CartoMerge, the method that uses the translation‐and‐rotation model and high‐density EAVM input resulted in a registration error of 4.32±0.69 mm as compared to 4.84 ± 1.07 (P <0.05); the method that uses the translation model and high‐density EAVM input resulted in a registration error of 4.60 ± 0.65 mm (P = NS); and the method that uses the translation model and a single anatomical landmark input resulted in a registration error of 6.58 ± 1.63 mm (P < 0.05). No significant difference in scar correlation was observed between all 3 methods and CartoMerge (P = NS). Conclusions: During VT ablation procedures, accurate integration of EAVM and DE‐MRI can be achieved using a translation registration model and a single anatomical landmark. This model allows for image integration in minimal mapping time and is likely to reduce fluoroscopy time and increase procedure efficacy. (J Cardiovasc Electrophysiol, Vol. 23, pp. 74‐80, January 2012)