Thoracogenic scoliosis: coronal deformity progression in paediatric patients.

PURPOSE: To assess the risk of developing thoracogenic scoliosis (THS) in paediatric patients, depending on the side of lateral thoracotomy (LT) and of spine deviation in the coronal plane by means of logistic regression and scoliosis-free survival analyses. METHODS: A total of 307 consecutive patients undergoing LT were retrospectively reviewed; 32 patients met the inclusion criteria: 1) underwent LT and developed THS; 2) age < 15 years at LT; 3) clinical and radiographic follow-up ≥ 5 years. Patients were divided into ipsilateral group (convexity ipsilateral to LT) and contralateral group (convexity contralateral to LT). RESULTS: The mean follow-up was 10 ± 4.7 in the ipsilateral group (n = 13) and 12 ± 4.8 years in the contralateral group (n = 19). The contralateral group underwent LT at a younger age (4.4 vs 6.4 years, p = 0.55), developed more severe coronal deformity (27.8° vs 18.9°, p = 0.15), had higher rate of THS > 20° (47.5% vs 38.4%; p = 0.34) and > 45° (21.1% vs 0%; p = 0.99). The mean time from LT to THS diagnosis was 4.2 ± 2.9 in ipsilateral group and 5.5 ± 4.2 years in contralateral group. Left-side LT (p = 0.03) and age > 5 years (p = 0.01) showed a lower risk of THS diagnosis. The group variable had a statistically significant effect on the risk of developing THS > 45° (p = 0.03). CONCLUSION: In this series, children that developed THS with a convexity contralateral to the side of LT had more severe and progressive coronal spine deformity. LEVEL OF EVIDENCE: III.

Prevalence of acid gastroesophageal reflux disease in infants with esophageal atresia/tracheoesophageal fistula.

BACKGROUND: Given the high prevalence and complication risks of acid gastroesophageal reflux (GERD) in the first months of life in infants with esophageal atresia, the ESPGHAN/NASPGHAN consensus statement recommends systematic treatment with proton pump inhibitors (PPIs) until the age of 1 year and checking for acid GERD thereafter. However, these recommendations have not been evaluated. METHODS: This prospective study was conducted from 2007 to 2016. We evaluated the prevalence of acid GERD in 100 consecutive infants presenting with esophageal atresia/tracheoesophageal fistula after the age of 18 months when PPI treatment was stopped. The diagnosis of acid GERD was based on positive pH-metry and/or evidence of complications (e.g., peptic esophagitis, need for jejunal nutrition, or antireflux surgery). Those with acid GERD at a median age of 18 months received a control examination every year or adapted to their clinical situation. RESULTS: The prevalence rates of acid GERD were 64.3% at 18 months and 22.8% at the last follow-up (median age 65 months).There is no risk factor for acid GERD identified. CONCLUSIONS: This study shows a high prevalence of acid GERD in late infancy and supports the recommendation of systematic checking for acid GERD when treatment with PPI is stopped. IMPACT: Acid gastroesophageal reflux disease (GERD) is a frequent complication of esophageal atresia in infants. The ESPGHAN/NASPGHAN consensus, which is based on expert opinion, recommends systematic treatment of children with PPI until the age of 1 year. The prevalence rates of acid GERD were 64.3% at 18 months and 22.8% at the last follow-up. This study shows a high prevalence of acid GERD in late infancy and supports the recommendation of systematic checking for acid GERD when treatment with PPI is stopped.

Thoracogenic scoliosis: a retrospective review of 129 pediatric patients with a mean follow-up of 10 years.

PURPOSE: To determine the midterm outcome of lateral thoracotomy (LT) in skeletally immature patients concerning thoracogenic scoliosis development and lung parenchyma resection (LPR) extent. METHODS: In total, 129 children met the inclusion criteria: (1) LT during the study period; (2) skeletally immature at the time of LT; (3) clinical and radiographic follow-up of at least 3 years; and (4) no spinal or thoracic deformity on radiographs before LT. Patients were grouped according to their underlying disease, age at LT, and LPR extent. Radiographic parameters were assessed. Kaplan-Meier survival curves and univariate and multivariate analysis were performed. RESULTS: Of 129, 108 patients underwent pneumonectomy (9; 9.1%), lobectomy (79; 61.2%), segmentectomy (20; 15.5%) and 21 patients LT without LPR. The mean age at LT and at last radiological follow-up was 5.5 years (birth-17.8) and 15.2 years (3.4-33.2). The mean follow-up was 10 years (3-28.1). Scoliosis developed on average 5.3 years after LT. The mean Cobb angle was 22.1° (11-90°); > 10° in 37/129 patients (28.7%), of whom 5/129 (3.9%) had > 45°. The average vertebral rotation was 16.2° (2-43°; grade 0-II). RVAD was 26.5° (8°-33°) and 15.3° (2-43°) in patients with Cobb > 45° and ≤ 45°. Gender, age at surgery, and extent of LPR were not risk factors for post-thoracotomy scoliosis (p > 0.05), although younger patients developed a more severe deformity. CONCLUSION: Although post-thoracotomy scoliosis is not associated with significant rotation, the risk of curve progression > 45° is relatively high. Regular follow-up is required as scoliosis may develop several years after LT with or without LPR.

Dilated Cardiomyopathy Caused by Wilms Tumor.

The authors report the case of a 7-month-old girl with unilateral Wilms tumor with dilated cardiomyopathy, leading to cardiac failure and requiring intensive care. After chemotherapy and tumor removal, cardiac function has improved. This is a rare report on the association between nephroblastoma and dilated cardiomyopathy without hypertension.

Necrotizing enterocolitis as an adverse effect of recombinant interleukin-2 and Ch14.18 in maintenance therapy for high-risk neuroblastoma.

Recombinant interleukin-2 is used with ch14.18/CHO to improve the cytotoxic activity of NK lymphocytes against neoplastic cells. The efficacy of this treatment is limited by its potential side effects. We report an unusual case of necrotizing enterocolitis associated with the administration of interleukin-2 and ch14.18/CHO in maintenance therapy for localized NMyc amplified neuroblastoma (NBL). This case highlights the potentially significant toxicity of this immunotherapy that is currently being tested in the high-risk NBL-1.5 protocol. Further, short-term, medium-term, and long-term follow-up in this patient population will be warranted to judge the potential benefit of this treatment versus the short-term, medium-term, and long-term side effects in a patient population with an outcome that is better than that of stage 4 NBL patients.

Intestinal ischaemia as a severe presentation of Kawasaki disease leading to short-bowel syndrome.

The authors describe a case of Kawasaki disease who presented fever, coronary artery aneurysm, and ischaemic intestinal obstruction requiring stage resections. A review of literature of such association is performed. Atypical Kawasaki disease is more complex to diagnose and in combination of persistent fever with no definite cause and surgical abdominal pain, Kawasaki disease should be considered in the differential diagnosis.

Barrett esophagus and esophagojejunal anastomotic stenosis as complications of esophagogastric disconnection in children with esophageal atresia.

Total esophagogastric disconnection (TED) is an alternative surgical procedure in resistant gastroesophageal reflux disease. We report 2 severe, not yet described long-term complications of TED occurring in 4 children with a history of esophageal atresia. Three children presented with stenosis of the esophagojejunal anastomosis 5 months to 9 years after TED, requiring repeated dilations associated with mitomycin C application in one of them. Barrett esophagus was observed in 3 children 8 to 9 years after TED. Careful long-term clinical and endoscopic follow-up of children who underwent TED is required.

Porous Ceramic Sternal Prosthesis Implantation in a 13-Year-Old Patient Presenting with Metastatic Ewing's Sarcoma.

Ewing's sarcoma is the second most frequent primary malignant bone tumor in adolescents and young adults. Locations on the thoracic wall represent up to 20% of primary and secondary locations. We present the case of a 13-year-old patient treated with the use of a radiolucency porous bioceramic prosthesis as a sternal replacement for a wide tumor resection in an oncologic context. Focal radiation therapy was not possible due to the high risk of severe myocardial injuries caused by the sternal location of the tumor. The sternum CERAMIL® (I.CERAM, Limoges, France), in porous alumina (Al 2 O 3 ) has already been implanted into adults in sternal replacement during its invasion by a tumor or its infectious destruction. There were no complication concerning the surgery. The last follow-up at 2 years postoperatively reveals a satisfactory clinical situation with any functional thoracic complaint and nor any functional respiratory symptoms. The porous alumina sternal prosthesis offers a reliable alternative for sternal replacement indications for children in an oncologic context.

Prolonged enteral feeding is often required to avoid long-term nutritional and metabolic complications after esophagogastric dissociation.

BACKGROUND: Total esophagogastric dissociation (TED) was first described in 1997 by Bianchi as a new surgical procedure to treat severe gastroesophageal reflux disease (GERD) in children with neurological impairment. Recently, TED has been proposed in other conditions, such as esophageal atresia, esotracheal cleft, or caustic esophageal lesions. Although the long-term results in terms of GERD control have been previously reported, those regarding the nutritional and metabolic status have never been documented. PATIENTS AND METHODS: All patients without neurological impairment with TED between 1999 and 2004 at Robert Debre Hospital and Jeanne de Flandre Hospital, France, were prospectively investigated, paying particular attention to their metabolic and nutritional status (blood concentration of iron and vitamins A, D, E, and B12; lipid malabsorption; and hyperglycemia test) and growth. RESULTS: Seventeen children underwent TED. Six received primary procedures, whereas 11 were operated on because of severe respiratory diseases or failure to thrive. The mean follow-up was 6 years (range 3-8 years). Two children died (12%). Seven children were weaned from enteral nutrition support, but 5 of them had failure to thrive, steatorrhea, and/or malabsorption of vitamin B12 and/or fat-soluble vitamins. Eight patients had dumping syndrome, which was symptomatic in 6 cases. CONCLUSIONS: TED is an effective procedure for treatment of GERD. However, nutritional and metabolic complications including dumping syndrome and chronic digestive malabsorption are frequent after TED, especially after enteral nutrition weaning. A long-term follow-up of these patients is thus necessary and prolonged enteral nutrition support is recommended.

Original bar fixation technique in minimally invasive repair of pectus excavatum in adolescents: A 36-case series.

In pectus excavatum, minimally invasive endoscopic repair is the current gold standard in adolescents. The main postoperative pitfall is bar displacement, making fixation a major issue. We report an original transcostal technique using absorbable USP-2 suture in 36 patients. There was a single case of bar displacement (2.78%; 95% CI: 0.07-14.53). Bar stability was comparable to literature reports; but the present technique avoided the complications potentially incurred by use of metal wire, pericostal fixation points and stabilizers.

[Complexity of pathological interpretation in megacystis-microcolon-intestinal hypoperistalsis syndrome]. / Complexité de l'interprétation anatomopathologique dans le syndrome mégavessie-microcôlon-hypopéristaltisme intestinal.

Megacystis-microcolon-intestinal hypoperistalsis syndrome is very rare, and is the most severe of the chronic intestinal pseudoobstructions. Diagnosis is usually made in the neonatal period, is clinical and radiological, and is confirmed by manometric studies. Microscopic abnormalities are variable, inconstant and nonspecific. They involve the smooth muscle more often than the intrinsic innervation of the gut and the bladder. A girl, currently seven years old, presented with megacystis observed on prenatal ultrasound at 21 weeks of gestation. At first, amniotic fluid volume was appropriate for gestational age, and then hydramnios appeared at 30 weeks of gestation. Microcolon was discovered at birth, with microileum, dilatation of the duodenum and proximal jejunum, intestinal malposition, and severe hypoperistalsis of the entire gastrointestinal tract, which indicated enterostomy and total parenteral nutrition from birth. At pathological examination, rectal biopsy and enteric nervous plexuses were normal. There was hypoplasia of the external longitudinal layer of the muscularis propria in the colon and ileum. Cajal cells could not be demonstrated immunohistochemically in the colon. This case highlights the complexity and difficulties of pathological interpretation in this syndrome, and the necessity of a large study of controls at different ages and different levels of the digestive tract and the bladder.

Long-term parenteral nutrition, via the azygos system, in an adolescent with cystic fibrosis.

Venous access device is critically needed for long-term parenteral nutrition (PN), especially in children with chronic disease such as cystic fibrosis, short bowel syndrome, and permanent intestinal failure. When traditional sites are unavailable and venous access is very limited, alternative central routes are required. The access to the azygos system has been shown to be a safe, relatively easy solution in those special situations. We report the case of an adolescent who benefited from this central venous access for long-term PN and antibiotic treatment for >7 years with a limited number of complications.

Postoperative lower esophageal dilation in children following the performance of Nissen fundoplication.

OBJECTIVES: The purpose of this study is to study the frequency and factors associated with lower esophageal dilation (LED) after Nissen fundoplication. METHODS: This retrospective monocentric study included 288 patients who had undergone Nissen fundoplication from 1998 to 2009. The frequency of children requiring LED was assessed. The clinical characteristics of the patients at the time of fundoplication, their symptoms, and outcomes were recorded. The population with LED (group 1) was compared with the population without LED (group 2) to identify factors associated with postfundoplication LED using multivariate analysis. RESULTS: LED was required by 70 patients (24%) because of postoperative dysphagia, and 45/70 were dilated within the first 6 months. The mean age at dilation was 72 months (standard deviation [SD] 65), with an average post-Nissen delay of 9 months (SD 13). Surgical revision was required by 11 patients because of LED failure (n = 10) or postdilation perforation (n = 1). Patients who required post-Nissen dilation were significantly more frequently fed orally than those in group 2 and had more postoperative complications (dumping syndrome, surgical revision). CONCLUSIONS: A significant frequency of postfundoplication LED was observed in this pediatric population. Dilation was associated in children with preoperative feeding or postoperative complications (dumping syndrome, surgical revision).

Long-term outcome of children with oesophageal atresia type III.

OBJECTIVE: The aim of this study was to evaluate the outcome of patients with oesophageal atresia type III (EA), focusing on the presence of late sequelae and quality of life. METHODS: This was a retrospective case ascertainment followed by clinical assessment of patients. The study parameters included the patients' demographic characteristics, associated abnormalities, presence of gastro-oesophageal reflux disease (GERD) and digestive or respiratory symptoms, results of a clinical examination to evaluate nutritional status, spirometry results and quality of life assessed using the PedsQL 4.0 questionnaire. RESULTS: Of 81 patients with EA type III treated in our institution over a 10-year period, 57 (mean age 13.3 (SE 2.8) years) participated in the study. 39% of the patients underwent fundoplication and 46% presented with anastomotic stenosis requiring dilation. 75% of patients had normal nutritional status (16% were obese, 9% were undernourished). Only 19% of participants had no digestive symptoms; 61% had dysphagia and 35% had symptoms of GERD at the last follow-up. The main respiratory symptoms were chronic cough (19%) and dyspnoea on exertion (37%). Only 37% of patients had no respiratory symptoms. Spirometry showed that 50% of patients had proximal obstruction and/or pulmonary distension, and 11% had restriction syndrome. Their quality of life was good but was lower than in healthy controls (80 vs 84, p<0.05) and lower in patients born prematurely, with symptoms of GERD and with a barky cough. CONCLUSION: The high frequency of late sequelae in EA type III justifies regular and multidisciplinary follow-up through to adulthood.

Anastomotic stricture after surgical repair of esophageal atresia: frequency, risk factors, and efficacy of esophageal bougie dilatations.

AIMS: The aim of this study was to evaluate the frequency and risk factors of postoperative anastomotic stricture, and the efficacy and complications of esophageal bougie dilatations for symptomatic anastomotic stricture in a population of children with esophageal atresia. PATIENTS AND METHODS: The medical records of 62 children operated on for esophageal atresia type III (Ladd and Gross) over a 5-year period were retrospectively reviewed. RESULTS: Anastomotic stricture developed in 23 (37%) of patients. Anastomotic tension during primary repair of esophageal atresia was associated with subsequent stricture formation (P < .05). Patients required esophageal dilation at a mean age of 149 days (range, 30-600 days). Stricture resolution occurred after a mean of 3.2 dilatations per patient (range, 1-7). Dilation was successful in 87% of patients. Three patients continued to present mild (n = 1) to severe (n = 2) dysphagia, mainly related to esophageal dysmotility. No complications were observed during or after the dilatation sessions. CONCLUSIONS: Anastomotic stricture, secondary to the surgical treatment of esophageal atresia, remains a frequent complication in patients with esophageal atresia. Esophageal dilation with Savary-Gilliard bougies is a safe and effective procedure in the management of strictures.

Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia.

OBJECTIVES: Although ursodeoxycholic acid has been used to treat various cholestatic liver diseases in children, few data are available about its efficacy in biliary atresia. The aim of this study was to assess the effect of ursodeoxycholic acid treatment on liver function in children who underwent successful surgery for biliary atresia. PATIENTS AND METHODS: We prospectively studied 16 children with biliary atresia who underwent successful portoenterostomies (postoperative conjugated bilirubin concentration: <34 micromol/L) and were treated with ursodeoxycholic acid for at least 18 months after surgery. Ursodeoxycholic acid treatment was then discontinued. Clinical and biological assessment was performed at the time of discontinuation of ursodeoxycholic acid treatment (T0), at follow-up (T1) and, if the clinical or biological status worsened, after resumption of ursodeoxycholic acid treatment (T2). RESULTS: Ursodeoxycholic acid treatment was resumed in 13 cases. In 1 patient, jaundice recurred after ursodeoxycholic acid therapy was discontinued but abated after resumption of treatment. In 13 children, liver function worsened significantly when ursodeoxycholic acid was discontinued. T1 versus T0 concentrations expressed as multiples of the upper limit of the normal range (in parentheses) were as follows: alanine aminotransferase, 3.0 xN (0.8-7.0) vs 1.5 xN (0.5-5.4); gamma glutamyl transpeptidase, 8.0 xN (1.8-30.2) vs 4.2 xN (0.5-27.4); and aspartate aminotransferase, 1.7 xN (0.7-6.0) vs 1.3 xN (0.6-3.4). When ursodeoxycholic acid treatment was resumed, liver function had improved in all patients by T2. Concentrations of endogenous bile acids tended to be elevated at T1 (not significant) and were significantly decreased at T2. CONCLUSION: Our study demonstrates the beneficial effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia.

Advantages of intraoperative semiquantitative evaluation of myenteric nervous plexuses in patients with Hirschsprung disease.

BACKGROUND: The reappearance of an occlusive syndrome after surgical treatment of patients with Hirschsprung disease is often caused by incomplete resection of the affected segment. Intraoperative examination of frozen biopsies assists surgery, but interpretation of biopsies in the transitional zone is difficult. METHODS: We performed retrospective semiquantitative evaluations of myenteric nervous plexuses at the proximal limits of resection specimens from 41 children who were treated for rectocolic Hirschsprung disease using intraoperative examination of transparietal biopsies. The results of the evaluations were correlated with the postoperative courses of the children. RESULTS: After formalin fixation, the mean length of the aganglionic segments was 8.9 cm (range, 1-37.5 cm). At the proximal limit, the percentage of the circumference of the muscularis propria that was composed of plexuses with ganglion cells was more than 95% in 7 children, 90% to 95% in 21 children, 40% to 80% in 6 children, and 30% or less in 7 children (17%), 4 of whom subsequently developed occlusive syndromes. CONCLUSIONS: Semiquantitative intraoperative examination of the entire circumference of resected segments facilitates precise evaluation of intrinsic innervation at the level of the anastomosis and may reduce the frequency of recurrence of occlusive symptoms after surgery.

Electro-stimulation in preterm neonates with congenital auriculo-ventricular block. Report of three cases.

UNLABELLED: Congenital auriculo-ventricular blocks (CAVB) are usually linked to maternal auto-immune diseases and their management remains controversial. We report three cases of CAVB in preterm newborns managed using electro-stimulation with an external, followed by an internal pacemaker. Results and long-term follow-up were satisfactory in all three children. CONCLUSION: the transitory placement of an external pacemaker seems necessary in low birth weight infants in order to allow weight gain and therefore the insertion of a permanent pacemaker. The prognosis seems good but a regular echocardiographic surveillance is of great importance in order to detect the onset of cardiac failure secondary to dilated cardiomyopathy.