Anorectal malformations and neurospinal dysraphism: is this association a major risk for continence?

BACKGROUND/PURPOSE: Anorectal malformations (ARM) are a spectrum of defects ranging from the very minor ones, with an excellent functional prognosis, to those that are more complex, often associated with other anomalies, difficult to manage and with a poor functional outcome. A significant number of these children suffer from fecal and urinary incontinence despite major advances in the management of ARM patients have significantly improved the quality of life. The role of sacrovertebral anomalies/dysraphism (SD) and neurospinal cord anomalies/dysraphism (ND) associated with ARM on the continence of these patients is still controversial. The authors made a review of their experience in a period of 5 years, focusing on the role of neurospinal cord anomalies in patients with ARM. MATERIALS: At colorectal clinic of our department of pediatric surgery 215 patients who underwent a procedure of posterior sagittal anorectoplasty for ARM are followed-up in a multidisciplinary clinic. Among them 60 patients with either SD or ND were documented. In 37 patients the anomaly involved the spinal cord (ND). 12 of these 37 patients underwent neurosurgical treatment and 25 were managed conservatively. Data collected from their follow-up were analyzed and compared, focusing on their bowel and urinary continence. RESULTS: All 37 patients acquired regular bowel movements with an appropriate bowel management according to Peña's protocols. Urinary incontinence required clean intermittent catheterization in four cases. None of the patients who did not receive neurosurgical treatment developed acute complications due to the progression of the neurospinal anomaly, like acute urinary retention, orthopedic and motility problems or acute hydrocephalus. From literature review we were unable to find good evidence that the presence of ND worsens the functional prognosis of patients with ARM. We were also unable to find convincing evidence to support the practice of prophylactic neurosurgical procedures. CONCLUSIONS: The present study supports the theory that for ARM patients the prognosis in terms of continence depends mainly on the type of malformation and is not complicated by the association with ND. In our series neurosurgical treatments did not have any effect in improving the continence of ARM patients and a conservative management of ND did not expose the patients to the sequelae of progressive deterioration, reported elsewhere, requiring rescue neurosurgery. We believe that the correct practice of pediatric surgeons following-up ARM patients is a protocol which includes appropriate investigations to detect the presence of a SD or ND and, once these entities are detected, it is mandatory to manage the patient with a multidisciplinary team, where a conservative non-operative management is initially justified and advocated in the absence of neurosensorymotor symptoms.

[Spontaneous regression of an adrenal mass detected in utero]. / Risoluzione spontanea di massa surrenalica diagnosticata in utero.

The case of a left adrenal mass detected by ultrasonography in a male fetus at the 37th week of gestation is presented. After an uneventful delivery at the 38th gw, the newborn was sonographically monitored, and during the follow-up clinical evaluations were performed, in order to make a differential diagnosis between an adrenal hemorrhage and a congenital neuroblastoma. VMA and HVA were within normal levels, and MIGB scan was negative. A MR of the the abdomen performed at the age of one month was suggestive for an adrenal hemorrhage in involution. We therefore decided to keep monitoring the mass by ultrasound, avoiding any surgical approach. The sonographic complete disappearance of the disease was documented at the end of the fourth month.

[Spontaneous regression of an adrenal mass detected in utero]. / Risoluzione spontanea di massa surrenalica diagnosticata in utero.

The case of a left adrenal mass detected by ultrasonography in a male fetus at the 37th week of gestation is presented. After an uneventful delivery at the 38th gw, the newborn was sonographically monitored, and during the follow-up clinical evaluations were performed, in order to make a differential diagnosis between an adrenal hemorrhage and a congenital neuroblastoma. VMA and HVA were within normal levels, and MIGB scan was negative. A MNR of the the abdomen performed at the age of one month was suggestive for an adrenal hemorrhage in involution. We therefore decided to keep monitoring the mass by ultrasound, avoiding any surgical approach. The sonographic complete disappearance of the disease was documented at the end of the fourth month.

[Chronic idiopathic gastric volvulus in children]. / Il volvolo gastrico cronico idiopatico in età pediatrica.

OBJECTIVE: We report clinical, radiograpic and instrumental data about 18 cases of gastric chronic idiopathic volvulus observed since 1997 to june 2000. METHOD: All cases diagnosed by barium meal, performed for vomiting or after a pHmetric esophageal monitoring, in cases of atipical symptoms of gastroesophageal reflux disease. RESULTS: All patients underwent a conservative treatment (antireflux position and procinectics): 5 recovered after 1 year of treatment and 13 were operated on because they did not improve during medical therapy or relapsed after the end of conservative treatment. CONCLUSION: Abnormal position of the stomach is not an absolute indication for surgical treatment. When medical treatment does not obtain improvement in symptoms, surgical option has to be discussed.