The surgical approach to oesophageal achalasia.

The term "oesophageal achalasia" describes a neuropathic disorder characterized by abnormal motility of the oesophagus and incomplete or absent relaxation of the lower oesophageal sphincter. In these patients with "paroxysmal" dysphagia, barium swallow and manometric study confirm the diagnosis. In our opinion, the treatment of choice is extramucosal tardiomyotomy (Heller) which should be followed by gastric fundoplication in order to protect the mucosa and prevent gastrooesophageal reflux. We present our experience in the laparoscopic approach to Heller cardiomyotomy in children. An anterior 180 degrees hemi-fundoplication, according to Dor technique, is performed suturing the left and right oesophageal muscular margin to the gastric wrap. A manometric examination is mandatory in order to detect the complete incision of the lower oesophageal sphincter and to confirm the creation of the new-high pressure zone. This preliminary experience confirms that the laparoscopic approach can be used for the treatment of oesophageal achalasia also in children.

[Protective efficacy of 3 anesthetic methods with reference to surgical stress in children]. / Efficacité protectrice de trois méthodes anesthésiques à l'égard du stress chirurgical chez l'enfant.

Hormonal-metabolic stress responses have beyond doubt an effect on morbidity/mortality related to surgery. The present study med to determine which anaesthetic technique could afford the best protection in children, through analysis of the perioperative cortisol, prolactin and beta-endorphin plasma levels. Thirty-six young patients 3-10 years old, ASA I-II, scheduled for hypospadias or vesicoureteral reflux surgery of a duration > 60 min, were randomized into three groups (n = 12). Children of group I were given initially propofol and fentanyl then isoflurane 1%; group 2 received TIVA with propofol and fentanyl, group 3 received initially propofol then an epidural lumbar block with bupivacaine 0.25% (single shot) and continuous propofol i.v. infusion. Cortisol, prolactin and beta-endorphin levels increased significantly in group 1 only. No significant differences were observed between group 2 and 3. Early postoperative analgesia was better in group 3. These data suggest that TIVA and particularly epidural block could afford a better protection against the surgical stress in children submitted to subumbilical operations.

[Gastroesophageal reflux disease and respiratory disease]. / Malattia da reflusso gastro-esofageo e patologia respiratoria.

The patients treated for oesophageal atresia present a correlation between the clinical sintomatology after recanalization characterized by disfagia, dispnea, recurrent cough, chronic pneumopaties and oesophageal anomalies. Where morphological alterations accounting for the presence of gastro-oesophageal reflux (GOR) were not evident, possible functional alterations of the motility were considered. The incidence of GOR was considerably high and, expression of a congenital alteration of the lower oesophageal sphincter and of oesophageal peristalsis, becomes even more severe due to further stretching of the gastro-esophageal junction. The authors underline that the early demonstration of histological changes, even before recanalization, and the motility disorders of the oesophagus have to be well studied, while the LES is normalized, in order to prevent and treat the possible appearance of the well-known complications of GOR.

Neuronal intestinal dysplasia: clinical experience in Italian patients.

The authors present a review of 431 children biopsied and studied with the following histochemical and immunohistochemical techniques: 1) acetylcholinesterase activity; 2) alphanaphthylesterase activity; 3) S-100 protein immunohistochemical technique; 4) glyoxylic acid method. Two hundred forty-eight patients of our series presented different forms of dysganglionosis, 12 of them (4.8%) presenting neuronal intestinal dysplasia type B. In 7 cases, NID type B was diffuse, whereas in 5 recto-colonic NID type B was confined to the splenic flexure. Male:female ratio was 9:3. Familial recurrence was present in 2 of the 12 cases of our series, affected by severe neuronal intestinal dysplasia extended to the small intestine, associated with intestinal malrotation and short bowel syndrome. Four of the 7 cases of diffuse NID type B and 2 of the 5 cases of rectocolonic NID type B were surgically treated. Three patients with diffuse NID died from sepsis within the 2nd year of life. This study confirms that NID type B is a form of dysganglionosis which can be diagnosed in a Mediterranean country if histochemical techniques are applied in the study of a large series of constipated and pseudo-Hirschsprung patients. From a pathogenetic point of view, the authors compared the histochemical findings of biopsies from their series of NID patients with those of recto-colonic biopsies from patients with MEN II B syndrome. The similarity of GI symptoms in MEN II B and NID pediatric patients suggests that the two disorders could be the result of mutations affecting the same domain of the RET proto-oncogene.

Long-gap oesophageal atresia: a combined endoscopic and radiologic evaluation.

The final aim of the treatment of oesophageal atresia is to restore continuity without the interposition of intestinal segments. The authors present the results of a new diagnostic method combining endoscopy and radiology to evaluate the development of pouches in oesophageal atresia. This new method has been successfully adopted in four patients aged between 4 and 8 months, three with type I and one with type III complicated atresia. Direct end-to-end anastomosis was performed in all patients. We think this method can be easily applied and is a suitable diagnostic examination in all cases in which surgical strategy aims at restoring oesophageal continuity by means of direct anastomosis.

Leiomyosarcoma of the cecum in pediatric age: a case report and review of Italian reports.

The authors report a case of leiomyosarcoma of the cecum and review all the Italian reports of mesenchymal neoplasms registered in the RMS-Italy register. They emphasize the extreme rarity of intestinal leiomyosarcoma, especially with colon involvement: their case appears to be the only one registered in Italy in the last 12 years.

Autosomal recessive microcephaly with early onset seizures and spasticity.

We describe two siblings, a male and a female pair, born of consanguineous parents, affected with a rare genetic form of congenital microcephaly. The clinical syndrome is characterized by early onset myoclonic seizures, spasticity, and profound psychomotor retardation without detectable brain malformations. To date, only two kindreds and one sporadic case with a similar clinical picture have been observed and reported (Tolmie et al. 1987, Bundey & Griffiths 1977). The severity of the neurological features and their perinatal onset differentiate the syndrome from the more common autosomal recessive microcephaly with spasticity/seizures.

Hypertrophic gastropathy with transient sessile polyps.

We report an 8-year-old boy with hypertrophic gastropathy (HG) associated with duodenal Giardia lamblia infestation. The follow-up was complicated by the development of gastric polyps at the site of previous biopsies that spontaneously disappeared within 15 months. Despite the histological similarity, the different course between Ménétrier's disease (MD) in adults (chronic, with frequent development of sessile or pedunculate polyps) and HG (uncomplicated and usually spontaneously resolving) suggests a different pathogenesis. Viral (cytomegalovirus) and bacterial (Helicobacter pylori) infections have been described in association with HG and they could play an important pathogenetic role. The term HG better defines the childhood disease in which a conservative management is recommended.

[Unusual pelvic pathology treated with posterior sagittal anorectoplasty]. / Patologia pelvica rara trattata secondo i principi della PSARP.

Posterior sagittal approach is very useful for the correction of anorectal malformations, but it can be used also for the treatment of pelvic or perineal masses. The authors present 3 patients affected one by a perineal hamartoma, one by a rectal duplication and one by a retrorectal cystic teratoma. All the patients were treated following the PSARP technique, using the Penã's bipolar electro-stimulator just in order to respect all the muscles of the anorectal sphincters. Using this procedure the authors were able to preserve the full anorectal continence in all the 3 patients.

Leiomyomatosis of oesophagus, congenital cataracts and hematuria. Report of a case with rectal involvement.

Isolated oesophageal leiomyomatosis in children is very rare. The association between leiomyomatosis of oesophagus and female genital tract and an Alport like hematuric nephritis has been recognized as a distinct entity by Garcia-Torres and Guarner. Since then few other cases of this syndrome have been observed. Congenital cataracts and neurosensory deafness have been added to the clinical spectrum. Autosomal dominant inheritance is suggested by the familial cases. We report a patient who presents, as a new finding, leiomyomatosis of the rectum.

Adenocarcinoma of the rectosigmoid colon in a 10-yr-old child.

The authors describe a case of adenocarcinoma of the rectum in a 10-yr-old boy. The clinical picture, the surgical technique, and the histology are described, an reference is made to the small number of similar cases appearing in the world literature.