PubMed · 4579333
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W H Hendren. 1973-09-06. Pediatric surgery. 2.. https://doi.org/10.1056/nejm197309062891005
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PURPOSE: To delineate the clinical and MR findings in children with an unusual type of spinal dysraphism, the terminal myelocystocele. Infants with a terminal myelocystocele carry a favorable neurologic prognosis if the entity is diagnosed early. Understanding the MR characteristics of this entity will allow for earlier and more accurate diagnosis. METHOD: Analysis of the medical charts and MR studies in 15 children with surgically and histologically proven myelocystocele. RESULTS: In all 15 children, MR demonstrated the primary findings of a terminal cyst of the central canal of the spinal cord which is tethered and herniated with arachnoid and cerebrospinal fluid through an area of spinal dysraphia onto the back as a mass. Of these children, 10 had additional findings (one or more) on MR of Chiari I (five cases), Chiari II (one case), cervicothoracic hydromyelia (two cases), lumbar hydromyelia (two cases), hydrocephalus (2 cases) segmentation anomalies of vertebrae (3 cases) and partial agenesis of sacrum (six cases). Of the clinical findings, all 15 children had a back mass, 10 also had cloacal exstrophy. One had imperforate anus, 10 were girls, five had ambiguous genitalia and all were neurologically intact. CONCLUSION: Children with a terminal myelocystocele present with a back mass and there is a high association with cloacal exstrophy. MR is the best noninvasive modality to diagnose all of the components of a terminal myelocystocele and the associated central nervous system findings.
In this report we document the concurrence of a large omphalocoele and anal atresia in a female newborn with EEC syndrome (Ectodermal dysplasia-Ectrodactyly-Cleft palate). Up to now, the association anal atresia-EEC syndrome has been noted in 4 other unpublished patients indicating that anal atresia may constitute another true manifestation of this autosomal dominant syndrome with variable expression.
The posterior sagittal approach was used to treat 792 patients with anorectal malformations. From these, 387 cases were evaluated 6 months to 13 years later. Voluntary bowel movements were present in 74.3% of the entire series. When distributed by diagnosis, the percentages varied: 100% in patients with rectal atresia and perineal fistula; 93.2% in those with vestibular fistula; 80.9% in those with bulbar fistula; 71.1% in those with cloacas; 66.7% in those with prostatic fistula, and 15.8% in those with bladder-neck fistula. Soiling was present in 57% of all cases. Patients with voluntary bowel movements and no soiling were classified as totally continent; 40.8% of the series belong to this group. Distributed by diagnosis, it varied from 100% in cases with rectal atresia or perineal fistula, 65.9% in those with vestibular fistula, 34% in those with bulbar fistula, 31.6% in those with cloacas, 26.3% in those with prostatic fistula; none of the patients with vaginal fistula or bladder-neck fistula was totally continent. Constipation was detected in 43.1% of all patients, and was more frequent in those with simple defects. Urinary incontinence was found in 19% of patients with cloacas who had a common channel shorter than 3 cm, and in 68.8% of the patients who had longer common channels. Other patients suffered from urinary incontinence only when they had an absent sacrum or other severe bladder or urethral congenital defects. An accurate diagnosis and evaluation of the sacrum allows us to establish, with reasonable accuracy, functional prognosis in most children. Those with functional disorders must be treated properly medically, to improve their quality of life.