Craniopagus: a proposed nursing care protocol.

Craniopagus is a rare condition in which the skulls of conjoined twins, and the neurovascular structures in their skulls, are incompletely separated. Separation surgery is complex and requires a range of skills and expertise, including from all the nursing teams involved. A multidisciplinary group of professionals in a tertiary paediatric care setting in Italy developed a comprehensive protocol for the nursing care of craniopagus twins in hospital, informed by the findings of a literature review and the expertise of its members. The protocol was applied to the management of a pair of craniopagus twins who successfully underwent separation surgery at the authors' hospital. The protocol describes the crucial role of nurses in the care of craniopagus twins and their families before, during and after separation surgery.

Continence management in children with severe caudal regression syndrome: role of multidisciplinary team and long-term follow-up.

PURPOSE: Caudal regression syndrome (CRS) is a rare congenital abnormality including orthopedic deformities, urological, anorectal, and cardiac malformations. The clinical spectrum of CRS varies in severity, therefore multiple surgeries and complex medical care may be required with the efforts and support of a multidisciplinary team to ensure the most accurate treatment and the best outcome. The aim of our retrospective study was to evaluate the role of a multidisciplinary treatment and the long-term outcome in patients with severe CRS. METHODS: Clinical, surgical and psychological data were collected for all patients with diagnosis of CRS, treated at our Pediatric Hospital from January 1995 to December 2020. Patients with a severe form of CRS according to Pang's classification were included in the study. All patients have been followed by a multidisciplinary team composed by urologists, neurosurgeons, neonatal and digestive surgeons, orthopedic surgeon, physiatrists and psychologists. On admission, patients were screened for CRS with sacrum, spine and legs X-ray, spine MRI, renal ultrasound, voiding cystography and urodynamic, and renography. Clinical data about bowel function were evaluated. RESULTS: During the study period, 55 patients with CRS were treated at our Institution. Six out of 55 (10.9%), presented with severe form of CRS (5 pts with type 1; 1 pt, with type 2) and represent our study group. Diagnosis of severe CRS was made at birth because of the typical deformities of the pelvis (fusion of the iliac wings), and of the lower extremities (undeveloped legs with flexion of the knees, clubfoot). All patients presented with neurogenic bladder, 4/6 (66.6%) with vesicoureteral reflux (VUR) and 2/6 (33.3%) with renal agenesia and contralateral ectopic hypoplastic kidney. Bowel symptoms occurred in 5/6 pts (83.3%). All patients were started with an earlier clean intermittent catheterization (CIC) regimen. In 3 patients oxybutynin has been effective or well tolerated, while in other 3 onabotulinumtoxin A has been used. Vesicoureteral reimplantation has been performed in 1 patient, 2 required endoscopic treatment of VUR. On long-term, 2 patients required bladder augmentation with ileum and appendicostomy (Mitrofanoff). Low adherences in CIC have been observed in three patients, mainly at puberty. Two patients presented with chronic renal failure. One patient reported suicide proposal. Regarding social life, only one is regularly performing sport activity. CONCLUSION: CRS is characterized by maldevelopment of the caudal half of the body with variable involvement of the gastrointestinal, genitourinary, skeletal, and nervous systems. Management of CRS includes a wide variety of interventions to address the full spectrum of possible anatomical abnormalities. Hence, a multidisciplinary team is also mandatory for a correct bladder and bowel management, in order to maintain continence and preserve renal function, improve quality of life and increase self-esteem.

Anorectal malformation, urethral duplication, occult spinal dysraphism (ARM-UD-OSD): a challenging uncommon association.

PURPOSE: Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. AIM: To share our experience and to discuss the management of ARM-UD-OSD association. METHODS: We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation. RESULTS: All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). CONCLUSIONS: UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.

Validity and reliability of the Italian version of the cardiac quality of life questionnaire for pediatric patients with heart disease (PedsQLTM).

BACKGROUND: Congenital heart disease (CHD) accounts for nearly a third of all major congenital anomalies. Advances in pediatric cardiology shifted attention from mortality to morbidity and health-related quality of life (HRQOL) of patients with CHD and impact on their families. The purposes of this study were to assess the validity and reliability of the Italian version of the Pediatric Quality of Life (PedsQL) Cardiac Module and to create normative data for the Italian population. METHODS: This was an observational cross-sectional study of pediatric patients (aged 2-18 years) with congenital or acquired Heart Disease (HD) and their parents. Families were asked to complete the cardiac pediatric health-related quality of life questionnaire (the Italian PedsQL™ 3.0 Cardiac Module) and the generic pediatric health-related quality of life questionnaire (PedsQL™ 4.0 Generic Core Scales). The sequential validation procedure of the original United States version of the PedsQL™ 3.0 Cardiac Module was carried out under the instruction of the MAPI Research Institute. To assess construct validity, Pearson's correlation coefficients were assessed between scores on the Cardiac Module scales and scores on the scales of the General Module. To determine agreement between patient self-report and parent proxy-report, we used intraclass correlation coefficients (ICCs). To evaluate Internal consistency of items, we used Cronbach's alpha Coefficient. RESULTS: The study enrolled 400 patients. Construct validity is good between PedsQL Cardiac Module total scores and PedsQL total scores (p < 0.001). The recommended standard value of 0.7 was reached on the Cardiac and General Module core scales. Intercorrelations between PedsQL Cardiac module and PedsQL scores revealed medium to large correlations. In general, correlations between Patient self-reports are poorer than Parent-proxy ones. CONCLUSIONS: Cardiac PedsQL scores are valid and reliable for pediatric patients with congenital and acquired HD and may be useful for future research and clinical management.