Freehand correction of scoliosis in Rett’s syndrome

We present the case of an 18-year-old girl with a scoliosis (long C-shaped curve) in association with an atypical Rett’s syndrome. In order to attain a full correction and to provide her with adequate posture and sitting balance, segmental instrumentation was utilised from the high thoracic spine to the pelvis. We describe the procedure including the relevant pre-operative, intra-operative and post-operative imaging.     

Lisfranc injury: how frequently does it get missed? And how can we improve?

Lisfranc injuries are sometimes easily missed especially if they present in a subtle form. In this study, we have tried to measure the level of accuracy of plain radiographs in detecting this type of injury. We have selected 30 sets of foot radiographs with various diagnoses. Eighteen of them had a Lisfranc type of injury which was confirmed by operative findings or further imaging. These radiographs, together with a small diagnostic questionnaire, were presented to nine senior clinicians. We found that only 11 of the 18 cases (61%) were detected by all the readers. Eight of nine readers (90%) missed one particular case of subtle Lisfranc injury in a diabetic neuropathic foot. One case of a divergent injury was missed by five readers (56%). Another case with a coexistent Frieberg's disease was missed by three readers (33%). The mean accuracy for all injuries was 87% (95% confidence interval (CI) 84.6-89.9%). The mean accuracy level for detection of Lisfranc injury among the whole series was 92% (CI 89-95%). We think that the presence of a strong clinical index of suspicion or the presence of impaired pain sensation, further imaging is a must to avoid missing this type of injury at early presentation, and consequently the long term poor prognosis.     

Intercellular adhesion molecules in systemic lupus erythematosus patients with lupus nephritis

Cardiovascular events are markedly increased in systemic lupus erythematosus (SLE), and the mechanism of atherogenesis remains poorly understood. Several methods have been employed to assess endothelial function, among these is the measurement of biomarkers of endothelial activation and dysfunction [intercellular adhesion molecule (ICAM-1)]. It has been reported that such biomarkers play a more important role than traditional risk factors in cardiovascular disease. The objectives of this study were to determine the level of ICAM-1 as markers of endothelial dysfunction in 40 Egyptian patients who have SLE with various degrees of activity and to investigate their relationship to disease activity. Sixty people (40 with SLE and 20 healthy as the control group) were the subject of this study; their clinical disease activity was scored according to the SLE disease activity index (SLEDAI), and serum sampling was obtained for ICAM-1 level assay. Renal biopsy was carried out and examined by light microscopy by a pathologist blinded to the clinical activity. The mean level of ICAM-1 was significantly higher in SLE patients with active disease (826.05 ± 367.1 Pg/ml) compared to those with inactive disease (441.33 ± 225.19 Pg/ml) and the healthy control volunteers (111.5 ± 17.36 Pg/ml). There was a positive correlation between serum ICAM-1 and SLEDAI (r = 0.66). A high concentration of soluble ICAM-1 in SLE patients with nephritis is reported in this paper. Our finding of increased concentrations of ICAM-1 in SLE patients with nephritis underlines the importance of inflammation and endothelial involvement in this disease, but their predictive value in the disease monitoring need to be further studied.     

Semisynthesis of new sarcophine derivatives with chemopreventive activity

The natural cembranolide sarcophine (3) and its lactone ring-opened analogue (10) were oxidized using selenium dioxide under different reaction temperatures to prepare hydroxylated derivatives. Nine new compounds were obtained, six of them targeted hydroxylated derivatives. The determination of regio- and stereochemistry as well as the mechanistic considerations on the selectivity observed in these reactions are discussed on the basis of 2D NMR and molecular modeling. In preliminary in vitro tests on inhibition of EBV-EA activation, compounds 10 and 12-15 have shown higher activity than the known chemopreventive agent sarcophytol A.     

The triad of seizures, hypertension, and neuroblastoma: the first described case

The combination of seizures, hypertensive encephalopathy, and neuroblastoma has not been described before. The authors report one case, which is not only of interest in its own right, but also emphasizes the importance of including blood pressure measurement in the clinical examination of children, especially when hypertension could be the cause of the symptoms.     

What is the definition of “conversion” in laparoscopic colorectal surgery?

Background

A web-based survey was conducted among colorectal surgeons who represented members of both SAGES and ASCRS to find out how they define conversion for laparoscopic colorectal surgery.

Methods

Questionnaires were designed based on MCQs, including three parts: surgeon information, different definitions for conversion, and four different clinical scenarios. Surgeons were asked to choose the best definition(s).

Results

325 (28.5%) of 1,140 surgeons, 28.5% responded; approximately half of them were part of private-based practices. Fifty-three percent had more than 10 years experience; 35.9% performed more than 50 laparoscopic colon cases per year, 12% performed more than 25 laparoscopic rectal cases per year, and 60% less than 10. The majority (68.4%) agreed that any incision made earlier than planned is conversion. Whereas 81.4% felt that incision >5 cm is not a conversion, only 53.4% considered incision >10 cm a conversion, and 37% did not. Neither extracorporeal vessel ligation (73.8%), bowel resection (81.2%), anastomosis (77%), or incision made for specimen retrieval (91.1%) was counted as conversion. In clinical case scenarios, 62% found an incision made to facilitate phlegmon dissection after laparoscopically mobilizing the left colon up to and around the splenic flexure to be laparoscopic-assisted. A 10-cm incision required for fistula take down after finishing laparoscopic dissection was defined as conversion (55.6%). A 10-cm incision made for the rectal dissection in rectopexy was described as conversion in 51% and laparoscopic-assisted in 48%. Increasing a 5–12-cm for specimen extraction, 49.3% was declared a laparoscopic-assisted case.

Conclusions

It was considered clear that any incision made earlier than planned a conversion, whereas extra corporeal vessel ligation, bowel resection and anastomosis were not. However, there seem to be many views of conversion regarding incision length, and some clinical situations that might influence outcome among different centers.     

Familial pseudarthrosis of the clavicle: does it need treatment?

The eighth family with multiple cases of congenital pseudarthrosis of the clavicle is described. This usually presents as a clavicular lump and is distinct from more common conditions such as birth fractures, craniocleidal dysostosis and neurofibromatosis. There has so far been no clear indication on whether familial pseudarthrosis of the clavicle should be treated operatively. We recommend that when a suspected case of congenital pseudarthrosis of the clavicle is diagnosed the parents and siblings be examined also. If other family members are affected, we advise that the treatment should be conservative.     

Chemopreventive effects of sarcophine-diol on skin tumor development in CD-1 mice

The objective of this study was to determine the chemopreventive effects of sarcophine-diol (SD) on 7,12-dimethylbenz(a)anthracene initiated and 12-O-tetradecanoylphorbol-13-acetate promoted skin tumor development in mice and its possible mechanisms of action. SD pretreatment significantly (P<0.05) decreased skin papilloma development during promotion phase. SD significantly (P<0.05) increased caspase-3 and decreased cyclooxygenase-2 during initiation phase or promotion phase. SD significantly (P<0.05) increased caspase-8 during promotion phase. SD resulted in a 95% reduction in 12-O-tetradecanoylphorbol-13-acetate-induced DNA synthesis. SD could be an effective chemopreventive agent for skin cancer by enhancing apoptosis and decreasing cell proliferation.     

An infant with localized vasoconstriction following topical propranolol exposure for infantile hemangioma

We describe a 3‐month‐old child with an infantile hemangioma on the forehead with a blanched macule provoked by topical treatment with propranolol. This observation demonstrates that topically applied (non‐selective) beta‐blockers may induce blanched macules at the site of application, a side effect due to peripheral vasoconstriction of blood vessels by non‐selective beta‐2 blockade. This side effect was linked due to overuse and was reversible. This case illustrates the importance of providing thorough instructions regarding topical propranolol application.