Musculoskeletal deformities after thoracic surgery in children: An observational long-term follow-up study.

PURPOSE: This study reports the incidence, severity, and predictors of musculoskeletal deformities (MD), including scoliosis and chest wall anomalies, following thoracic procedures in children. METHODS: Children younger than 14 years who had thoracic surgery between 1997 and 2012 and had no other predispositions to MD, underwent longitudinal follow-ups with dedicated musculoskeletal examination performed in an esophageal atresia, orthopedic, or research clinic. Incidence of MD was calculated, and logistic regression methods were used to determine independent predictors, including sex, gestational age, age at procedure, serratus anterior muscle division, and chest tube placement. RESULTS: The study cohort consisted of 104 patients followed for a median of 10.8 years (range 3-21). A total of 56 MD developed in 41 patients (39%), including scapular winging (24; 23%), scoliosis (17; 16%), and chest wall anomalies (15; 14%). The majority of MD were subclinical, with only 8 patients [8% (6 thoracotomies, 2 thoracoscopies)] requiring intervention. Among patients who underwent thoracotomies (93, 89%), serratus anterior muscle division was the only significant predictor of the development of MD [OR 8.9; 95% CI 2.8-32.6]. CONCLUSION: Musculoskeletal deformities develop in a significant proportion of children following thoracic surgery, but most are subclinical. A muscle-sparing technique decreases the incidence of these deformities. TYPE OF STUDY: Prospective Cohort Study. LEVEL OF EVIDENCE: Level II.

Does the presence of non-refluxing hydroureter impact the management and outcome of high-grade hydronephrosis?

INTRODUCTION: We aimed to evaluate the impact of non-refluxing hydroureter on the initial management of high-grade hydronephrosis (HGH) management. Moreover, we evaluated the occurrence of febrile urinary tract infection (fUTI) and surgical intervention for conservatively managed units. METHODS: Patients' charts with postnatal hydronephrosis from 2008-2014 were retrospectively reviewed. We included patients who presented in the first year of life. All included patients had HGH (Society of Fetal Ultrasound [SFU] grades 3 and 4) and all were non-refluxing using voiding cystourethrogram (VCUG). We categorized renal units into two groups: with hydroureteronephrosis (HUN) and without hydroureter (isolated hydronephrosis [IH]). We recorded the initial management. We evaluated the impact of non-refluxing hydroureter on hydronephrosis fate, fUTI, and change to surgery for those managed conservatively. RESULTS: We included 169 patients (180 units). IH was diagnosed in 146 units (137 patients), whereas 34 units (32 patients) had HUN. Median followup was 42.9 months. A total of 25.3% (37/146) of IH units had initial surgical management in comparison to 5.1% (2/34) of HUN units (p=0.01). During conservative management, nine HUN patients (30%) and 11 IH patients (10.7%) experienced fUTI (p=0.009). Surgical intervention after failed conservative management was indicated for 12 IH units (11%) and six HUN units (18.8%) (p=0.25). Anteroposterior diameter (APD) worsening was significantly associated with the change to surgery in IH group (p=0.003). More than half (52.3%) of IH units resolved during conservative management in comparison to 18.7% of HUN (p<0.001). HUN had longer time to resolution (log rank=0.004). CONCLUSIONS: IH units had more initial surgical interventions. The fUTI rate was much higher in association with dilated ureter even under antibiotic coverage. HUN was associated with less and slower resolution rate.

A Notch more: Molecular players in bicuspid aortic valve disease.

The prevalence of calcification of human aortic valve in populations 65 years old and greater is estimated to be 2-3%. Bicuspid aortic valve disease (BAVD) is a common etiology of aortic stenosis in populations aged 60-75 years of age; 30-50% of operated cases of aortic stenosis were due to calcified BAVD. Dysregulation of the Notch and the canonical Wnt pathway has been well documented to be associated with calcification of the aortic valve. However, recent studies have increased this scope to include the non-canonical pathway where Wnt5a, Wnt5b and Wnt 11 levels were significantly greater in calcified human aortic valves than normal valves, and with Wnt5b specifically being implicated in BAVD pathogenesis. More recently, Lipoprotein(a) [Lp(a)] has been implicated as a key player in the pathogenesis of calcific aortic valve disease. It has been shown that the osteogenic effect of Lp(a) is mediated through the oxidized phospholipid pathway as well as oxidized phospholipid independent pathways involving mitogen-activated protein kinases (MAPK), glycogen synthase kinase (GSK) and Wnt. Moving forward, further work needs to be conducted in order to elucidate the crosstalk between the different signaling cascades, specifically with regard to BAVD.

Musculoskeletal deformities following neonatal thoracotomy: long-term follow-up of an esophageal atresia cohort.

BACKGROUND: Musculoskeletal deformities (MD), including scoliosis and chest wall anomalies, are potential long-term complications of neonatal thoracotomies. METHODS: We studied the incidence of MD in patients who underwent open repair of esophageal atresia between 1997 and 2012, had no other predisposition to MD, and subsequently received longitudinal follow-up in a multidisciplinary esophageal atresia clinic. Detailed chest wall and musculoskeletal exams were performed at each visit. Incident rate and incident rate ratios were used to determine the incidence of deformities. Logistic regression methods were used to test the effect of independent variables including sex, gestational age, muscle division, number of thoracotomies, and operative complications on the occurrence of MD. RESULTS: The study cohort consisted of 52 patients followed for a median of 8 (range 1-19) years. MD developed in 13 (25%), with an incident rate of 2.92 per 100 child-years. Division of the serratus anterior was associated with a significantly higher probability of developing MD (log-rank p=.0237) and was also a strong predictor of the same [OR 8.6 (95% CI 1.8-42.1)] after adjusting for possible confounders. CONCLUSIONS: Musculoskeletal deformities develop in a significant proportion of neonates following thoracotomy. A muscle-sparing technique decreases the incidence of these deformities. TYPE OF STUDY: Prospective Cohort Study. LEVEL OF EVIDENCE: II.