Wide Resection for Pediatric Extremity Vascular Malformation-A Tertiary Hospital's Experience.

BACKGROUND: Vascular malformations of the extremities (VM) are common benign lesions that tend to grow throughout the patient's lifetime. They can cause various issues like pain, swelling, and even limb length discrepancies. Sclerotherapy was the preferred treatment choice in previous studies. However, sclerotherapy and many other treatments have the potential to result in higher recurrence rates. Surgical treatment has been shown to be effective and safe in many cases. Hence, this study aims to evaluate the suitability of wide resection surgery for VM to reduce recurrence. METHODS: Fort-seven VM cases that underwent wide resection were identified retrospectively in the institution of study. Demographics, depth of malformation, whether malformations were local or diffuse, location and size of malformations, and histology records were taken note of. Records of recurrence and postoperative function were also gathered. We utilized self-reported questionnaires, QuickDASH and Lower Extremity Functional Scale, to determine patients' postsurgical physical function. RESULTS: Out of 47 cases that underwent wide resection, we found a recurrence rate of 2.1%. No patients sustained any loss of function postsurgery, with few patients experiencing minor complications like tenderness, hypertrophic scars/keloids, as well as numbness. Good functionality posttreatment was also seen through self-reported questionnaires, with an average score of 2.12 for QuickDASH and 99.96% for LEFS. CONCLUSION: Where margins can be obtained without functional impairment, surgical-wide resection for VM is a viable treatment option to minimize recurrence. LEVEL OF EVIDENCE: Level-IV.

Fibrous dysplasia in cardio-facio-cutaneous syndrome: A case report and review of literature.

Cardio-facio-cutaneous (CFC) syndrome (OMIM #:115150, 615278, 615279, 615280) is a rare genetic condition caused by variants in the RAS/mitogen-activated protein kinase (MAPK) signal transduction pathway. Up to 75% of cases are caused by mutations in the BRAF gene, whereas KRAS gene mutation has only been reported in <2% of cases. CFC syndrome is characterized by cardiac abnormalities, distinctive craniofacial dysmorphism, and various cutaneous abnormalities. Musculoskeletal and orthopedic manifestations are also prevalent in patients with CFC syndrome, among which the most common are skeletal deformities and joint laxities. Dysplastic bone disorders, on the other hand, have not been reported in CFC syndrome before. We report on a case of symmetrical polyostotic fibrous dysplasia (FD) in a patient with CFC syndrome with the KRAS(NM_004985.5):c.57G>C; p.Leu19Phe variant. The FDs were incidentally picked up, and patient was conservatively managed and remained asymptomatic on follow-up. The same variant was reported previously in a patient with Oculoectodermal Syndrome (OES), who developed polyostotic non-ossifying fibroma (NOF). This case explores FD as a possible new clinical feature of CFC syndrome, and when linked to the historical case of OES, explores whether the KRAS(NM_004985.5):c.57G>C; p.Leu19Phe mutation may potentially contribute to the development of dysplastic bone lesions in patients with this particular mutation.

A pilot study comparing parent and adolescent online health information seeking behaviours in elective pediatric surgical situations.

PURPOSE: Little is known of how children seek health information. This study evaluates online health information (OHI) seeking behaviours in adolescents undergoing major elective surgical procedures and compares responses within parent-child dyads. METHODS: With institutional approval, we prospectively surveyed parents of children admitted to our institution for major elective operations between November 2017 and November 2018, using convenience sampling. Patients aged 12 years and above were also invited. Each respondent completed an anonymized modification of a previously published survey on Internet usage. Chi squared tests were used for categorical data, with significance at P value < 0.05. RESULTS: Ninety-one parents and 19 patients (median age 15 years, range 12-18) responded, with 13 parent-child pairs. Daily Internet access was reported by 84 (93%) parents and 18 (95%) children, but OHI was sought in 77% of parents and 74% of children. Six (32%) children could not name their admitting condition, compared to 10 (11%) parents. Nine (50%) children consulted family and friends for information compared to 27 (30%) parents. Parents were more likely to access hospital websites (n = 15, 44%) compared to no children (p = 0.01), while most children (n = 7, 70%) accessed non-health websites (e.g. Wikipedia). In the 13 parent-child pairs, only one parent accurately assessed what their child understood of their condition. Most patients (63.6%) did not understand the aspects of their condition that their parents deemed important. CONCLUSIONS: This study highlights the differences in parental and child behaviours. Children are equally important to include when counselling. Surgeons can guide both parties to reliable Internet sources for health information.

Five-year outcomes of minimally invasive versus open transforaminal lumbar interbody fusion: a matched-pair comparison study.

STUDY DESIGN: Retrospective analysis of prospectively collected data. OBJECTIVE: To compare midterm clinical and radiological outcomes of minimally invasive surgery (MIS) versus open transforaminal lumbar interbody fusion (TLIF). SUMMARY OF BACKGROUND DATA: Open TLIF is a proven technique to achieve fusion in symptomatic spinal deformities and instabilities. The possible advantages of MIS TLIF include reduced blood loss, less pain, and shorter hospitalization. To date, there is no published data comparing their midterm outcomes. METHODS: From 2004-2007, 40 cases of open TLIF were matched paired with 40 cases of MIS TLIF for age, sex, body mass index, and the levels on which the spine was operated. Oswestry Disability Index, neurogenic symptom score, the 36-Item Short Form Health Survey, and visual analogue scale scores for back and leg pain were obtained before surgery, 6 months, 2 years, and 5 years after surgery. Fusion rates were assessed using Bridwell classification. RESULTS: Fluoroscopic time (MIS: 55.2 s, open: 16.4 s, P < 0.001) was longer in MIS cases. Operative time (MIS: 185 min, open: 166 min, P = 0.085) was not significantly longer in MIS cases. MIS had less blood loss (127 mL) versus open (405 mL, P < 0.001) procedures. Morphine use for MIS cases (8.5 mg) was less compared with open (24.2 mg, P = 0.006). Patients who underwent MIS (1.5 d) ambulated earlier than those who underwent open fusion (3 d, P < 0.001). Patients who underwent MIS (3.6 d) had shorter hospitalization than those who underwent open fusion (5.9 d, P < 0.001). Both groups showed significant improvement in Oswestry Disability Index, neurogenic symptom score, back and leg pain, SF-36 scores at 6 months until 5 years with no significant differences between them. Grade 1 fusion was achieved in 97.5% of both groups at 5 years. The overall complication rate was 20% for the open group and 15% for MIS group (P = 0.774), including 4 cases of adjacent segment disease for each group. CONCLUSION: MIS TLIF is comparable with open TLIF in terms of midterm clinical outcomes and fusion rates with the additional benefits of less initial postoperative pain, less blood loss, earlier rehabilitation, and shorter hospitalization.