Larsen syndrome and its anaesthetic considerations

Larsen syndrome is a complex syndrome with genetic heterogeneity, and with both autosomal dominant and autosomal recessive patterns of inheritance. It is characterized by congenital dislocation of joints, flat faces and complicated by issues relating to respiratory, cardiac, musculoskeletal and central nervous systems. This report describes the anaesthetic management of two patients with Larsen syndrome. The first case is a 4-year-old patient who had cervical cord compression secondary to cervical instability and who was scheduled for anterior corpectomy with fusion of cervical vertebrae and placement of halo frame. This patient had transient loss of evoked potentials during positioning and a stormy postoperative course requiring reintubation and a prolonged stay in the intensive care unit. The second case is a 22-month-old child who was scheduled for a repeat posterior cervical spinal fusion due to failure of her initial fusion procedure. This patient had an uneventful perioperative course. Relevant anaesthetic issues in patients with Larsen syndrome are discussed.     

Range of motion and function are similar in patients undergoing TKA with posterior stabilised and high-flexion inserts

It is debatable whether high-flexion (HF) total knee arthroplasty (TKA) designs will improve postoperative flexion and function or will diminish the need for manipulation under anaesthesia (MUA). We retrospectively analysed range of motion (ROM), flexion, Knee Society Score (KSS), and rate of MUA in a consecutive group of patients who underwent TKA with a conventional posterior stabilised (PS) insert or an HF insert using identical surgical technique, implant design, and postoperative care. Fifty TKAs with a standard PS insert were matched for patient’s age, gender, preoperative ROM, and KSS with 50 TKA performed with an HF insert. The patient’s ROM and KSS were evaluated at six weeks, four months, and one year postoperatively. The outcome variables (flexion, ROM, KSS, and manipulation rate) in both groups were compared using the generalised estimating equations method. A second analysis of patients with preoperative flexion ≥120° was performed. The ROM, flexion, and patient-reported KSS was similar in both groups at each time period. The rate of MUA was also similar. Patients with a preoperative ROM of at least 120° showed similar results. Our study found that one year after surgery, patients who underwent TKA with a PS or an HF insert achieved similar flexion, ROM, and function.     

Patients’ preferred mode of travel to the orthopaedic theatre

AIM: To determine the preferred mode of travel to the operating theatre for elective orthopaedic patients.METHODS: Data was collected prospectively over a 2-wk period at an elective Orthopaedic Treatment Centre. Patients were asked to complete a patient satisfaction questionnaire following their surgery on their experience and subsequent preferred mode of transport to theatre. The data was then recorded in a tabulated format and analysed with percentages. Fisher’s exact test was used to determine if there was any statistical association between patients’ preference to walk and various groups; in-patient or day case procedures, and whether patients were < 60 years or > 60 years of age.RESULTS: Seventy patients (40 females and 30 males) fully completed the questionnaire. In total there were 33 d-cases and 37 in-patients. The spectrum of orthopaedic sub-specialties included was knee (41%), hip (17%), foot and ankle (24%), spine (13%) and upper limb (4%). Patient satisfaction for overall experience of travelling to theatre was either excellent (77%) or good (23%). Following their experience of travelling to theatre, 87% (95%CI: 79%-95%) of the total cohort would have preferred to walk to the operating theatre. There was a statistically significant association (P = 0.003) between patients’ preference to walk and whether they were day-case or in-patients. Similarly, there was a statistically significance association (P = 0.028) between patients’ preference to walk and whether they were < 60 years or > 60 years of age.CONCLUSION: This study confirms the majority of Orthopaedic elective patients would prefer to walk to theatre, when given the choice and if practically possible.     

Outcome of laparoscopic nephron sparing surgery using a Satinsky clamp for hilar control: a trusted tool (SKIMS experience)

Background:Nephron sparing surgery is a well-established surgical procedure for patients with small/bilateral renal masses. During the procedure, hilar control can be achieved by using bulldog clamps individually on the renal vessels, the renal artery alone without clamping the vein, or a laparoscopic Satinsky clamp for en bloc hilar clamping. In our series, we described the outcome of laparoscopic nephron sparing surgery using a Satinsky clamp for hilar control.Materials and methods:All eligible cases with confirmed diagnosis of a renal mass were advised of nephron sparing surgery. The short-term outcomes were evaluated by warm ischemia time (using a Satinsky clamp especially when CT renal angiography was not available), average blood loss, and length of postoperative hospital stay. The oncological outcome was evaluated by noting the surgical margins of histopathological specimen, local recurrence, and distant metastasis.Results:Of 30 cases 20 were male. The mean age was 54.25 years. On preoperative evaluation, 24 cases were T1a stage and the rest were 6 T1b stage. Four tumors were located in the upper pole, 4 in the posterior midpole, and 22 in the lower pole. Twenty-six patients had a low complexity score on RENAL scoring (ie, 4–6) and 4 patients a medium complexity score (ie, 7–9). Three patients were converted to open partial nephrectomy because of technical difficulty in intracorporeal suturing and difficulty in achieving hemostasis. Among these 3 patients, 2 patients had posterior base tumors and 1 had a lower polar tumor. Average blood loss was 350 ml, warm ischemia time was 28.46 minutes, and postoperative stay was 4.55 days. Of 30 specimens for histopathology, 23 (76%) were clear cell renal cell carcinoma (RCC), 4 (13%) were papillary RCC, 1 (3.3%) was chromophobe RCC, whereas 2 (6.6%) were benign (oncocytoma). Margins were free of tumors in all the patients with no recurrence in 2 years of follow-up.Conclusion:Laparoscopic partial nephrectomy by using a Satinsky clamp as a tool for en bloc hilar clamping in the proper axis at the hilum takes care of multiple vessels irrespective of size and number, particularly when renal angiography is not available. This technique of en bloc hilar clamping is quite useful especially in developing countries where robotic facilities are not available. The Satinsky clamp decreases blood loss and intraoperative time.     

Determination of the optimal stylet strategy for the C‐MAC® videolaryngoscope

The C‐MAC^® videolaryngoscope is a novel intubation device that incorporates a camera system at the end of its blade, thereby facilitating obtaining a view of the glottis without alignment of the oral, pharyngeal and tracheal axes. It retains the traditional Macintosh blade shape and can be used as a direct or indirect laryngoscope. We wished to determine the optimal stylet strategy for use with the C‐MAC. Ten anaesthetists were allowed up to three attempts to intubate the trachea in one easy and three progressively more difficult laryngoscopy scenarios in a SimMan^® manikin with four tracheal tube stylet strategies: no stylet; stylet; directional stylet (Parker Flex‐It?); and hockey‐stick stylet. The use of a stylet conferred no advantage in the easy laryngoscopy scenario. In the difficult scenarios, the directional and hockey‐stick stylets performed best. In the most difficult scenario, the median (IQR [range]) duration of the successful intubation attempt was lowest with the hockey‐stick stylet; 18 s (15–22 [12–43]) s, highest with the unstyletted tracheal tube; 60 s (60–60 [60, 60]) s and styletted tracheal tube 60 s (29–60 [18–60]) s, and intermediate with the directional stylet 21 s (15–60 [8–60]) s. The use of a stylet alone does not confer benefit in the setting of easy laryngoscopy. However, in more difficult laryngoscopy scenarios, the C‐MAC videolaryngoscope performs best when used with a stylet that angulates the distal tracheal tube. The hockey‐stick stylet configuration performed best in the scenarios tested.     

Preoperative Neutrophil-to-Lymphocyte Ratio as a Prognostic Predictor after Curative Resection for Hepatocellular Carcinoma

BACKGROUND: This study was designed to evaluate the impact of an elevated preoperative neutrophil-to-lymphocyte ratio (NLR) on outcome after curative resection for hepatocellular carcinoma (HCC). METHODS: Patients undergoing resection for HCC from January 1994 to May 2007 were identified from the hepatobiliary database. Demographics, laboratory analyses, and histopathology data were analyzed. RESULTS: A total of 96 patients were identified with a median age at diagnosis of 65 (range, 15-85) years. The 1-, 3-, and 5-year overall survival rates were 80%, 58%, and 52%, respectively. Although the presence of microvascular invasion, NLR >or=5, and R1 resection margin were adverse predictors of overall survival, there were no independent predictors identified on multivariate analysis. The 1-, 3-, and 5-year disease-free survival rates were 74%, 63%, and 57%, respectively. Preoperative tumor biopsy, NLR >or= 5, multiple liver tumors, microvascular invasion, and R1 resection margin were all predictors of poorer disease-free survival. Multivariate analysis showed that a NLR >or= 5 and R1 resection margin were independent predictors of poorer disease-free survival. The median disease-free survival of those with a NLR >or= 5 was 8 months compared with 18 months for those with a NLR < 5. CONCLUSION: Preoperative NLR >or= 5 was an adverse predictor of disease-free and overall survival.     

Hepatic Resection for Colorectal Metastasis: Impact of Tumour Size

Background Many colorectal liver metastasis patients are denied surgical resection on the basis of tumour size. The aim of this study was to explore the impact of metastasis size on modern liver resection.Methods Using a prospectively collected database, this was a retrospective analysis of 484 consecutive patients who underwent liver resection for colorectal liver metastases between 1993 and 2003. The cohort was divided into two groups: smaller metastases (<8 cm) and larger metastases (≥ 8 cm). Those with larger metastases were then further stratified into big metastases (8–12 cm) and giant metastases (>12 cm). Demographic, pathological, surgical technique and outcome data were compared between the groups.Results There were 88 (18%) patients with metastases measuring 8 cm or larger. There was an association between higher carcinoembryonic antigen (CEA) and cancer antigen (CA) 19-9 levels and larger metastases. The actuarial 5-year survival for patients with larger metastases was 38% compared with 42% for smaller metastases (not statistically significant). Patients with giant metastases had poorer overall and disease-free survival (both nonsignificant) compared with those with big metastases: 29% and 28% at 5 years, respectively.Conclusion Patients with colorectal liver metastasis greater than 8 cm and up to 12 cm in size should not be treated differently from those with smaller lesions.     

Bilateral actinomycotic blepharokeratoconjunctivitis

Actinomycotic conjunctivitis is usually unilateral and secondary to canaliculitis. We report an unusual case of bilateral actinomycotic blepharokeratoconjunctivitis in the absence of canaliculitis. Keratitis occurred without any preceding ocular trauma. Both eyes had remissions and relapses in response to the antibiotic-steroid drops. Diagnosis of A. israelii infection was made on the basis of Gram staining, culture and biochemical characteristics. Complete excision of the conjunctival ulcer along with penicillin therapy resulted in cure. This case highlights that microorganisms of the order Actinomycetales should be considered in the differential diagnosis if conjunctivitis or keratitis shows a waxing and waning course.     

Lack of c-kit mutation in familial urticaria pigmentosa

Somatic mutations within c-kit have been reported in individuals with mastocytoses, including urticaria pigmentosa (UP). We have identified three siblings with UP. We aimed to determine whether the c-kit proto-oncogene was playing a part in the aetiology of UP in these three siblings. Using seven microsatellite repeat markers spanning an 8-cM interval encompassing the c-kit gene we followed the transmission of the c-kit gene in this family. Furthermore, single-strand conformation polymorphism analysis was used to scan exon 17 of the c-kit gene for mutations in genomic DNA of all family members and somatic DNA extracted from skin of the eldest affected sibling, the proband. No mutations were found in exon 17 in either genomic DNA of all family members or somatic DNA of the proband. Patients with UP have been shown to possess somatic mutations of the c-kit gene. However, this locus has been excluded as playing a part in the three siblings examined here in whom a second gene locus must be determining their UP. Therefore, this study emphasizes genetic heterogeneity in UP. Future study to identify primary molecular determinants of UP should include affected sib-pair studies.