A Multicentric European Clinical Study on Custom-Made Porous Hydroxyapatite Cranioplasty in a Pediatric Population.

Background: Cranioplasty (CP) is a surgical intervention aiming to re-establish the integrity of skull defects. Autologous bone and different heterologous materials are used for this purpose, with various reported related complications, especially in children.This study aims to evaluate the rate of complication in a multicentric cohort of pediatric patients treated by porous hydroxyapatite (PHA) CP implantation and to assess the reliability of post-marketing clinical data collected by a manufacturing company. Methods: The authors proactively collected clinical data from 20 institutions in different European countries for patients under the age of 16 treated with a PHA implant. The data were obtained by conducting an on-site interview with physicians in charge of the patients (Post-Marketing Surveillance, PMS group). The endpoints were the incidence of adverse events and related implant removal. The clinical data were compared to the company-based register including all patients under the age of 16 who received the same implant from January 1, 2004 to December 31, 2020, and the collecting complications voluntarily reported by surgeons (Database, DB group). Results: The two groups were similar in terms of demographic characteristics and rate of complications. In the PMS group, a total of 11 (16.9%) complications were reported in the group of 65 patients that were proactively collected. Both fractures and infections were the most common complications with 4 cases each (6.2%). In the case of both infections and fractures, revision surgery was required for only one patient (1.5%). Three (4.5%) cases of displacements were reported, and in one (1.5%) case, a surgical revision was required, for a total of 3 (4.5%) cases requiring surgical revision. The average follow-up was 26.7 months. Conclusions: Different from a previous study on adult age, pediatric neurosurgeons are more prone to report even to the manufacturing company complications related to skull reconstruction in children. Therefore, these data can be compared with those of other clinical studies. The PHA CP in this series of 65 patients presents a complication rate collected on-site that is similar to other heterologous materials.

Urinary Tract Anomalies in Patients With Anorectal Malformations: The Role of Screening and Clinical Follow-Up.

OBJECTIVE: To evaluate the efficacy of the screening protocol and the clinical follow-up to detect urological anomalies (UA) in patients with ARM (ARM-P) in our institution. Secondary aim was to define the prevalence of UA and their relationship with severity of ARM. METHODS: ARM-P were selected from a prospectively maintained database from 2000 to 2016. Exclusion criteria were: incomplete or with less than 3 years of follow-up and absence of surgical correction of the anorectal anomalies. Data from urological screening and follow-up were collected. Patients were divided into complex malformations (Group 1) and less complex malformations (Group 2). RESULTS: One hundred seventeen of 149 were included in the study period (62 group 1, 55 group 2). UA were detected in 36/117 at birth (30.7%) with a difference between groups (P = .0005). VUR was detected in 16 (6 with hydronephrosis, 10 with normal ultrasound at birth). A bladder ultrasound after potty training showed 18 lower urinary tract dysfunction (LUTD; 15 with UA detected at birth, 3 with normal ultrasound). 8 LUTD were found during clinical follow-up, confirmed by ultrasound. Spinal MRI detected spinal cord anomalies (SCA) in 52/117 (44.4%). Considering the subgroups with neurogenic bladder and SCA there was a difference between groups (13/33 vs 1/19). CONCLUSION: This study suggests that ARM-P are at increased risk of UA, most of which were detected on neonatal ultrasound. While screening protocol can show 88% of UA, follow-up can detect 12.9 % of total abnormalities without difference between groups. This data has to be considered when planning follow-up for these patients.

Rare association between spinal dural arteriovenous fistulas and dysraphisms: Report of two cases and review of the literature with a focus on pitfalls in diagnosis and treatment.

Spinal vascular malformations are uncommon yet important spinal pathologies commonly classified in congenital and acquired lesions. Spinal lipomas consist of three subtypes: intramedullary lipomas, lipomyelo(meningo)celes and lipomas of the filum. Although the association of spinal arteriovenous malformations (AVM) with other congenital anomalies is well known, the coexistence of dural arteriovenous fistulas (AVF) and tethered spinal cord is exceptionally rare and only eight cases have been reported. We present two cases from our institution and speculate on the possible origin of such a rare but insidious association. We review the current literature with a focus on possible pitfalls in diagnosis and treatment.

Intraparenchymal ventricular diverticula in chronic obstructive hydrocephalus: prevalence, imaging features and evolution.

BACKGROUND: Intraparenchymal cavities communicating with the ventricles may appear in patients with chronic obstructive hydrocephalus despite no identifiable surgerical, vascular or traumatic causes. The rate, features, pathogenesis, evolution and clinical impact of intraparenchymal diverticula have not been outlined, yet. METHODS: Brain MRIs of 130 patients (mean age: 11.3 years; age range: 0-67; 60 females) with chronic obstructive hydrocephalus were analyzed. The pathogenesis, neurosurgical treatment, ventricle size, signs of transependymal reabsorption and septum pellucidum integrity of the hydrocephalus were recorded. Subarachnoid outpouching of the ventricles, post-hemorrhagic parenchymal cavities, paths of ventricular shunting and cavities not communicating with the ventricles were excluded. Of patients with intraparenchymal diverticula, all previous available CT and MRI scans were evaluated. RESULTS: Eight patients (6.2 %, mean age: 18.7 years; age range: 2-42) harbored 11 intraparenchymal diverticula sprouting from the temporal (6), occipital (3) or frontal (2) horns of the lateral ventricles. Intraparenchymal diverticula were more frequent in males (p = 0.04) and older patients (18.7 ± 12.7 vs 11.3 ± 9.8 years, p = 0.04). Their presence or evolution (mean neuroradiological follow-up 3.6 years; range: 0-8) showed a trend of association with hydrocephalus severity (bifrontal index) and did not correlate with the surgical treatment. In three patients the diverticula progressed during follow-up. One patient presented with hemiparesis consistent with the intraparenchymal lesion and improved after ventricular shunting. A DTI study revealed that the cortico-spinal tract was partly included in the septum between the ventricle and the intraparenchymal diverticulum. CONCLUSIONS: Clinicians dealing with chronic severe obstructive hydrocephalus should be aware of ventricular intraparenchymal diverticulation. Studies aiming at clarifying their pathogenesis and proper management are warranted.

Chiari 1 malformation and holocord syringomyelia in hunter syndrome.

Compressive cervical myelopathy is a well-known life-threatening complication in mucopolysaccharidosis (MPS) patients. Glycosaminoglycan accumulation in the growing cartilage results in dens dysplasia, atlanto-axial instability, and subsequent periodontoid fibrocartilaginous tissue deposition with upper cervical stenosis.Chiari malformation type 1 (CM1) is a congenital downward cerebellar tonsil ectopia determined by clivus and posterior cranial fossa underdevelopment, possibly leading to progressive spinal cord cavitation (syringomyelia) and severe neurological impairment.We present a boy affected with Hunter syndrome (MPS II) and cerebellar tonsil ectopia who developed a holocord syringomyelia at the age of 6 years. The child underwent atlanto-occipital decompressive surgery with rapid clinical and neuroimaging improvement.Sharing a primary mesenchymal involvement of the cervical-occipital region, the coexistence of CM1 in MPS might be not unexpected and complicate further the disease course. In these patients, strict monitoring and prompt treatment might be of foremost importance for preventing major neurological complications.

Long-term operative failure of endoscopic third ventriculostomy in pediatric patients: the role of cine phase-contrast MR imaging.

OBJECT: Although a rarely reported occurrence, late failure of endoscopic third ventriculostomy (ETV) may occur in children as a result of a variety of factors. Delay in recognition of symptoms can lead to harmful deterioration in the patient's condition. The authors undertook this study to assess the capacity of cine phase-contrast MR imaging to identify late failure in asymptomatic pediatric patients treated with ETV for hydrocephalus. METHODS: This study was a retrospective evaluation of cases involving patients who underwent ETV between January 1, 1999, and December 31, 2008, at the pediatric neurological surgery service of the University of Padua. Before 2004, patients were routinely followed up with cine MR imaging at 3, 6, and 12 months after ETV. In 2004, a protocol of annual cine MR follow-up was instituted as a result of a case of fatal late failure. The authors evaluated all cases of late failure identified through cine MR imaging and performed a statistical analysis to investigate the relationship between ETV failure and several variables, including the cause of hydrocephalus for which ETV was originally indicated. RESULTS: In a series of 84 patients (age range 6 days-16 years), 17 patients had early ETV failure. Of the remaining 67 patients, 5 (7%) were found to have no CSF flow through the fenestration and recurrent ventriculomegaly when assessed with cine MR imaging at 1, 2, 3, 4, and 7 years after ETV. The patient in whom ETV failure was identified 1 year postoperatively had Dandy-Walker malformation. The patients in whom ETV failure was identified 2, 3, and 4 years postoperatively all had undergone ETV for treatment of postinfective hydrocephalus. The patient in whom ETV failure was identified 7 years postoperatively had a cystic arachnopathy in the fourth ventricle after cerebellar astrocytoma removal. CONCLUSIONS: Patients who undergo ETV for infective hydrocephalus and Dandy-Walker malformation should receive long-term follow-up, because late closure of the stoma may occur progressively and slowly. Intraoperative observation of thickened arachnoid membranes at the level of the interpeduncular cisterns at the first ETV and a progressive decreasing of CSF flow through the stoma on routine cine MR imaging should be considered unfavorable elements entailing a significant risk of deterioration.

Age-dependent presentation of tectal plate tumors: preliminary observations.

Tectal plate tumors are intrinsic midbrain tumors that behave more like hamartomas than neoplasms. Postulating that the presentation depends on the age of the affected patients, the authors reassessed the presentation of 10 consecutive patients. All patients presented with headache and papilledema. Four children younger than 10 years presented with a short symptom interval, vomiting, and Parinaud's syndrome. The remaining 6 children presented with a long symptom interval, gait abnormalities, cognitive impairment, vision impairment, tremor, macrocephaly, impaired visual acuity, and ataxia and sometimes with pyramidal signs as well. The difference between groups was statistically significant. Tumor size was similar in the groups. It is therefore concluded that the presentation of tectal plate tumors varies with age.

What's growing on? The growing teratoma syndrome.

BACKGROUND: The growing teratoma syndrome (GTS) consists of a mature teratoma paradoxically enlarging during or after chemotherapy for malignant nongerminomatous germ cell tumors. METHODS AND RESULTS: We report two cases of GTS occurring in association with NSGCT of the pineal gland. Although an unusual event, clinicians and radiologists should be aware of its natural history. CONCLUSIONS: When normalized tumor markers after chemotherapy are associated with imaging features of a growing mass, the hypothesis of GTS must be taken in consideration. When early diagnosed, GTS can be managed surgically with good results.

Hydromyelia associated with spinal lipoma of the conus: case report.

STUDY DESIGN: A case report and literature review of the treatment of "noncommunicating" syringomyelia. OBJECTIVE.: The aim of this report is to document the timing and the treatment of hydromyelia holocord after surgical treatment for both tethering and retethering of spinal lipoma. SUMMARY OF BACKGROUND DATA: Syringomyelia associated with spinal lipoma presents a different pathogenesis and treatment in comparison to the "communicating" hydromyelia in the myelomeningocele. After the primary retethering operation performed in symptomatic patients, recurrent retethering can occur with an increase of the syringomyelia signs and symptoms. METHODS: Syringomyelia treated with a thin silastic tube passed from the syrinx to the subarachnoidal space for drainage and decompression. Prior operations were: (1) initial untethering at birth, (2) second untethering at 5 years of age, (3) posterior fossa and cervical decompression. RESULTS: Magnetic resonance imaging 6 months post shunt operation demonstrated decompression of the hydromyelia holocord and syringobulbia with improvement of motor function of the legs and improvement in sensory symptoms. CONCLUSION: Usefulness of syrinx-subarachnoidal shunt is demonstrated in this case report after unsuccessful decompression and detethering. When the enlargement of the ependymal channel is greater than 50% of the spinal cord's diameter, neurologic, and urological symptoms are evident and the patient benefitted from cord untethering and syrinx drainage. (1) The terminal "noncommunicating" syringomyelia in lumbar sacral lipoma has been reported to be associated with retethering in spinal lipoma in the 25% of the cases. (2) The rise of distal syringomyelia isn't only linked to the kind of the spinal lipoma, but also to the difficulty to obtain the untethering and a smooth cerebrospinal fluid flow between the subarachnoidal space and the ependymal canal. (3) In patients with hydromyelia holocord greater than the 50% of the spinal cord's diameter a myelotomy and insert an ependymal channel/syrinx to the subarachnoidal space shunt can resolve of the syrinx. In this case, the enlargement of the ependymal channel in "noncommunicating" syringomyelia associated with lumbosacral lipoma is greater than 50% of the spinal cord's diameter; neurologic and urological symptoms occurred and the patient benefited from cord untethering and concurrent syrinx drainage.

Combined endoscopic-microsurgical approach for transsphenoidal (sphenopalatine) encephalocele with an intralesional pituitary gland. Case report.

The treatment of the transsphenoidal sphenopalatine encephalocele in infants has not been thoroughly described in the literature. Pterional and subfrontal transbasal approaches have been reported as the advised treatment for transethmoidal encephalocele, but their feasibility for transsphenoidal encephalocele remains controversial, particularly in neonates. The potential harm to vital structures within the herniated tissue and intraoperative bleeding have been considered the major cause of poor postoperative results. The authors present the case of a full-term newborn with a cleft palate and a large transsphenoidal sphenopalatine encephalocele, which filled the nasopharyngeal space and provided MR imaging evidence of an intralesional pituitary gland. The lesion is unusual, and the extracranial intraoral endoscopic approach with an optimal outcome and sparing of the pituitary tissue has never been described in detail in an infant of this age.

Anorectal malformation and spinal dysraphism: the value of diffusion-weighted imaging in detecting associated intradural (epi)dermoid cyst.

Anorectal malformations (ARMs) are associated with a variety of spinal dysraphisms, of which clinical impact is often underestimated. A 6-year-old girl, with a history of rectovaginal fistula, presented with gait disturbance, asthenia, and worsening of fecal incontinence. The spinal magnetic resonance imaging (MRI) at 12 months of age had revealed a tethered cord and a little intradural lipoma. Within the lipoma, a small cystic lesion, interpreted as a cerebrospinal fluid (CSF)-filled loculation, was also described. A consecutive MRI showed a marked increase in size of the CSF-like cyst that was clearly hyperintense on diffusion-weighted imaging (DWI) and presented reduced apparent diffusion coefficient values (855 +/- 109 s/mm(2)), not compatible with CSF values. This lesion, interpreted as an (epi)dermoid cyst, was removed and histologically confirmed; the spinal cord was untethered. The child's lower limb motor deficit resolved rapidly after surgery, and the fecal incontinence slowly returned to the previous bowel habit. There is a growing interest in recognizing and defining spinal dysraphism in ARM patients because some abnormalities may carry severe clinical consequences. For this purpose, a standardized MR protocol is required, in which DWI plays a pivotal role to disclose associated dysembriogenetic lesions, in particular when a CSF loculation is detected.

Evaluation of health status and health-related quality of life in a cohort of Italian children following treatment for a primary brain tumor.

BACKGROUND: This study is a pilot experience aiming to investigate the compliance of an institutional cohort of Italian children treated for a malignant disease and their families in completing the health utilities index2, (HUI2) and the effectiveness of this measured in terms of their health status (HS) and health-related quality of life (HRQL). It specifically, it aimed to compare the HS and the HRQL, as expressed by the HUI2 global utility score, in cohorts of patients who had brain tumors, extra-cerebral solid tumors, or leukemia/lymphoma. PROCEDURE: Fifty survivors of brain tumors, between 8 and 30 years at the time of the assessment ("self") and/or their parents ("proxy"), attending the Pediatric Oncology Out Patient clinic of Padua, Italy, completed the HUI2 questionnaire. Eighty-nine children with acute leukemia/lymphoma and 74 with extra-cerebral solid tumors and/or their parents were also assessed. RESULTS: The mean "self" and "proxy" HUI2 global utility scores in the brain tumor patients were 0.87 and 0.84, respectively, while in the cohorts of children with other solid tumors and leukemia/lymphoma, there were 0.94, 0.91, 0.96, and 0.92, respectively. The differences between the HUI2 global utility scores in the "self" and "proxy" assessment within each cohort of children were not statistically significant. In decreasing order of frequency, the attributes affected most commonly were: "emotion," "pain," "sensation," and "cognition" both by "self" and "proxy" assessment. CONCLUSIONS: In this Italian population of childhood cancer survivors the HUI2 questionnaire proved to be a user-friendly tool, which provided information regarding HS and HRQL. A larger cohort of cancer children is needed to confirm the efficacy of the HUI2 questionnaire in distinguishing groups of children on this basis by disease category.

Spinal cord injury without radiographic abnormalities.

Spinal cord injury without radiographic abnormality (SCIWORA) has a reported rate of incidence varying from 19% to 34% of all spinal cord injuries in children. This acronym refers to the presence of neurological lesion, despite normal plain radiographs, but where magnetic resonance imaging (MRI) shows significant pathology. The clinical evidence of the damage could be delayed in 6-54% of cases, usually within 48 h after the trauma. We report two patients affected by SCIWORA in the pediatric population. The first patient was a child of 22 months who had fallen when attempting to get out of bed. The second patient was an 11-month-old child admitted to the Emergency Service department after a car accident. Spinal cord injury has to be suspected in the presence of neurological signs, despite normal plain radiography. MRI is the appropriate diagnostic examination to identify the presence of SCIWORA.

Erythropoietin therapy and acute preoperative normovolaemic haemodilution in infants undergoing craniosynostosis surgery.

BACKGROUND: A retrospective study was performed to evaluate whether pretreatment with erythropoietin and iron combined with acute preoperative normovolaemic haemodilution (APNH) could decrease homologous blood transfusion in craniosynostosis (CS) surgery. A treated group was compared with a historical group of infants who underwent surgery with no pretreatment. METHODS: The charts of 25 healthy infants who underwent CS surgery were reviewed. Nine of them underwent surgery with no treatment beforehand. Sixteen infants were given erythropoietin at a dosage of 300 U.kg -1 two times per week and iron (elemental iron 10 mg.kg-1.day-1) for 3 weeks before surgery. On the day of surgery APNH was performed after induction of general anaesthesia; a precalculated amount of autologous blood was withdrawn and replaced by hydroxyethyl starch 6%. RESULTS: Eleven of the 16 infants of the study group received only autologous blood. Five of 16 received homologous blood transfusion vs seven of nine infants in the control group. CONCLUSIONS: APNH combined with erythropoietin was effective in reducing homologous blood requirements during CS surgery. Further studies are necessary on a larger scale to assess the role of this technique in avoiding homologous blood transfusion and to evaluate how infants can benefit from this combined approach.

Primary intracranial fibrosarcoma.

INTRODUCTION: Primary fibrosarcomas of the brain are very rare tumors, so that information regarding the treatment is scarce. We report the contributions that different therapeutic options made to the treatment of a child with one of these aggressive tumors. CASE REPORT: A 13-year-old boy underwent a complete resection of a left temporo-parietal mass that had been diagnosed as a fibrosarcoma by two independent pathologists. Adjuvant chemotherapy with vincristine, actinomycin-D, ifosfamide and Adriamycin was started, but after 3 months tumor relapse was evident. The boy subsequently received radiation therapy during which there was evidence of progressive tumor shrinkage. A second surgery was performed 6 months after radiotherapy and a small enhancing lesion, revealed to be gliosis, was resected. The child remains alive and well 44 months after diagnosis. CONCLUSION: Our experience supports the importance of total resection followed by radiation therapy, and radiotherapy should be started as soon as possible after surgical resection, rather than administering chemotherapy first.