1/6. The split notochord syndrome with dorsal enteric fistula, meningomyelocele and imperforate anus.A male infant was referred to our department because of lumbosacral meningomyelocele, dorsal enteric fistula and imperforate anus. The mother had received a parenteral drug containing estradiol benzoate and progesterone for inducing abortion in the first trimester. She also used an anal pomade containing triamcinolone and lidocaine-HCl during the pregnancy for hemorrhoids. Sigmoid end colostomy was performed after meningomyelocele repair. On abdominal exploration a wandering spleen was detected but no other anomalies. Two months later, an abdominoperineal pullthrough was performed, and the patient was discharged well after three weeks. Our case is the sixth that had split notochord syndrome associated with dorsal enteric fistula and imperforate anus. Additionally, penoscrotal transposition and wandering spleen were present in this case. To our knowledge, these associated anomalies have been extremely rare.- - - - - - - - - - ranking = 1keywords = meningomyelocele (Clic here for more details about this article) |
2/6. An unusual fetus with complete absence of thoracic, lumbar and sacral vertebrae, bilateral renal agenesis, VSD, meningomyelocele, imperforate anus, and teratoma.We present a 30 week old male fetus who had a very interesting malformation complex which can not be explained by teratogenic or hereditary diseases. The aim of this paper is to discuss this complicated entity and compare it with other reported cases.- - - - - - - - - - ranking = 0.66666666666667keywords = meningomyelocele (Clic here for more details about this article) |
3/6. Ankyloblepharon filiforme adnatum with hydrocephalus, meningomyelocele, and imperforate anus.A 3,780-g newborn girl had multiple, bilateral eyelid adhesions taht connected the upper and lower eyelids by bands of extensile tissue. Although the eyelid lesion was benign, the associated systemic abnormalities may have been serious. We doubt that a single intrauterine insult led to ankyloblepharon filiforme adnatum and the associated anomalies.- - - - - - - - - - ranking = 0.66666666666667keywords = meningomyelocele (Clic here for more details about this article) |
4/6. Sacral hemangiomas and multiple congenital abnormalities.Five infants with sacral hemangiomas and a particular constellation of congenital abnormalities are described. Three of the five infants had an imperforate anus associated with a fistula. Three of the five had renal anomalies; four had bony abnormalities of the sacrum, and three of these also had a lipomeningomyelocele. Four had skin tags, three of which were in the genital and sacral areas. Two of the five also had abnormalities of the external genitalia. This constellation of defects associated with a sacral hemangioma has not been, to our knowledge, elaborated previously.- - - - - - - - - - ranking = 0.16666666666667keywords = meningomyelocele (Clic here for more details about this article) |
5/6. Perinatal features of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS complex) associated with large meningomyeloceles and severe limb defects.Omphalocele-Exstrophy-Imperforate anus-Spinal defects (OEIS complex), a combination of omphalocele, exstrophy of the bladder, an imperforate anus and spinal defects, arises from a single localized defect in the early development of the mesoderm that will later contribute to infraumbilical mesenchyme, cloacal septum, and caudal vertebrae. In this report, we document the perinatal features of two cases of OEIS complex associated with meningomyeloceles and severe lower limb defects, and discuss the prenatal diagnosis, inheritance, and differential diagnosis of this association of malformations. Although long-term survival can be achieved by successful corrective surgery, the associated structural defects such as large meningomyelocele and severe limb aplasia or hypoplasia, as seen in our patient, can influence the patient's quality of life. We would like to emphasize that an accurate prenatal diagnosis of OEIS complex and associated malformations is important for the detailed counseling of the family as well as appropriate perinatal management by the obstetricians, pediatric surgeons, urologists, neurosurgeons, and neonatologists.- - - - - - - - - - ranking = 1keywords = meningomyelocele (Clic here for more details about this article) |
6/6. kidney transplantation in patients with neurovesical dysfunction.BACKGROUND: Five renal recipients with neurovesical dysfunction (NVD) were retrospectively reviewed focusing on anatomical and urodynamic abnormalities of the lower urinary tract and their management prior to kidney transplantation. methods: The underlying anomalies in these 5 patients were a posterior urethral valve (1 with an imperforate anus; n = 2), meningomyelocele (n = 2) and a congenital short urethra with an imperforate anus (n = 1). Their urinary tracts were evaluated prior to transplantation with voiding cystourethrography, urethrocystoscopy, cystometrography and electromyography of the external urethral sphincter to identify a possible focus of urinary tract infection, urine storage and voiding function. RESULTS: All 5 patients had NVD proven by urodynamic studies or by documentation of urinary retention in the absence of mechanical outlet obstruction. Bilateral high grade vesicoureteral reflux was noted in all patients, requiring ureteroneocystostomy. Clean intermittent catheterization (CIC) was ultimately employed for bladder emptying in all patients. Two patients with poor bladder compliance underwent augmentation cystoplasty before transplantation. The Mitrofanoff procedure was used in 2 patients with structural urethral abnormalities to access the bladder for catheterization. After eradication of possible sources of infection and establishment of a low-pressure urine storage system with bladder emptying by CIC, kidney transplantation was performed. Following kidney transplantation, all of the recipients were asymptomatic for urinary tract infections using CIC. Although 1 patient lost his graft due to chronic rejection, the other 4 other patients have good renal function. CONCLUSION: kidney transplantation in patients with NVD can be performed provided that their urinary tract problems are properly resolved.- - - - - - - - - - ranking = 0.16666666666667keywords = meningomyelocele (Clic here for more details about this article) |