Determination of the anal position in newborns and in children with chronic constipation: comparative study in two European healthcare centres.

INTRODUCTION: The objective of this study was to determine the normal anal position index (API) in neonates and in a group of older children with chronic constipation, and to identify the incidence of anterior ectopic anus (AEA) in the two groups. MATERIALS AND METHODS: A prospective random study was carried out in two European hospitals determining the API in 1,012 neonates (529 in Group A, and 483 in Group B) and in 64 children suffering chronic constipation of ages between 3 months and 12 years (Group C). The API is defined as the ratio between the fourchette-anal and fourchette-coccygeal distances in girls and the scroto-anal and scroto-coccygeal distances in boys. The gestational age, weight, meconium evacuation and anal calibre were recorded in Groups A and B, and age, sex, and API in Group C. Statistical comparisons were made using Student's t test and Welch's t test for independent sample groups. RESULTS: The mean API in Group A was 0.53 ± 0.06 in males and 0.40 ± 0.05 in females, and in Group B 0.51 ± 0.06 in males and 0.39 ± 0.08 in females, with significant differences between the sexes (p < 0.0001) in both groups; 1.7% of the neonates in Group A and 2.4% of Group B presented AEA. 4/13 newborns females with AEA presented with severe constipation during their first months of life. For the 64 patients in Group C: 59.3% were female; the mean API was 0.47 ± 0.1 in males and 0.36 ± 0.1 in females (p < 0.0001). The incidence of AEA was 47% in females and 35% in males. CONCLUSIONS: In the neonates (Groups A and B), the position of the anus in the perineum was more anterior in females than in males (p < 0.0001); 31% of the newborns females with AEA presented with constipation in their first months of life. In the Group C patients, there was a high incidence of AEA, especially in the females, and we consider it to be the probable cause of this defecation disorder.

Anorectal malformations (ARM): quality of life assessed in the functional, urologic and neurologic short and long term follow-up.

UNLABELLED: Imperforate anus represents a wide spectrum of anorectal malformations associated with urologic, neurologic and orthopedic malformations. The outcome from the various corrective measures has improved due to new surgical techniques as well as to a better understanding of the pathology. Other factors which influence the overall outcome include the degree of patient acceptance, family support as well as the ability of the health care structure to support the patient's needs on a clinical, functional and psychologic level. AIM OF THE STUDY: Assess (with the new techniques available) the quality of life in the adult patient with ARM and compare it to that experienced by a younger patient; also we wish to determine the correlation between the observed abnormalities with the functional, neurologic and urologic outcome. MATERIALS & METHODS: Sixty-six patients were subjected to PSARP (36 M; 30 F). Six presented with cloaca and 60 with ARM (23 high and 37 low). All patients underwent the same workup to include L/S MRI diagnostics, evaluation for incontinence (urinary and bowel), a urology screening, and if required, a subsequent urodynamic study with rehabilitation and/or bowel management. All answered questionnaires (AIMAR: Italian parent's association of ARM) in order to assess their satisfaction with the current health condition, with the information received and with the treatment and follow-up sessions. The patients were classified into one of two groups. Group A, totaled 33 patients (4 cloacae) with an age range between 2 and 12 years who were operated after 1995. The second, group B, was made up of 33 patients who had been surgically treated before 1995 (age range 15-41 years), had followed the study protocol and had also a neuropsychiatry consult. RESULTS: Overall fecal continence was 69% and of this number 37% were clean without constipation. Twe2nty-one patients (32%) suffered from some form of constipation. Constipation was the most common functional disorder observed in patients who have undergone PSARP. The highest incidence of constipation was found in the ARM (low type), a favorable prognostic group with 43% constipation. Patient with "high" defects and a cloaca had a lower incidence of constipation (18%). Of the 59 patients evaluated, 85% were urinary continent and 15% were incontinent. All of the incontinent patients were in the unfavorable prognostic group of malformations. Urodynamic studies showed 7 neurogenic bladders (NB) and 2 patients with a neurovescical dysfunction (NVD). Of the 50 "dry" patients. 20 had voiding disturbances due to a voiding dysfunction, in the absence of neurologic abnormalities, and presented occasional daytime or nighttime wetting. There was no correlation between the level of the anatomic defect and the urodynamic patterns in the group. Abnormal MRI findings were observed in thirty out of fifty-two patients evaluated. The MRI findings were classified as follows. Severe abnormalities: 7 patients (13%) presented with a combination of skeletal (sacral/lumbar) and spinal cord anomalies. Only spinal cord abnormalities: 12 patients (21%). Only skeletal abnormalities: 11 (19%) patients. Patients were divided into high, low and cloacal malformations. A high degree of statistical correlation was noted between the patients belonging to the cloacae and high defect groups and the abnormal MRI findings. No significant correlation was found between the low defect group and dysrafism, abnormal MRI results and the severity of the malformation. The incidence of Tethered Cord (TC) in our limited number of patients was limited in our study (9% in the high and 7% in the low defect group) when compared to the current literature. Furthermore there was no statistically conclusive evidence that TC by itself affects the urinary or fecal control in our patients. Our recommendation is nevertheless to obtain an MRI study in all patients with ARM. CONCLUSION: All patients 17 and older reported a "good quality of life". Four are married, two with children. Aclose working relationship with the medical personnel is not only necessary but is also well received by the family particularly when younger patients are involved. The adult patient easily adapts even when information is initially scarce. He quickly reaches autonomy with personalized solutions but prefers a longer follow-up time during which, specialized medical facilities will play an important role in the treatment of ARM. Our findings illustrate the importance of both global disease-specific functioning and perceived psychosocial competencies for enhancing the QL of these patients.

[Serious dog bite lesions in childhood: presentation of 3 cases and literature review]. / Lesioni gravi da morso di cane in età pediatrica: descrizione di 3 casi e revisione della letteratura.

Animal bite lesions in the pediatric patient are mainly accounted for by dog bites in 80% to 90% of the cases. They often present a favorable prognosis but serious lesions do account for 5% to 20% of the total incidence. We will present three particular clinical cases which required urgent surgical treatment as well as a review of the current literature to include both medical and surgical treatment methods for this kind of lesion.

Pneumatosis cystoides intestinalis: a rare cause of nonsurgical pneumoperitoneum in an infant.

The authors describe a 3-year-old boy with pneumatosis cystoides intestinalis (PCI) and associated duodenal stenosis who came to them with abdominal pain, significant abdominal distension, large gastric residue, and pneumoperitoneum. PCI is a rare condition in children characterized by the presence of multiple gas-filled cysts within the wall of some part of gastrointestinal tract that may break and cause pneumoperitoneum without an intestinal perforation. Even if in most cases the standard treatment is surgical, to keep in mind this rare condition could be useful to adopt a most rational treatment approach and avoid unnecessary operation in case of "benign" pneumoperitoneum.

Successful use of transureteroureterostomy in children: a clinical study.

The purpose of this review of patients was to look at the current role of transureteroureterostomy in children. In addition, this study should give an indication of the long-term safety of transureteroureterostomy in children with neurogenic bladder. From 1972 on, 70 subjects between the ages of 2 and 13 underwent transureteroureterostomy. In 68 patients (97 %), transureteroureterostomy was used as a salvage renal procedure after unsuccessful ureteroneocytostomy attempts at other institutions. In 2 patients (3 %), transureteroureterostomy was used as a diversion procedure. The postoperative course was uneventful except for 1 case of early anastomotic occlusion successfully treated by application of a double-J stent. No patient required surgical revision of the ureteral anastomosis. However, 4 patients with neurogenic bladder from myelomeningocele developed sequelae. Of these, 1 presented with stenosis at the end of the recipient ureter, 2 had occurrence of vesicoureteral reflux in nonreimplanted recipient ureter, 1 developed calculi in the donor pelvis. This study supports the view that transureteroureterostomy is highly useful for the management of children with a wide spectrum of urological anomalies. However, long-term monitoring of patients with neurogenic bladder who have undergone this procedure is essential to achieve a long-term good outcome.

[Severe chronic constipation: limitations of medical therapy and indications for surgical intervention. II]. / Stipsi cronica severa: limiti della terapia medica e indicazioni all'intervento chirurgico. Parte seconda.

Severe chronic constipation is a common problem in children. Most patients have functional constipation, only few (approximately 5%) have an underlying organic disease who requires surgical treatment. Anatomic problems with a painful defecation (anal rhagades, fistulas, hemorrhoids) or with difficult defecation (anal stenosis, tumor) require only a careful physical examination. Other organic problems due to neurogenic and myogenic colonic abnormalities require extensive investigations, including histological and histochemical study, for diagnosis. Ultrashort Hirschprung's disease, neuronal intestinal dysplasia type B and hypoganglionosis represent different levels of a developmental problem of the enteric nervous system. Surgical treatment is recommended in these cases. After surgery is recommended a long period of medical treatment to avoid a functional constipation.

[Fecal incontinence in pediatric age: diagnostic-therapeutic protocol and possible surgical procedures]. / Incontinenza fecale in età pediatrica: protocollo diagnostico-terapeutico e prospettive chirurgiche.

Fecal incontinence or soiling always endangers the personality development of a children. For very severe cases, the patient will be limited on his educational and professional training as well as on his sexal life. The outlook of severe cases, the chances of completing school, the choice of professional training and of sexual life in the future will be limited. A corrective procedure for anorectal incontinence depends on the exact investigation of all factors contributing to continence. The aim of this paper is to analyze the diagnostic aspects concerning continence in order to choose for each patient the most appropriate procedure for the treatment of all disturbance of stool control. A long term review of 312 patient with MAR, Myelomeningocele and severe stipsis with soiling was undertaken to determine the level of continence achieved. The choice between conservative measures and surgical procedures depends on clinical and manometric findings.

[Cryptorchidism with didymo-epididymal dissociation and Robinow's syndrome: 2 case reports]. / Testicolo ritenuto con dissociazione didimo-epididimaria e sindrome di Robinow: presentazione di due casi.

Two brothers of normal not consanguineous parents, with bilateral intrabdominal cryptorchidism, were admitted to our Institution. Both children had short stature, limb and hand malformations and craniofacial patterns of Robinow syndrome. During the orchidopexy, bilateral epididymal and vasal abnormalities were found in both of them. This anomaly associated with Robinow syndrome has never been reported before. These two cases provides the Authors with the opportunity of reviewing clinical features, genetics and radiological patterns of this rare syndrome.

[Cystic-papillary neoplasm of the pancreas: a case report in childhood]. / Neoplasia cistico-papillare del pancreas: descrizione di un caso in età pediatrica.

A solid and cystic tumor of the pancreas in a 11-year-old girl is reported. The child presented with abdominal pain and vomiting. A voluminous tumor, arising from the pancreas tail, was removed with distal pancreatectomy. These neoplasma occur predominantly in girls and young women, the prognosis is usually good in spite of histologic finding of apparent malignancy. The histochemical, immunohistochemical and electron examinations of this tumor show polymorphic differentiation, which means that part of the all can differentiate into duct epithelium, acinar or endocrine line. So up to, recently this tumor has been misclassified within other pancreatic tumors, and frequently has been managed with aggressive surgery. But as the neoplasm usually behaves like a very low grade malignancy, its complete removal is the treatment of choice for the tumors arising anywhere in the pancreas. The authors emphasize the importance of accurate diagnosis and of extirpative surgery in children with pancreatic malignancy.

[Non-invasive urodynamic approach to the diagnosis, treatment and follow-up of voiding disorders in pediatric patients]. / Approccio urodinamico non invasivo nella diagnosi, trattamento e follow-up dei disturbi minzionali in età pediatrica.

The authors reports their experience on 248 patients affected by minctional disorders isolated or related to UTI (upper tract infections) and VUR (vesico-ureteral reflux). All the patients were assessed throught a predominantly non invasive diagnostic approach which included: pediatric urologic examination with aimed anamnestic and clinical freaming, functional examination of the lower urinary tract using uroflowmetry + EMG of the perineal plane muscles (UR + EMG), kidney and bladder ultrasound. This methodology has permitted a widening of the indications in the study of vesical function as well as limiting the selected cases (hight UTI, uncertain diagnosis) of mini-invasive examination, such as flow pressure study, minctional cystourethrogram (MC) and or renal scintigraphy. An MNR of the lumbo-sacral medulla (cord) and a neurological and/or neurosurgery evaluation were only carried out were there was a suspected occult neurological pathology. The clinical sintomatogy was as follows: approximately 70% of the patients suffered from partial diurnal incontinence, 42% were affected by secondary nocturnal enuresis while 58.6% suffered from recurrent UTI. In those patients with UTI, 11% (16 patients, 24 ureteral units) suffered from associated VUR while 3.5% suffered from either congenital or acquired urethral stenosis. From the urodynamic examination, we determined the presence of detrusorial instability in 158 patients (64%) and lazy bladder or vescical hypotonia in 84 patients (34%). The suggested therapy foresees the use of: hospital home-based uroriabilitation (minctional biofeedback), endoscopic therapy (sub-ureteral bulking, urethral dilatation) and corrective VUR surgery (only in those cases that did not respond to medical treatment). The percentage of total recovery in patients with detrusorial instability was 80%, the minctional biofeedback both associated and unassociated with drug therapy lead to complete recovery in 66% of patients with lazy and uncordinated bladder, wile 26% showed improvement and 7 patients (8.4%) did not respond. All the patients with VUR (24 reflux units) recovered; and endoscopic therapy and or surgery (12 reflux units) was carried out in some of this patients.

[Retroperitoneal nodular fasciitis: analysis of a case]. / Fascite nodosa a sede retroperitoneale: analisi di un caso.

A case of nodular fasciitis occurring in a 14-year-old girl is described and the clinical-pathological profile and the diagnostic problems are analyzed. The Authors believe this report useful to be reported for the relative rarity of the disease, especially in children, the very great dimension and the unusual retroperitoneal localization.

[Testicular and paratesticular tumors in children]. / Tumori dei testicoli e dei tessuti paratesticolari in età pediatrica.

Testis tumors in children occur infrequently and exibit differences in their histopathology, clinical behaviour and therapy from their adult counterparts. From 1979 to 1994, 17 children and adolescent with testicular tumors were treated at the Pediatric Surgical Department of Vicenza Regional Hospital. Paratesticular rabdomiosarcoma were present in 3 cases, 4 patients had embrional carcinoma, 1 Sertoli cell tumor, 2 Leydig cell gonadal stromal tumor, and leukemic infiltrates of the testis were clinically evident in 7 patients. We report our clinical series and discuss in relation to clinical characteristic, histopathology and therapy and conclude that the improved survival during the past decade is attributable to better diagnostic imaging thecniques, the availability of serum tumor markers to monitor disease activity and more effective chemotherapy.

[Ovarian tumors in children]. / Tumori ovarici in età pediatrica.

Ovarian tumors in children are uncommon. We reviewed all cases of ovarian tumors treated at the Department of Pediatric Surgery of Vicenza Regional Hospital from 1979 to 1994. They include 6 cases of cystic teratoma and 1 gonadoblastoma; the malignant lesions included 1 immature teratoma, 1 dysgerminoma, 1 yolk-sac tumors and 1 patient with ovarian infiltration from malignant leukemia. Only the patient with leukemia died of progression of the disease. These data show that most ovarian masses are benign. Germ-cell tumors are the most common malignancy and epithelial cell tumors are less likely. Epithelial cyst and teratomas are the most common benign lesions. Although ultrasound was 100% accurate in the diagnosis of ovarian pathology, the ultrasonogram could not distinguish between benign and malignant lesions.

[Neonatal chylous ascites: a case report and review of the literature]. / Ascite chilosa neonatale: descrizione di un caso e revisione della letteratura.

The case of a newborn with asymptomatic chylous ascites operated on because of voluminous inguino-scrotal hernia is reported and a review of the recent literature about aetiology, diagnosis and treatment of neonatal chylous ascites is made.

Management of severe blunt pancreatic trauma in children. Personal experience.

Blunt abdominal trauma is the most common cause of pancreatic injuries in children. Non-operative management of traumatic injuries in the absence of complete duct transection is safe in children and does not appear to be associated with adverse sequelae. The purpose of this report is to describe the diagnostic techniques, clinical management and survival of 9 children with traumatic injuries of the pancreas treated in our institution over a period of 7 year.

The Pediatric Bowel Management Clinic in children with severe chronic constipation. Personal experience.

The multifactorial nature of functional constipation in children suggests that a multidisciplinary management approach may be effective and leads to both patients and parents' satisfaction and significant short term improvement. The authors describe their experience with the study and treatment of children with severe chronic constipation in a newly-created Pediatric Bowel Management Clinic (BMC). Further studies will examine the long-term impact of the clinic.

Juxta-adrenal malignant schwannoma.

A case of malignant schwannoma occurring in an 11-year-old boy is described. To our knowledge, this is the third case of malignant juxta-adrenal schwannoma reported in the literature. It was misdiagnosed at the onset of the disease as a post-traumatic renal hematoma. Angiography revealed that the tumor derived its blood supply from collateral vessels of the aorta, splenic, and left gastric arteries. An analysis of the diagnosis, clinical course, and prognostic factors of this rare tumor is performed.