Prognostic evaluation of biofeedback response in patients treated for anorectal malformation.

PURPOSE: Functional bowel outcome in patients with anorectal malformation often is poor. For fecal incontinence resulting from sphincter dysfunction, biofeedback (BFB) training appears to be effective. The aim of study was to investigate the bowel function in incontinent children treated for ARM, using a clinical score, a manometric and pelvic magnetic resonance evaluation, in order to establish predictive parameters of response after BFB. METHODS: 25 children (median age of 6.5 years) with true fecal incontinence were evaluated by clinical score, anorectal manometry and magnetic resonance imaging (MRI). According to these evaluations patients were divided in 4 groups: group 1 (favorables manometry and MRI); group 2 (favorable manometry and unfavorable MRI); group 3 (unfavorable manometry and favorable MRI); group 4 (unfavorables manometry and MRI). All groups started a cycle of BFB and six months after end of BFB, were reevaluated by clinical score and manometry. RESULTS: The overall response to BFB was excellent in 44%, discrete in 40% and poor in 16%; a better response was found in groups 1 and 2 than groups 3 and 4. The differences between groups before BFB proportionally correlated with values after BFB; a correlation with genitourinary and spinal anomalies was found. CONCLUSIONS: Our results showed that BFB is an effective for fecal incontinence when the assessment pretreatment (functional and morphologic) is favorable; the manometry can evaluate the potential sphincterial recovery after BFB with a further prognostic benefit if correlated to morphologic evaluation with MRI.

Comprehensive laparoscopic approach to pediatric varicocele based on preoperative color Doppler ultrasound assessment.

BACKGROUND: This study aimed to assess whether laparoscopic treatment for any kind of varicocele is possible after preoperative identification of refluxing veins by color Doppler ultrasound (CDUS). METHODS: At the authors' institution, 98 patients with a median age of 11.3 years (range, 7.1-16 years) were evaluated for a left varicocele. Preoperatively, all the patients underwent ultrasound scan assessment of testicular volume and CDUS to rule out reflux into the internal spermatic vein (ISV), deferential vein, or cremasteric vein. In all the patients, laparoscopic division of the spermatic artery and veins was performed as close as possible to the internal inguinal ring. The other vessels were coagulated and divided if shown to be refluxing on CDUS. RESULTS: Color Doppler ultrasound showed reflux only in the ISV in 87 cases (88.7%), but in both the ISV and the deferential in the remaining 11 cases (11.2%). During a median follow-up period of 18 months (range, 6-49 months), none of the authors' patients experienced varicocele recurrence either clinically or according to CDUS scanning. The median left testicular volume increased significantly postoperatively. CONCLUSION: The proposed technique based on laparoscopic interruption of the ISV and testicular artery very close to the internal inguinal ring, meticulous CDUS assessment to rule out reflux in the deferential vein, and coagulation of refluxing deferential veins allows successful laparoscopic treatment of most varicoceles.

The hairy elbows syndrome: clinical and neuroradiological findings.

The hairy elbows syndrome (HES) is a rare congenital phenotype characterized by an abnormal increase in long hairs localized on the upper limbs extensor surfaces. This feature is often associated with short stature, facial asymmetry, dysmorphisms, intrauterine growth retardation (IUGR), and mental and speech delay. We report a case with hypertricosis cubiti associated with infantile spasms, behaviour disorders and cerebral hemisphere asymmetry. Although these findings have not been previously described we are uncertain whether they are unusual or underestimated. However, it is likely that these neurological findings are strongly interrelated leading to a more severe phenotype of the syndrome.

Primary repair in esophageal atresia. The results of long term follow-up.

AIM: The aim of this study was to assess the impact of postoperative morbidity during a long-term follow-up (6-12 years) in children with esophageal atresia treated at birth by primary anastomosis. METHODS: Fifteen children with esophageal atresia and tracheoesophageal fistula were surgically treated at birth and their follow-up was extended to at least 6 up to 12 years. Data included clinical examination, evaluation of nutritional habit, continuous video recording of barium esophagogram, esophageal manometry, 24-h esophageal pH-monitoring and esophageal endoscopy. RESULTS: All the 15 patients completed the clinical evaluation and the set of tests. In the first 6 years, mild dysphagia and gastroesophageal reflux (GER) was observed in 3 cases whereas GER without dysphagia in 4 cases. These 7 patients were informed about simple nutritional behaviours to minimize symptoms and treated with H2-blockers. At long-term twelve-year analysis, all patients were between 50 degrees and 75 degrees percentile of expected growth. It was not referred peculiar food restrictions. Five patients showed mild dysphagia with solid foods; early satiety, epigastric burning and regurgitation were less frequent. Furthermore they showed multiple non-peristaltic body contractions at esophagogram and moderate impairment of esophageal motility at esophageal manometry. The 24-h esophageal pH-monitoring showed normal patterns in all patients. No major lesions of esophageal mucosa were detected at esophagoscopy. CONCLUSIONS: Although GER and esophageal dysmotility are reported as frequent findings in patients who underwent primary repair for esophageal atresia, these disorders don't cause any relevant impairment to the quality of their nutritional habit.

Needle perforation of the bowel in childhood.

Accidental ingestion of foreign bodies occurs frequently in childhood. The majority of them are passed spontaneously, and conservative management generally is recommended for foreign bodies in the stomach and duodenum. However, in some cases, operative intervention should be considered to prevent undesirable complications, such as intestinal perforation. Two cases of intestinal perforation owing to accidental ingestion of a needle are reported.

Esophageal achalasia and hypertrophic pyloric stenosis associated with a phytobezoar in an adolescent.

Esophageal achalasia is an uncommon condition in children. The authors report on a 14-year-old girl who showed a very unusual association of cardiospasm and hypertrophic pyloric stenosis with a gastric phytobezoar.

[Neuroendocrine carcinoma of the skin]. / Il carcinoma neuroendocrino della cute.

The neuroendocrine carcinoma of the skin (NCS) is an uncommon disease, having been described about 400 cases in world literature. Two cases of NCS in a 52-year-old woman and in a 44-year-old man are reported; their histogenesis is still uncertain. It is advisable a wide cutaneous excision of the neoplasm extending 2-3 cm. over its macroscopic borders, in order to decrease the incidence of local recurrences, reaching 33% in some reports; when regional nodes are involved a lymphadenectomy is recommended. The authors also recommend a prophylactic radiotherapy of locoregional node chains, because of the high radiosensitivity of these tumors.

[Paratesticular rhabdomyosarcoma: presentation of a case load and considerations on the current therapeutic management]. / I rabdomiosarcomi paratesticolari: presentazione della casistica e considerazioni sugli attuali atteggiamenti terapeutici.

The Authors report their experience in the management of paratesticular rhabdomyosarcomas observed in the last 5 years (3 cases). Clinical and instrumental findings are analyzed; their therapeutic planes and suggestions of National Protocol RMS 88 are compared.

Role of the fourth complement component (C4) in the regulation of contact sensitivity. II. Qualitative differences between C4 molecules from high- and low-C4 mouse strains.

Lymph node cells collected from CBA/J mice 4 days after painting with picryl chloride induce contact sensitivity in naive recipient mice by virtue of hapten IgM immuno complexes. The immunizing capacity of these cells ("4-day" cells) is abolished after incubation of the cells with a C4-deficient guinea pig serum reconstituted with plasma or purified C4 from mice with high C4 levels (C4h), but not with plasma or purified C4 from mice with low C4 levels (C41). The inhibition of the immunizing capacity of 4-day cells is due to the activation of the early components of the classical complement pathway which is likely to result in the solubilization of membrane-bound immunocomplexes. However, the same amounts of CBA/J and BALB/c C4 have a different effect in inhibiting the induction of contact sensitivity by 4-day cells. In fact, by dose-response experiments, we have found that the amount of C41 able to inhibit the induction of contact sensitivity is about threefold higher than that of C4h. Analysis of the covalent binding ability of C4h and C41 reveals that C4h is able to bind to the surface of 4-day cells more efficiently than C41 and this probably accounts for the difference of the two C4 molecules in inhibiting the immunizing capacity of 4-day cells. Results are discussed in terms of different reactivities of C4h and C41 with the surface of 4-day cells.