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PubMed2003

[Ventriculomegaly without dysraphic malformations--retrospective analysis fetuses diagnosed in the Department of Gynecology & Obstetrics of the Postgraduate Center of Medical Education in Warsaw, between 1997-2002].

OBJECTIVES: Our objective was to determine if ventriculomegaly not related to the open neural tube defects is associated with other anomalies and aneuploidy. What we should do after such diagnosis. We also analyzed gestational age at the first examination. DESIGN: We retrospectively studied ultrasound reports of the fetuses with this type of ventriculomegaly. MATERIALS AND METHODS: We analyzed 71 cases diagnosed between September 1997 and March 2002. Ventriculomegaly was a assumed to be present when atrial width was equal or greater than 10 mm. Antenatal karyotyping was performed in all fetuses. RESULTS: 21 cases were concomitant with other CNS malformations, in 50 cases ventriculomegaly was the only one brain anomaly. The incidence of chromosomal abnormalities was 8.5% and it was strongly related to the presence of multisystem malformations. The median gestational age at diagnosis was 27.6 weeks. CONCLUSIONS: Our results suggest that in fetuses with ventriculomegaly not related to the open neural tube defects a detailed ultrasonographic survey and karyotyping should be done. Furthermore, every pregnant woman should undergo ultrasound screening before the 24th gestational week.

Abnormalities, Multiple↗
PubMed1995

Treatment of neurogenic bladder dysfunction in infants and children with neurospinal dysraphism with clean intermittent (self)catheterisation and optimized intravesical oxybutynin hydrochloride therapy.

Clean intermittent (self)catheterisation (CIC) in combination with oral anticholinergic drugs (oxybutynin hydrochloride [OH] is the present standard therapy for neurogenic bladder dysfunction (NBD) with detrusor hyperactivity. However, complete suppression of detrusor contractions and complete urinary continence is not always obtained despite maximal dosage, and the high incidence of severe systemic anticholinergic side-effects often impairs therapeutic compliance, resulting in dose reduction or even discontinuation of therapy. The intravesical administration of OH has been shown recently to be an effective alternative for treating persistent detrusor hyperactivity, and occurrence and severity of systemic side-effects appeared to be significantly decreased. However, available data are limited from a paediatric view. Furthermore, it is our belief that the use of crushed OH tablets with consequent problems of impracticability accounts for the variable long-term patient compliance reported to be the only disadvantage to intravesical OH. Using an optimized drug preparation we demonstrate the superiority of intravesical OH for treatment of NBD in 15 children (range 0.6-13.75 years, mean 6.1) with incomplete detrusor activity suppression and/or intolerable systemic side-effects on oral OH therapy. Since the previous reported problems of impracticability and variable long-term patient compliance can be resolved by optimized drug preparation, we therefore conclude that the era of crushing OH tablets should be over in order to allow the intravesical OH therapy on a long-lasting and large-scale basis.

Administration, Intravesical↗
PubMed1997

Hand function in subjects with spina bifida.

The aim of our study was to assess the influence of the Arnold-Chiari malformation, cervical cord dysraphism and hydrocephalus on hand function in subjects with meningomyelocele, but without many of the other confounding factors commonly found in this group e.g low IQ and cerebral palsy, which in themselves cause poor hand function. Three groups of subjects who attended main stream schools underwent a basic neurological examination of the upper limbs and a battery of hand function tests. Group 1 consisted of subjects with meningomyelocele and shunted hydrocephalus (n = 21), Group 2 subjects had isolated shunted hydrocephalus (n = 13) and Group 3 were normal controls (n = 18). The results show that subjects with meningomyelocele had weaker power in the small muscles of the hand as compared to the controls and those with isolated hydrocephalus (p = 0.01) and poorer fine motor control and co-ordination. The meningomyelocele group scored significantly poorer in all of the tests as compared to the control group using the dominant hand (overall, p = 0.0003), and all of the tests but one using the non-dominant hand (overall, p = 0.0005). Our study has demonstrated that children with meningomyelocele have poorer hand function than healthy controls, which is due to causes other than hydrocephalus, poor IQ or cerebral hemisphere pathology. Instead, cerebellar and cervical cord abnormalities appear to be exerting a significant deleterious influence on the function of the upper limbs.

Adolescent↗
PubMed2004

Complications associated with clean intermittent catheterization in children with spina bifida.

PURPOSE: Clean intermittent catheterization (CIC) is commonly used in the management of voiding dysfunction associated with neurospinal dysraphism. We determined the incidence of genitourinary complications associated with this technique in a population of children with spina bifida. MATERIALS AND METHODS: The charts of all children younger than 13 years attending a multi-disciplinary spina bifida clinic between 1987 and 2002 were reviewed. Children in whom CIC had been performed for a minimum of 5 years were identified. Catheterization was performed with a polyvinyl chloride catheter and a water-based lubricant. All genitourinary complications that had occurred in this group were recorded. The caregiver of each patient was then contacted by telephone to confirm the accuracy of our data. RESULTS: There were 32 females and 27 males identified in whom CIC had been performed for a minimum of 5 years. Mean duration of CIC was 10.5 years (range 5 to 15). Of the patients 45 (76%) learned to perform CIC independently at a mean age of 8 years (range 4.7 to 15.3). Two complications were gross hematuria and a false passage in the bulbar urethra. Both complications occurred in males early in the course of CIC, and while being performed by a caregiver. Neither complication was associated with long-term sequelae. CONCLUSIONS: The incidence of genitourinary complications associated with CIC in children with spina bifida is low. We identified 2 complications during a period of 570 patient-years for an incidence of 3.5 complications/1000 patient-years (95% confidence interval -1.3, 8.3) of observation.

Adolescent↗
PubMed1976

[Diencephalic cyst--in relation to other midline dysraphism (author's transl)].

The abnormal midline cyst of diencephalic origin was termed "diencephalic cyst" by Brocklehurst in 1973. Since then, several case reports of so-called "diencephalic cyst" have appeared, but its clinical picture, especially its relation to other midline dysraphisms, does not seem to be well understood. Our experience with four cases of so-called "diencephalic cyst" were reported and their relation to holoprosencephaly as well as simple agenesis of corpus callosum was discussed. (1) In all of these cases, there was an abnormal space in the midline which freely communicated with the lateral ventricle. In the first two cases, their clinical pictures as well as the features of contrast studies were typical of alobar holoprosencephaly. In the third case, the absence of the olfactory tract, the incompletely separated cerebrum, and the monoventricle were confirmed at autopsy. In the last case, the ventricle was also incompletely separated. From these observations, such a malformation should better be considered as a variant of holoprosencephaly, and it would not be appropriate to classify this malformation, as Brocklehurst claimed, as "a maldevelopment lying between the group associated with anterior neuropore closure and prosencephalization (anterior encephaloceles and holoprosencephaly) and the group associated with maldevelopment of the hind brain (cerebellar and fourth ventricle encephaloceles and Dandy-Walker cyst of the rhombencephalon)." (2) This malformation is apparently formed before the 40 mm stage of the embryo, since by this time the diencephalic roof has already sunk to the level of the adult form. This maldevelopment should be clearly differenciated from simple agenesis of the corpus callosum which is formed after the 60 mm stage of the embryo. In simple agenesis of the corpus callosum, the deep venous system might take an abnormal course, but is well formed, whereas in holoprosencephaly or so-called "diencephalic cyst", the internal cerebral vein as well as the straight sinus and the inferior sagittal sinus is absent. (3) In all of our cases, there was an abnormal space posterior and inferior to the elevated lateral sinus. Autopsy done in two cases revealed that this abnormal space was a Dandy-Walker cyst in one case, the the dorsal sack of diencephalic origin in the other case. The latter case tells that in the cases of holoprosencephaly the lateral sinus does not necessarily mark the boundary between the cerebrum and the cerebellum. (4) "Diencephalic cyst" often accompanies severe hydrocephalus. However, even with successful surgical treatment of hydrocephalus, the outcome of intelligence in these cases would be grave.

Agenesis of Corpus Callosum↗
PubMed1975

Optic nerve anomalies in basal encephalocele.

Basal encephalocele should be suspected in child when hypertelorism, defect of the bridge of the nose, and other midfacial anomalies are present. Optic nerve anomalies may occur with basal encephalocele. Those previously described are pale discs, coloboma of the optic nerve head, and pit of the optic disc. Two additional disc anomalies that are associated with basal encephalocele are megalopapilla and optic nerve dysplasia. A mass in the nose or nasopharynx under these circumstances is most likely an encephalocele and biopsy is contraindicated.

Abnormalities, Multiple↗
PubMed2006

Inpatient health care use among adult survivors of chronic childhood illnesses in the United States.

OBJECTIVES: To describe the inpatient length of stay and related charges for adults in the United States with childhood-onset chronic disease and to examine patterns with respect to different hospital settings. DESIGN: We analyzed data from the 2002 Nationwide Inpatient Sample, a nationally representative data set of hospital discharges. We performed a case-mix-adjusted, sample-weighted regression analysis as well as descriptive statistics of hospital use among adults with childhood-onset chronic disease. SETTING: United States. PARTICIPANTS: We identified hospitalizations for persons aged 18 years or older with a diagnosis of complex congenital heart disease, cystic fibrosis, sickle cell disease, or spina bifida. Main Exposure Childhood chronic illness. MAIN OUTCOME MEASURES: Length of stay and total charges among pediatric, adult, and mixed hospitals. RESULTS: In multivariate adjusted analyses, patients with complex congenital heart disease and cystic fibrosis had a significantly longer length of stay in pediatric hospitals than in adult or mixed hospitals (P<.001); no similar difference was found for sickle cell disease and spina bifida. For all of the 4 conditions, hospital charges were significantly greater in pediatric hospitals than in adult or mixed hospitals (P<.001 for cystic fibrosis, complex congenital heart disease, and sickle cell disease, and P<.01 for spina bifida). CONCLUSIONS: The vast majority of persons who have survived to adulthood with complex congenital heart disease, cystic fibrosis, spina bifida, or sickle cell disease are hospitalized in hospitals that predominantly care for adults, where charges for care appear to be lower than in pediatric hospitals.

Adult↗