The Investigation and Management of Iliac Artery Endofibrosis: Lessons Learned from a Case Series.

OBJECTIVE/BACKGROUND: The objective was to summarise the lessons learned, and evolution in local practice over the last 7 years, in the investigation and surgical management of iliac artery endofibrosis. METHODS: This was a retrospective case series. A case note review of consecutive patients investigated for suspected iliac artery endofibrosis by a single surgeon, over a 7 year period, was undertaken. Included were cases of first presentation and those who had previously undergone intervention. RESULTS: Some 63 patients were referred with suspected endofibrosis in the period 2011-17, four of whom had previously undergone surgery for the condition. After investigation of both limbs, 50 symptomatic limbs in 46 patients had a confirmed diagnosis; amongst those 46 patients, iliac artery endofibrosis was found in a further six asymptomatic, contralateral limbs. Individuals were diagnosed at a median age of 36 years (range 18-52 years) and typically presented with thigh claudication, foot numbness, and limb weakness on exercise. The median delay to diagnosis was 3 years (range 0-14 years). Complete external iliac artery occlusion was a feature in three cases. Overall, 27 limbs in 25 patients underwent operative repair; a further five limbs in four patients underwent operative repair at other centres internationally. There were three post-operative superficial wound infections (11%) and one below knee deep vein thrombosis (4%). Symptoms resolved in 23 cases (85%) with a median follow up of 2.1 years (range 65 days-5.7 years). Of the four limbs developing recurrent symptoms, two had undergone surgery for an occluded external iliac artery. CONCLUSION: Surgical repair in the medium term appears effective in resolving symptoms in most patients. Further investigation is needed to establish the durability of surgery and to delineate the natural history of the disease.

Outcomes when congenital heart disease is diagnosed antenatally versus postnatally in the UK: a retrospective population-based study.

BACKGROUND: For major congenital heart disease, the benefits of antenatal diagnosis on some post-natal measures have been suggested. However, findings have been inconclusive and focus on short term outcome measures alone with little data from a UK population. Our aim is to describe differences in reported outcomes for patients born with isolated Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries in a UK population, following either antenatal or postnatal diagnosis. METHODS: Retrospective population-based study with case note review covering a 15 year period (1st January 1998 to 31st December 2012) in the British county of Leicestershire. Cases were identified from two local registers: the East Midlands and South Yorkshire Congenital Anomaly Register and a list of surgical patient held by the East Midlands Congenital Heart Centre. RESULTS: In total 52 cases of Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries were identified with 24 (46.2%) diagnosed antenatally. Maximum and minimum follow up was 181 and 16 months respectively. Median follow up was 83 months (IQR: 44-111). The risk of intubation in the postnatal period (OR: 4.64, 95% CI: 1.40 - 15.32) was greater in cases of Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries diagnosed after birth when compared to those diagnosed antenatally. There was a non-significant increase in the risk of metabolic acidosis in the postnatal period (OR: 12.5, 95% CI: 0.64 - 245.46). No differences in mortality or long-term outcomes were demonstrated between antenatally and postnatally diagnosed cohorts. CONCLUSIONS: These results confirm data from American and European populations that, for a British population, an antenatal diagnosis of a major congenital heart disease can have a favourable impact on some postnatal outcome measures. There appears to be no evidence that time of diagnosis impacts on long-term outcome measures.