Laparoscopic gastropexy for the treatment of gastric volvulus associated with wandering spleen.

A 2.5-year-old boy was referred to the emergency room for a sudden onset of diffuse and increasing abdominal pain with lethargy, abdominal distension, and vomiting, all in the past 24 hours. A plain abdominal X-ray showed gastric distension. Two liters of gastric contents were evacuated by suction. The abdominal sonogram showed an unusual position of the spleen in the left-lower quadrant, with no splenic ischemia. The diagnosis of gastric volvulus associated with a wandering spleen was then evoked. Laparoscopic exploration revealed a nonischemic spleen, absence of normal supporting ligaments for the spleen, and gastric distension with flaccid gastric walls. The spleen was then easily moved in the left-under quadrant. A parietal peritoneal posterolateral incision was made, opposite the large gastric curve, up to the diaphragm (7 cm). This delimitated a sharp demarcation zone between the two edges of the incised peritoneum. The stomach was fixed to the peritoneal incision, covering and anchoring the spleen in a good position. Recovery was uneventful, and an abdominal sonogram performed 4 years after the surgery shows a viable spleen in its correct location. The rarity of gastric volvulus associated with a wandering spleen and its fast clinical improvement with medical treatment often delays the diagnosis and the surgical treatment. Laparoscopy in this case has a dual relevance: diagnosis and therapeutic management (splenectomy or gastropexy). Laparoscopic gastropexy for the treatment of gastric volvulus associated with a wandering spleen is an easy procedure and combines the advantages of all the surgical techniques previously described.

Thoracic skeletal anomalies following surgical treatment of esophageal atresia. Lessons from a national cohort.

INTRODUCTION: Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity. MATERIALS AND METHODS: Pediatric surgery departments from our national network were asked to send recent thoracic X-ray and operative reports for patients born between 2008 and 2010 with esophageal atresia. The X-rays were read in a double-blind manner to detect costal and vertebral anomalies. RESULTS: Among 322 inclusions from 32 centers, 110 (34.2%) X-rays were normal and 25 (7.7%) displayed thoracic malformations, including 14 hemivertebrae. We found 187 (58.1%) sequelae of surgery, including 85 costal hypoplasia, 47 other types of costal anomalies, 46 intercostal space anomalies, 21 costal fusions and 12 scoliosis, with some patients suffering from several lesions. The rate of patients with these sequelae was not influenced by age at intervention, weight at birth, type of atresia, number of thoracotomy or size of the center. The rate of sequelae was higher following a classical thoracotomy (59.1%), whatever the way that thoracotomy was performed, compared to nonconverted thoracoscopy (22.2%; p=0.04). CONCLUSION: About 60 % of the patients suffered from a thoracic wall morbidity caused by the thoracotomy performed as part of surgical treatment of esophageal atresia. Minimally invasive techniques reduced thoracic wall morbidity. Further studies should be carried out to assess the potential benefit of minimally invasive approaches to patient pulmonary functions and on the occurrence of thoracogenic scoliosis in adulthood. LEVELS OF EVIDENCE: Level III retrospective comparative treatment study.

Thoracoscopy and solid tumors in children: a multicenter study.

PURPOSE: The aim of this study was to evaluate the efficacy and safety of thoracoscopy in the treatment of solid thoracic tumors in children. METHODS: A retrospective, multicenter review of 139 thoracoscopies was performed. The procedures were either resection of pulmonary lesions or biopsy. Complications, histologic biopsy results, and outcomes were reviewed. RESULTS: One hundred thirty-nine procedures were performed in 134 children. There were 72 males and 62 females, with a mean age of 9.2 years (3 months to 17 years). The mean follow-up was 4.5 years. Thirty-eight primitive tumors were managed, 20 of which had a complete thorascopic resection, and nine biopsies were performed. In 9 additional cases, an open conversion was necessary. Among the 20 complete resections, 17 of the lesions were neurogenic tumors (i.e., neuroblastoma or ganglioneuroma). Three complications occurred: 2 chylothoraces and 1 with Horner syndrome. All three complications regressed. The mean follow-up was 3 years, and no recurrence has been noted. Fifty-three metastatic lesions were managed: 29 had a complete resection, 11 had a biopsy, and an open conversion was necessary in 13 cases. Among the complete resections, more than half were metastases from bone tumors. The mean follow-up was approximately 4 years and 5 pulmonary recurrences developed. Four deaths occurred, with 2 having had lung metastases. Forty-eight malignant hemopathies were managed; 9 biopsies for primitive tumors were performed. There were 36 secondary lesions, of which 5 were completely resected; 30 were biopsied and 1 required an open conversion. CONCLUSIONS: Thoracoscopy is a safe, effective approach for the evaluation and resection of solid lung tumors and for biopsy. Thoracoscopic resection of metastatic lesions is reasonable for nephroblastoma, but a thoracotomy is suggested for other metastases.

Botulinum toxin injections in the management of non-neurogenic overactive bladders in children.

INTRODUCTION: Non-neurogenic detrusor overactivity in children leads to varying degrees of functional impairments (urinary urgency, pollakiuria, urge incontinence, nocturia). Botulinum toxin has shown its effectiveness in the management of detrusor overactivity in neurological patients. OBJECTIVES: To evaluate the relevance of intravesical Botulinum toxin injections for the treatment of non-neurogenic overactive bladders in children. These pediatric patients were resistant to all the usual therapeutics (e.g. bladder/bowel rehabilitation, anticholinergic drugs, management of diet/hygiene habits and constipation, percutaneous posterior tibial nerve stimulation). MATERIALS AND METHODS: 8 children (mean age: 12.5years), 5 girls, 3 boys with daytime and/or nighttime incontinence and non-neurogenic detrusor overactivity validated by urodynamic testing. Urodynamic testing was conducted before the injections as well as 6weeks and 1year post injections. We used Dysport® 8 Speywood Units/kg injected via cystoscopy into 25 different sites. RESULTS: We noted improvements without any complaints during bladder voiding for all patients, in 6 patients the overactivity disappeared after 1 injection. Compliance was improved early-on in half the cases and at 1year for all cases (from 12% to 61%, p=0.01). Noninhibited contractions decreased constantly in both frequency and intensity. Clinical symptoms improved: mean of 7.75 daytime urinary incontinence episodes (IE) per week before the injection vs. 3 after the procedure (p=0.04). For nighttime IE the improvement was even more noticeable with 7.38 nighttime IE episodes per week before the injection vs. 2.06 after the procedure (p=0,02). CONCLUSION: Intradetrusor Botulinum toxin injections are a potential therapeutic option for the management of non-neurogenic detrusor overactivity in children resistant to the usual treatments.

Management of brachial artery aneurisms in infants.

Brachial artery aneurisms in children under 1 year of age are very rare. The main risk is distal ischaemic complication. We report four infants suffering from brachial artery aneurism of unknown origin. In all cases we used Doppler ultrasonography to validate the clinical diagnosis. Pre-operative vascular check-up was negative for other aneurismal location. Surgical excision with direct end-to-end anastomosis was possible in one patient; the others required interposition of an autologous venous graft. At discharge, patients were given oral aspirin for a few weeks. Histological examination revealed one pseudoaneurism and three true aneurisms. There were no complications either postoperatively or at 18 months follow-up. Arterial ligation might be indicated in only two situations: aneurism distal to profunda brachii artery, or chronic wall thrombus completely occluding (but distal perfusion through a neovascularization must be assessed first on angiography). Surgical excision with arterial reconstruction is the standard treatment. Endovascular treatment is not suitable because such a procedure in an infant would generate excessive radiation exposure, and a risk of stent migration with limb growth. In the case of an initial isolated and idiopathic presentation, or of false aneurism, clinical follow-up at 1 year is sufficient. In the case of secondary lesion, multiple initial presentation or relapse, life-long follow-up with repeated corporal imaging should be performed.

Deletion 2q36.2q36.3 with multiple renal cysts and severe mental retardation.

Interstitial 2q36 deletion is a rare event. We report on a patient with a de novo del(2)(q36.2q36.3) interstitial deletion of the long arm of chromosome 2 diagnosed by classical banding. The phenotype comprised facial dysmorphism, enlarged kidneys with multiple renal cysts, abnormal minora labia, asymmetric lower limbs with dysplastic patella, and severe mental retardation. By physical mapping, using array-comparative genomic hybridisation (CGH) confirmed by Fluorescent In Situ Hybridisation (FISH), the breakpoints of the deletion were mapped and the size of the deletions was measured: 5.61+/-0.19Mb. A skin biopsy was analysed using electronic microscopy showing an alteration of the structure and organisation of the dermal and peri-neuronal basement membrane. The relation between the phenotype and the deletion of both COL4A4 and COL4A3 genes, located in 2q36.3 loci, as well as the disruption of TRIP12 were discussed.