Iniencephaly and long-term survival: a rare case report

Introduction Iniencephaly, a neural tube defect involving occiput and inion and combined with rachischisis of the cervical, thoracic spine, and retroflexion position of the head is a very rare congenital abnormality of the fetus–newborn with a 0.1–10 of 10,000 prevalence. This abnormality’s prognosis is thought to be dismal. This abnormality can be associated with other abnormalities such as anencephaly, encephalocele, hydrocephalus, cyclopia, absence of the mandible, cleft lip and palate, cardiovascular disorders, diaphragmatic hernia, renal abnormalities, overgrowth of the arms compared to the legs, and club food and gastrointestinal atresia. Discussion Most of the patients are dead born, and the others die in a few hours. There are only six previously documented long-term survivors. In our case, our patient with iniencephalic signs and findings is still living. She is 2 years old now. We think that this patient presents a mild form of iniencephaly.     

Comparative early and midterm results of open juxtarenal and infrarenal aneurysm repair

Background and aims Since the introduction of endovascular aortic aneurysm repair (EVAR) for aortic aneurysms, the number of juxtarenal aortic aneurysms (JRA) has been growing steadily due to selection bias (neck morphology for EVAR). This case-match study compares the perioperative outcome and midterm results of suprarenally clamped JRA with infrarenal aortic aneurysms (AAA). Methods From 1997 to 2004, patients who received open surgery with suprarenal clamping for JRA were included in the study and compared to matched patients with infrarenal clamping (AAA). Measurements analyzed were the in-hospital mortality and morbidity. Midterm results were obtained through clinical investigation and magnetic resonance angiography imaging. Results Thirty-five patients (mean age, 68.4 years; 30 male and 5 female) received suprarenal cross-clamping for JRA. The overall in-hospital mortality for JRA and for the controls (AAA) with elective aortic repair was 4.5% (6.1% JRA; 3% AAA, p = 0.058). The morbidity of JRA was elevated according to the rate of pulmonary complications (p = 0.021) and the need for re-operation (p = 0.019). The mean follow-up time was 2.3 years (range, 8–96 months). At follow-up, 28 patients (80%) from the JRA group and 29 patients from the AAA group (82.9%) were alive. Conclusion Open aortic surgery for JRA with the need for suprarenal cross-clamping shows a slightly elevated in-hospital mortality rate without statistical significance and equal midterm mortality results in comparison with infrarenally clamped aortic aneurysms.     

Individualized Mycophenolate Mofetil Dosing Based on Drug Exposure Significantly Improves Patient Outcomes After Renal Transplantation

Efficacy and safety of mycophenolate mofetil (MMF) may be optimized with individualized doses based on therapeutic monitoring of its active metabolite, mycophenolic acid (MPA). In this 12-month study, 137 renal allograft recipients from 11 French centers receiving basiliximab, cyclosporine A, MMF and corticosteroids were randomized to receive either concentration-controlled doses or fixed-dose MMF. A novel Bayesian estimator of MPA AUC based on three-point sampling was used to individualize doses on posttransplant days 7 and 14 and months 1, 3 and 6. The primary endpoint was treatment failure (death, graft loss, acute rejection and MMF discontinuation). Data from 65 patients/group were analyzed. At month 12, the concentration-controlled group had fewer treatment failures (p = 0.03) and acute rejection episodes (p = 0.01) with no differences in adverse event frequency. The MMF dose was higher in the concentration-controlled group at day 14 (p < 0.0001), month 1 (p < 0.0001) and month 3 (p < 0.01), as were median AUCs on day 14 (33.7 vs. 27.1 mg*h/L; p = 0.0001) and at month 1 (45.0 vs. 30.9 mg*h/L; p < 0.0001). Therapeutic MPA monitoring using a limited sampling strategy can reduce the risk of treatment failure and acute rejection in renal allograft recipients 12 months posttransplant with no increase in adverse events.     

Endovascular treatment (EVT) of acute traumatic lesions of the descending thoracic aorta--7 years' experience.

OBJECTIVES: To present a single centers' 7-year experience in the endovascular treatment of acute traumatic lesions of the descending thoracic aorta (ATL of the DTA). MATERIALS & METHODS: Between March 1999 and December 2006, 34 consecutive acute traumatic lesions of the descending aorta (23 men, mean age 44 years) were treated endovascularly. Stentgrafts used were TAG Excluder, Zenith TX2 and Talent. In 23 patients the Left Subclavian Artery (LSA) was covered. Mean procedural duration was 20 to 75 minutes. RESULTS: Exclusion of the rupture site was achieved in all cases with no conversion to open surgery. Overall 30-day mortality was 8.8%. Two patients died on post operative day (pod) 1 and one on pod 22 from cranial injuries. No death or neurological deficit related to the endovascular treatment was reported. Four type I endoleaks required treatment either by balloon reexpansion (n=2) or by additional stentgraft implantation (n=2). In two patients the stentgraft collapsed totally several days postoperatively. Two patients required secondary surgical procedures (iliac access complication and revascularisation of the left subclavian artery n=1). The average follow-up was 43.8 months (1-93 months). No stentgraft related abnormality has been subsequently documented. CONCLUSIONS: The endovascular treatment of ATL of the DTA may offer the best means of therapy in a polytrauma patient.     

Larynxtubus beim Traumapatienten

A multiply injured patient’s airway is primarily secured with a laryngeal tube by the responding EMS. For helicopter transfer the flight physician decides not to intubate the patient’s trachea. Mechanical ventilation was performed without complications.     

Perforation of a retrocaecal appendix presenting clinically as a right lumbar abscess.

Presentations of acute appendicitis represent an ongoing challenge to surgeons. This case of retrocaecal perforated appendicitis presented clinically as a right lumbar abscess, with the concomitant new diagnosis of type 2 diabetes mellitus in a 66-year-old male. This case emphasises the importance of an abdominal CT scan in any patient presenting with unusual sepsis near the abdomen.