Anesthesia of a patient with congenital cataract, facial dysmorphism, and neuropathy syndrome for posterior scoliosis: A case report.

BACKGROUND: Congenital cataract, facial dysmorphism, and neuropathy (CCFDN) syndrome is an extremely rare multiorgan disorder. Characteristics include congenital cataracts, facial deformation, extremity deformities, and demyelinating neuropathy. CCFDN syndrome is associated with increased risk during anesthesia including rhabdomyolysis or epileptic seizures. There is a lack of published information about difficult airways in these patients. Difficult airways during intubation represent one of the most dreaded anesthesia complications: A "can not intubate, can not oxygenate" scenario. Presented herein is the first described successful endotracheal intubation of a CCFDN syndrome patient. CASE SUMMARY: We report the anesthetic management of a 13-year-old girl with CCFDN syndrome scheduled for posterior neuromuscular scoliosis correction surgery. The patient suffered from extensive progressive neuromuscular scoliosis with a Cobb angle of 83°. Her limitations included neuropathy and a scoliotic curve. This condition negatively impacted her quality of life. This case reflects the potential anesthetic complications for posterior scoliosis correction and CCFDN syndrome. The challenge for our anesthetic team was the limited amount of data about anesthetic management of this condition. In total, one case report without any data about endotracheal intubation of patients with this condition was available. Endotracheal intubation in our case was uncomplicated. Another focus of our case was the prevention of possible complications associated with this syndrome, including rhabdomyolysis and seizures. Rhabdomyolysis can be triggered by some types of anesthetic agents like suxamethonium or volatile anesthetics, especially in patients with certain types of myopathies. CONCLUSION: Adequate understanding of the anesthetic management of CCFDN syndrome can reduce perioperative complications and improve patient outcome after surgery.

AIRWAY MANAGEMENT IN LAPAROSCOPIC CHOLECYSTECTOMY - A COMPARATIVE ANALYSIS.

In this study, we aimed to compare supraglottic airway devices (Supreme and i-gel laryngeal mask) with tracheal tube with respect to airway control and efficiency in ventilation and oxygenation. The study included 325 patients of ASA I-II who underwent laparoscopic cholecystectomy. In group 1, the airway was secured using endotracheal intubation (115 patients). In group 2 (103 patients), LMA Supreme was applied, whereas i-gel mask was used for airway management in group 3 (107 patients). Monitoring parameters were recorded and compared using t-test, analysis of variance (ANOVA), Tukey's test and χ2-test. The following parameters were monitored: insertion time, number of attempts for device placement, oropharyngeal seal pressure, etc. Insertion time was longest in group 1 (14.7±1.65 s) as compared to group 2 (15.5±1.05 s) and group 3 (14.1±1.27 s); ANOVA test yielded a statistically significant difference (p<0.01). Insertion success rate was almost identical in all three groups (p=0.907, χ2-test). Comparison of oropharyngeal seal pressure between group 2 (35.95±2.92 cm H2O) and group 3 (36.47±1.43 cm H2O) yielded no statistical difference (p=0.314, t-test). Endotracheal tube, Supreme and i-gel laryngeal masks were shown to be equally efficient in airway management in laparoscopic cholecystectomy. All three devices enabled efficient ventilation and oxygenation despite certain pathophysiological changes associated with laparoscopy.

How frequently does palmoplantar psoriasis affect the palms and/or soles? A systematic review and meta-analysis.

INTRODUCTION: Palmoplantar psoriasis (PPP) is a variant of psoriasis that affects the palms and/or soles. Although PPP is a disabling and therapeutically challenging condition, its epidemiology is poorly defined. AIM: To assess the prevalence of PPP locations (palms, soles or both), and to analyse epidemiological and clinical characteristics of the disease. MATERIAL AND METHODS: Two bibliographic databases (MEDLINE and SCOPUS) were used as data sources searched from inception to October 2017. The selection of articles was limited to human subjects and English or French languages. RESULTS: A search resulted in a total of 293 articles, out of which 24 were utilized for the current systematic review and 21 for meta-analysis. All listed studies comprised a total of 2083 patients with PPP, with more males than females. According to the results of meta-analysis, majority of patients had the highest prevalence of both palms and soles involvement (95% CI: 47-67), with an almost equal prevalence showing palmar (21%; 95% CI: 13-30) or plantar (20%; 95% CI: 12-29) involvement. The most prevalent type of PPP was plaque/hyperkeratotic, followed by the pustular type. CONCLUSIONS: Almost three-fifths (59%) of all PPP patients had involvement of both palms and soles, while exclusive palmar or plantar involvement was seen in 21% and 20% of patients, respectively. Future research should be performed to elucidate basic epidemiological and clinical characteristics of PPP, which would be helpful for proper consideration of this condition.

The titanium-made growth-guidance technique for early-onset scoliosis at minimum 2-year follow-up: A prospective multicenter study.

BACKGROUND: The management of early-onset scoliosis (EOS) remains a serious challenge in pediatric orthopedics. The growth-guidance system (GGS) is a surgical option that allows continuous growth along a rod, averting the need for repeated operative lengthening. OBJECTIVES: The objective of this study was to evaluate the outcomes of the GGS in the treatment of EOS. MATERIAL AND METHODS: A prospective study, including 81 patients from 4 departments treated with this method from 2013 to 2015, was conducted with a minimum follow-up period of 24 months. The follow-up data of 57 patients was available, thus the drop-out rate was 29.63%. There were 44 girls with a mean age of 10.03 years and 13 boys with a mean age of 8.04 years. RESULTS: The mean preoperative Cobb angle was 65.3° (range 36°-139°) was corrected to 23.7° (2°-94°), and at the end of the 2-year follow-up increased to 30.7° (8°-93°). The predominant proximal level of instrumentation was T5 and the distal was L1. The combined length of T1-T12 and T12-S1 increased on average by 33.19 mm in 24 months. The overall rate of serious complications was 43.86%. The most prevalent device-related complications were: the dislodgement of top screws because of the short length of the rod (14 cases), the implant failure (11 cases) and loss of correction (9 cases). CONCLUSIONS: The results show that the GGS used in this study allows for a good and stable correction while preserving the ability of the spine to grow in at least a 2-year follow-up. The complication rate is acceptable and comparable with other growth-friendly techniques. To date, this is the largest successful study on the use of titanium-made GGSs.

Quantitative morphometric analysis of the myenteric nervous plexus ganglion structures along the human digestive tract.

BACKGROUND/AIM: All the functions of the digestive system are controlled, guided and initiated by the autonomic nervous system. A special part of this system placed in the wall of the gastrointestinal tract is known as the enteric or metasympathetic nervous system. The aim of this study was to analyse myenteric nervous plexus in different parts of the digestive tract. METHODS: We examined the myenteric nervous plexus of the esophagus, stomach, duodenum, jejunum, ileum, transverse colon and rectum in tissue samples taken from 30 cadavers of persons aged 20-84 years. After standard histological processing sections were stained with hematoxylin-eosin, cresyl violet (CV) and AgNO3 method. Multipurpose test system M42 was used in morphometric analysis. The results were analyzed by t-test and analysis of variance. RESULTS: The number of neurons per cm² surface was the lowest in the esophagus (2.045 ± 310.30) and the largest in the duodenum (65,511 ± 5,639). The statistical processing showed significant differences (P < 0.001) in the number of neurons between the esophagus and all other parts of the digestive tract. The maximal value of the average surface of the myenteric nervous plexus neurons was observed in the esophagus (588.93 ± 30.45 µm²) and the lowest in the stomach (296.46 ± 22.53 µm²). CONCLUSION: There are differences in the number of ganglion cells among different parts of the human digestive tract. The differences range from a few to several tens of thousands of neuron/cm2. The myenteric nervous plexus of the esophagus was characterized by a significantly smaller number of neurons but their bodies and nuclei are significantly larger compared to other parts of the digestive tract.

Risk factors of postural defects in children at school age.

INTRODUCTION AND OBJECTIVE: Postural defects increasingly more often concern children and adolescents at school age. The lack of prophylaxis and neglecting adequate procedures may lead to limitations of physical and motor abilities, back pain, or the development of severe spinal deformities. Recognition of the risk factors conducive to the occurrence of the disorder allows the creation of adequate conditions for the psychomotor development of children, as well as the elaboration and implementation of specified educational schemes directed at schools and parents. The study concerned determination of the risk factors for the development of postural defects in school age children. MATERIAL AND METHODS: The study was conducted by means of a diagnostic survey. The study group covered 380 children aged 14 (175 girls (46.1%) and 205 boys (53.9%))--selected at random from schools in eastern Poland and the Czech Republic. The significance of the relationships between variables was investigated by means of chi-square test for independence. The differences between the empirical and theoretical sample distribution was examined by means of chi-square goodness-of-fit test. The significance level was set at p=0.05. RESULTS: The BMI in the population examined was 20.2, on average (from 14-39). Respondents living in rural areas and small towns constituted 57.63% of the study group, while inhabitants of medium-size and large cities--42.37%. The majority of children in the study had been previously examined for the occurrence of postural defects (74.2%), whereas nearly every tenth child had never undergone such an examination. As many as 16.3% of adolescents did not know whether they had ever participated in a screening test. A defect was detected in 14.7% of children, in 56.6% no asymmetry was detected, while approximately 30% were not aware if their body posture was normal or not. CONCLUSIONS: 1. There is a relationship between physical activity of the child and the occurrence of postural defects. 2. There is a relationship between the economic standard of the family and awareness of own state of health. 3. There is a need for the creation of a system of education for parents and children concerning postural defects and risks resulting from these defects.

Guided-growth implants in the treatment of early onset scoliosis. A pilot study.

BACKGROUND: The aim of treating children with early onset scoliosis is to guide the growth of the spine until the patients reach skeletal maturity. Regardless of its aetiology, progressive early-onset scoliosis requires multiple-stage surgery, usually at 6 to 12 months' intervals. However, precise coordination of the timing of each consecutive surgical procedure with the child's growth pattern is difficult, and the risk of complications that require additional surgery should be taken into account. The aim of this study is to present a new surgical method of treating early onset scoliosis which consists in a single-stage insertion of special implants that enable three-plane correction of spinal deformities, allows the spine to continue growing, does not require multi-stage surgical distractions, and ends after the growth period with a conventional spinal fusion. The results of this pilot study were obtained in a homogeneous group of patients treated identically by insertion of original implants guiding spinal growth. MATERIAL AND METHODS: The study involved 15 females and 2 males aged between 5 and 13 years (mean age: 9.8 years). All children in the study group had single-curve thoracic scoliosis. The duration of follow-up was between 6 and 40 months (mean duration: 18 months). The efficacy of the guided-growth implant treatment was assessed based on standard radiographs by evaluating the angle of the curvature, T1-S1 length, and apical vertebral rotation (AVR) 1. preoperatively, 2. postoperatively, and 3. in long-term follow-up. RESULTS: After surgery the scoliosis improved significantly in the range of 51% to 80% (mean improvement: 65%). The degree of the correction depended directly on the initial angle of curvature, which ranged from 56 to 95° (mean angle of curvature: 67°). During the entire follow-up period, twelve patients did not show any loss of correction, or the loss was within the bounds of measurement error. Because of a growth spurt, two female patients had to have the rods replaced with longer ones, since there was a risk that they might slide out of the farthest lower screws. In three patients further spontaneous improvement occurred during the follow-up period. Apical vertebral derotation was achieved during the surgery in all patients, and it was maintained throughout the follow-up period. All patients showed an increase in spinal length in the range of 7 to 40 mm (mean increase: 1mm/month). CONCLUSIONS: 1. The surgical method described by us provides for very good correction in the first stage of treatment. 2. The maintenance of the correction does not require the use of corrective braces or any indirect multi-stage surgical procedures. 3. The probability of complications during the insertion of the implants is not higher than that seen with conventional multi-stage treatment.