Claudia Rendeli

Assessment of health status in children with spina bifida

Study design:Prospective multidimensional study by means of: (1) clinical assessment, (2) parental-administered questionnaire for general health (CHQ-PF50), and (3) standardised disability measurements.Objectives:To assess the health-related quality of life (QoL) and disability in children with spina bifida (SB) and to correlate them with the clinical picture and our previous study on adolescents with SB.Setting:SB Centre at a University Hospital in Italy.Methods:A total of 29 consecutive children with SB (mean age 11.4, range 4–14 years)were evaluated through Child Health Questionnaire Parental Form (CHQ-PF50), the FIM instrument, and the Barthel index.Results:Disability was inversely related only (r=0.49; P=0.007) to the physical aspect of the QoL of children. Similarly, the disability was inversely related (r=0.37; P=0.005) to the emotional aspect of QoL of patients parents. Unexpectedly, for the mental aspects of QoL of patients, major disability was not associated with higher psychological distress and severe role disability due to emotional problems. At clinical examination, findings especially for continence and number of catheterisations were usually related to deterioration of physical aspects of QoL (r=−2.28; P=0.02) in children.Conclusion:The multiperspective assessment showed that there is a linear inverse correlation between disability and QoL in children with SB only for physical aspects. Conversely, there is linear inverse correlation between disability and QoL in patients parents regarding only emotional aspects. Moreover, this study provided useful information for clinical practice underlining that continence problems are those that most affect QoL in children with SB and their parents.

Contrast-enhanced gray-scale and color Doppler voiding urosonography versus voiding cystourethrography in the diagnosis and grading of vesicoureteral reflux.

The purpose of this study was to compare contrast‐enhanced gray‐scale voiding urosonography (CE‐VUS) and contrast‐enhanced color Doppler voiding urosonography (CE‐CDVUS) with voiding cystourethrography (VCUG) to verify whether the use of color Doppler imaging improves the diagnosis and grading of vesicoureteral reflux (VUR).

'No allogeneic blood transfusion' protocol for the surgical correction of craniosynostoses. II. Clinical application

Abstract The authors describe the results obtained in 13 consecutive cases of craniosynostosis operated on according to a protocol devised at avoiding allogeneic blood transfusion. The protocol is based on pre- and postoperative treatment with erythropoietin, preoperative autologous blood donation, preoperative normovolemic hemodilution and intraoperative blood salvage. Nine subjects were affected by simple forms of craniosynostosis, whereas the remaining 4 presented with oxycephaly or craniofacial syndromes. Five of the 13 children were under 7 months and a further 3, under 10 months of age at the time of the surgical operation. Seven children weighed less than 10 kg. Allogeneic blood transfusion was avoided in 11 of the 13 children considered. Two failures – defined as the necessity to reinfuse the patient with an allogeneic blood transfusion – were recorded, 1 of them resulting from an unexpected hemorrhage during surgery. The results obtained indicate that this protocol designed to avoid allogeneic blood transfusion can be safely applied in the great majority of children with craniosynostosis, even when the surgical correction is carried out early in life.

Polyethylene glycol 4000 vs. lactulose for the treatment of neurogenic constipation in myelomeningocele children: a randomized‐controlled clinical trial

Aim  To compare the therapeutic effectiveness and tolerability of low daily doses of polyethylene glycol 4000 vs. lactulose in the treatment of neurogenic constipation in children with myelomeningocele.

Transanal irrigation in myelomeningocele children: an alternative, safe and valid approach for neurogenic constipation

Study design:A total of 60 children with myelomeningocele referred to Spina Bifida Center of Rome (31 boys and 29 girls; aged 8–17 years) were treated with transanal irrigation for three months.Objective:To investigate whether transanal irrigation is a valid and alternative approach for neurogenic constipation in children with myelomeningocele.Methods:A questionnaire on bowel disturbances, quality of life and side effects was completed before the beginning and at the termination of the study.Setting:ItalyResults:About 60% (36/60) of patients reported relief from constipation and 75% (12/16) for fecal incontinence. Wheelchair-bound and walking patients showed same high improvement of bowel habit. Mean (s.d.) scores before and after the study were: neurogenic bowel dysfunction total score: 17.5 (5.2) versus 8.5 (4.3) (P<0.001); digital stimulation of anorectum: 4.2 (2.8) versus 1.3 (2.5) (P<0.01); frequency of fecal incontinence: 5.5 (1.2) versus 1.3 (1.7) (P<0.01) and degree of general satisfaction: 3.0 (2.4) versus 7.7 (1.5) (P<0.001).We observed a reduction of urinary tract infections during the course of treatment: 14 total urinary tract infections (9 caused by Escherichia coli) before versus 6 (3) during treatment (P<0.01)Conclusion:Transanal irrigation in children with myelomeningocele is an alternative and relatively safe approach for managing neurogenic constipation; in fact, it improves bowel disturbances, quality of life and seems to reduce the risk of urinary tract infections.

Myelomeningocele: the management of the associated hydrocephalus

BackgroundThe pathogenesis of the hydrocephalus associated with myelomeningocele (MMC) has been the subject of an extensive number of studies. The contemporary reduction of the incidence of the Chiari II malformation and of the associated active hydrocephalus after closure of the spinal defect in utero is in line with previous studies suggesting a prominent role of the posterior cranial fossa abnormalities, where even the increased venous pressure might be at least mostly a consequence of the constriction of the posterior cranial fossa structures. Pure absorptive abnormalities however coexist, the main ones documented to be abnormal cisternal spaces and peculiar cerebrospinal fluid chemical features.Materials and methodsWe reviewed the pertinent literature concerning the pathogenesis and management of the hydrocephalus associated to MMC. We also reviewed our personal experience in managing the hydrocephalus in such patients through an endoscopic third ventriculostomy.Results and conclusionsThe literature review demonstrated an overall reduction in more recent series of children with MMC needing to be treated for the associated hydrocephalus postnatally, questioning the role of the prenatal care of the disease in this context. Less severe conditions and a more conservative neurosurgical attitude have certainly contributed to the reduction of the reported active postnatal hydrocephalus rate. Long-term cognitive evaluation of the children with MMC that we managed with an endoscopic third ventriculocisternostomy (ETV) as primary as well as secondary procedure did not demonstrate significant differences in the outcome compared with non-complicated extrathecally shunted children, favouring ETV as a valuable option in this subset of patients.

Sublingual desensitization in children with congenital malformations and latex allergy

The frequency of latex allergy in children requiring multiple surgery ranges from 16.7% to 65%. The aim of this study was to investigate the safety and efficacy of latex desensitization in a group of 10 patients with a history of multiple surgical procedures and clinically manifested allergy to latex. We selected 10 children (female–male ratio = 5:5), aged 4–16 yr (mean ± s.d.: 9 ± 4), with a history of multiple surgical procedures, adverse reactions to latex and positive skin test to latex and/or specific immunoglobulin E (IgE). Latex allergy diagnosis was confirmed by specific provocation tests (cutaneous, sublingual, mucous, conjunctival tests). Rush (4‐day) sublingual desensitization was performed with increasing doses of latex extract (ALK Abellò) under patients’ tongue until the highest dose of 500 μg of latex. A maintenance therapy (10 drops of undiluted solution three times a week) was recommended. During the 2‐yr follow‐up mean values of specific IgG4 and IgE, eosinophilic cationic protein and total IgE did not show significant variations. Patients did not manifest any adverse effect during the rush phase and only two patients manifested mild local symptoms during the maintenance therapy. All the challenges showed a reduction in terms of percentage of positivity and mean scores. All the patients showed a reduction of the mean individual score (p < 0.001). Furthermore patients who needed dental examination or surgery underwent such procedures without the occurrence of symptoms. Our preliminary results show sublingual desensitization to latex can be an important therapeutic tool in the management of young allergic patients requiring multiple operations.

Improvement of obstructive sleep apneas caused by hydrocephalus associated with Chiari malformation Type II following surgery.

Chiari malformation (CM) is the downward herniation of the caudal part of the cerebellum and/or medulla oblongata into the spinal canal. It can alter several neurological functions, including respiratory control and upper airway motility, and can be the cause of sleep-disordered breathing (SDB). The authors describe a 6-year-old boy affected by CM Type II associated with myelomeningocele who showed symptoms indicative of severe airway obstruction during sleep. Polysomnography revealed severe obstructive sleep apnea syndrome (OSAS). Magnetic resonance imaging demonstrated herniation of the cerebellar tonsils and diffuse ventricular dilation with a large pseudocystic formation in the third ventricle. Surgical marsupialization of the cystic wall was performed, associated with ventriculocystostomy and endoscopic replacement of the ventricular catheter. Polysomnography repeated 2 months after surgery revealed a striking improvement in the sleep-related respiratory pattern. The pathogenesis of OSAS was probably referable to a combination of CM and elevated intracranial pressure. However, the striking improvement of symptoms after ventriculoatrial shunt placement suggested that hydrocephalus plays a major role in this condition. Assessment and effective treatment of SDB is crucial in the care of patients with CM.

Peristeen(®) transanal irrigation in paediatric patients with anorectal malformations and spinal cord lesions: a multicentre Italian study.

In paediatric and adult patients with neurogenic bowel, transanal irrigation (TAI) of the colon has gained popularity due to the introduction of a specifically designed device. The aim of this pilot study was to present the results of TAI using the Peristeen® TAI system in a group of paediatric patients with anorectal malformation (ARM) and congenital or acquired spinal cord lesions (SCLs).

Serum interleukin-18 in children with Henoch-Schonlein purpura: a promising marker of disease activity?

Henoch-Schönlein purpura (HSp) is the most common systemic vasculitis of childhood with typical skin involvement and concurrent signs involving joints, gastrointestinal tract, and kidney. HSp pathogenesis is still far from being completely understood, though a knotty cytokine complex is believed to contribute to its intimate processes. The aim of our evaluation is to establish the relationship between serum levels of interleukin (IL)-18 and disease outcome and establish its feasibility to provide a marker of disease activity or even a prognostic tool in clinical practice. We examined clinical/laboratory variables and serum IL-18 in 17 children hospitalized during a year for HSp, diagnosed by EULAR/PRINTO/PRES criteria; the same patients were re-evaluated after 6 months. All results were compared with 25 age-matched healthy controls. IL-12 and IL-6 were also evaluated in a cohort of the same patients and compared with controls. General and clinical variables (sex, edema of the extremities, gastrointestinal or renal complications, relapses and renal involvement at 6 months) had no relationship with cytokine levels. Serum IL-18 and IL-6 levels were found significantly increased at diagnosis in HSp patients when compared with healthy controls. After 6 months, serum IL-18 and IL-12 levels were significantly decreased in patients, while IL-12 and IL-6 levels were significantly increased compared to healthy controls. Though preliminary and expecting further confirmation on a larger sample, our data support the conclusion that serum IL-18 levels reflect HSp activity.