Effects of magnetically controlled growing rods surgery on pulmonary function in young subjects with spinal muscular atrophy type 2 and other neuromuscular scoliosis.

BACKGROUND: The aim of this study was to determine the effects of magnetically controlled growing rods surgery (MCGRS) of the scoliosis on pulmonary function in children with neuromuscular scoliosis. METHODS: Seven patients, 85.7% female (mean±SD age: 6.7±1.2 years), with neuromuscular scoliosis (4 SMA II, 2 congenital myopathies and 1 VACTER syndrome), received MCGRS of the spine in the thoraco-lumbar area. The outcome measures were clinical features and pulmonary function (forced vital capacity [FVC] and forced expiratory volume in 1st second [FEV1], were collected. All measures were collected at pre-, post-intervention and follow-up (short-term [0-6 months], mid-term [7-12 months], and long-term [13-24 months]). RESULTS: MCGRS reduced Cobb angle after intervention in 100% in subjects and this result was maintained at 24-month follow-up (all, P=0.001). There was no significant difference in FVC or FEV1 between preoperative and each postoperative period, (P>0.05). Analyses of the correlation coefficients indicated no significant associations between changes in pulmonary function and scoliosis. CONCLUSIONS: The current study found that MCGRS addressed to the scoliosis maintained pulmonary function during long-term follow-up; However, pulmonary function was not associated with scoiosis in children with neuromuscular scoliosis.

Orofacial granulomatosis in children: think about Crohn's disease.

BACKGROUND: The term orofacial granulomatosis is conventionally used to describe patients with granulomatous lesions affecting the orofacial tissues, in absence of intestinal lesions. Lip swelling and facial swelling are the most common clinical signs. Despite the fact that histologically it is not distinguishable from Crohn's disease, and that both diseases have a chronic/recurrent course, the relationship between orofacial granulomatosis and Crohn's disease is still debated. METHODS: Herein we present five cases of orofacial granulomatosis. RESULTS: All patients presented concomitant Crohn's disease, supporting the hypothesis that orofacial granulomatosis and Crohn's disease may be one single disease. Thalidomide was effective in inducing remission of oral and intestinal symptoms in all five cases and could be considered a valid treatment opportunity for these patients. CONCLUSIONS: Orofacial granulomatosis and Crohn's disease may be part of the same disease; both may respond to thalidomide.

Non-invasive positive pressure ventilation to facilitate the post-operative respiratory outcome of spine surgery in neuromuscular children.

PURPOSE: Scoliosis surgery may be associated with a high morbidity and even mortality in children with non-idiopathic scoliosis. The aim of the study was to report our experience with a pre-operative training to non-invasive positive pressure ventilation (NPPV) and a mechanical insufflator-exsufflator (MI-E) device to improve the post-operative respiratory outcome of children scheduled for scoliosis surgery. METHODS: Consecutive patients with non-idiopathic scoliosis undergoing posterior arthrodesis were trained to NPPV and MI-E before intervention. NPPV and MI-E were performed immediately after extubation. Length of intubation and intensive care unit (ICU) stay, duration of NPPV, and respiratory complications were assessed. RESULTS: Thirteen patients participated in the training (mean age 13.9 ± 2.6, mean vital capacity 52.3 ± 15.4% predicted). The patients had severe respiratory muscle weakness with a mean sniff oesophageal pressure of 35.8 ± 14.2 cmH2O (50% predicted) and a mean gastric pressure during a cough of 31.9 ± 7.8 cmH2O (30% predicted). The mean length of intubation was 19.9 ± 12.3 h with a mean length of ICU stay of 2.5 ± 2.5 days. NPPV was used during a mean of 2.7 ± 1.9 days after surgery. No respiratory complication was observed. One patient died 3 months after surgery from multi-organ failure of non-respiratory origin. CONCLUSIONS: No respiratory complications were observed after scoliosis correction surgery in children with non-idiopathic scoliosis after pre-operative training and post-operative use of NPPV and MI-E, underlying the interest of this management in these high-risk patients.

Lung function and respiratory health at school age in ventilated very low birth weight infants.

OBJECTIVE: To investigate respiratory health and lung function in school-aged children without broncho-pulmonary dysplasia (BPD), who were very low birth weight (VLBWi) and randomized at birth to high frequency oscillatory ventilation (HFOV) or volume guarantee (VG) ventilation for severe respiratory distress syndrome (RDS). METHODS: In this observational study, 7-y-old ex-preterm infants with severe RDS, randomly assigned at birth to receive assisted/control ventilation + VG (Vt = 5 mL/kg, PEEP = 5 cmH2O)(VG group; mean GA 27 ± 2 wk; mean BW 1086 ± 158 g) or HFOV (HFOV group; mean GA: 27 ± 2; mean BW: 1090 ± 139 g) (both groups were ventilated with Drager Babylog 8000 plus) were recalled. Neonatal clinical data and outcome were known. Actual outcomes were investigated with an interview; lung function was measured by whole-body plethysmography. RESULTS: Twenty five children were studied (VG group, n = 13 vs. HFOV group, n = 12). There were no differences in anthropometric data, drugs (steroids/bronchodilators and antibiotics) or hospital readmission for respiratory disorders. Compliance to the test was adequate. The authors found a similar obstructive deficit (elevated values: airway resistance (RAW), residual volume (RV), total lung capacity (TLC) with near-normal spirometry) in both groups suggesting a persistent airflow limitation even in absence of BPD. CONCLUSIONS: VLBW infants even in absence of BPD, need long term respiratory follow-up, because they frequently show an impairment of lung function, independent from initial respiratory support, even if at birth the choice is a lung protective approach (e.g., HFOV or VG ventilation).

Nocturnal hypoxaemia and hypercapnia in children with neuromuscular disorders.

The aim of the study was to identify daytime predictors of nocturnal gas exchange anomalies in children with neuromuscular disease (NMD) and normal daytime gas exchange. Lung function tests, respiratory muscle evaluation and nocturnal gas exchange were obtained as part of routine evaluation. We included 52 consecutive children with Duchenne muscular dystrophy (n = 20), spinal muscular atrophy (n = 10) and other NMD (n = 22). 20 patients had nocturnal hypoxaemia, defined as minimal arterial oxygen saturation measured by pulse oximetry (S(p,O(2))) <90% for ≥ 2% of night time, and 22 had nocturnal hypercapnia, defined as maximal transcutaneous carbon dioxide tension (P(tc,CO(2))) >50 mmHg for ≥ 2% of night time. Forced vital capacity and helium functional residual capacity correlated with minimal nocturnal S(p,O(2)) (p = 0.009 and p = 0.01, respectively). Daytime pH correlated negatively with maximal nocturnal P(tc,CO(2)) (p=0.005) and daytime arterial carbon dioxide tension (P(a,CO(2))) correlated with the percentage of time with a P(tc,CO(2)) >50 mmHg (p = 0.02). Sniff nasal inspiratory pressure correlated with minimal nocturnal S(p,O(2)) (p = 0.02). Daytime P(a,CO(2)) was a weak predictor of nocturnal hypercapnia (sensitivity 80%; specificity 57%). Daytime lung function and respiratory muscle parameters correlate poorly with nocturnal hypoxaemia and hypercapnia in children with NMD and normal daytime gas exchange, which necessitates more systematic sleep studies in these children.