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Anne Morice, Frédéric Kolb, Arnaud Picard, Natacha Kadlub and Stéphanie Puget

Reconstruction of complex skull defects requires collaboration between neurosurgeons and plastic surgeons to choose the most appropriate procedure, especially in growing children. The authors describe herein the reconstruction of an extensive traumatic bone and soft tissue defect of the cranial vault in an 11-year-old boy. The size of the defect, quality of the tissues, and patient's initial condition required a 2-stage approach. Ten months after an initial emergency procedure in which lacerated bone and soft tissue were excised, reconstruction was performed. The bone defect, situated on the left frontoparietal region, was 85 cm2 and was filled by a custom-made porous hydroxyapatite implant. The quality of the overlying soft tissue did not allow the use of classic local and locoregional coverage techniques. A free latissimus dorsi muscle flap branched on the contralateral superficial temporal pedicle was used and left for secondary healing to take advantage of scar retraction and to minimize alopecia. Stable well-vascularized implant coverage as well as an esthetically pleasing skull shape was achieved. Results in this case suggest that concomitant reconstruction of large calvarial defects by cranioplasty with a custom-made hydroxyapatite implant covered by a free latissimus dorsi muscle flap is a safe and efficient procedure in children, provided that there is no underlying infection of the operative site.

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Senta Kurschel, Stéphanie Puget, Marie Bourgeois, Michel Zerah, Petra Ofner and Dominique Renier

Object

In this study the authors' goal was to identify the complication rate of subduroperitoneal (SDP) shunts for the treatment of subdural hematomas (SDHs) in infants and to determine the influences on and predictive factors for these complications.

Methods

The authors present a case series spanning the years 1994 to 2003 and include a statistical analysis of 161 children 2 years of age or younger with SDH who were treated using a unilateral valveless SDP shunt. The patient history, characteristics, and treatment methods including prior therapies, neuroimaging findings, and clinical outcomes were measures of evaluation.

Thirty-six children (22.4%) suffered complications related to SDP shunts: obstruction in 27 (16.8%), infections in eight (5%), disconnection in four (2.5%), migration in three (1.9%), wound complications (leakage and skin ulceration) in two (1.2%), and symptomatic subdural rebleeding in one (0.6%) necessitating bur hole evacuation. Seventeen children (10.6%) underwent placement of a second SDP shunt because of ipsilateral or contralateral persistent fluid collections, or premature shunt removal. With the exception of 12 patients (7.4%), shunt removal was performed systematically and resulted in the following minor complications in 30 children (18.6%): an adherent proximal catheter in 16 (9.9%), transient symptoms of intracranial hypertension in six (3.7%), subcutaneous cerebrospinal fluid accumulation in four (2.5%), local infections in three (1.9%), and hydrocephalus requiring placement of a ventriculoperitoneal shunt in one (0.6%).

Status epilepticus at presentation and neuroimaging findings such as areas of hyperdensity on computed tomography (CT) scans representing fresh blood in the subdural fluid collections before shunt insertion and at follow up were predictors of shunt-related complications. Correlations were also discovered for the following CT findings: ischemic lesions before shunt treatment, cerebral atrophy and ventricular dilation during the last follow up, and residual medium to large collections before shunt removal. Children who attained a good outcome were less affected by shunt-related complications, unlike those who presented with focal deficits and/or visual impairment.

Conclusions

Subduroperitoneal shunt placement for the treatment of SDH in infants is—despite the complication rate—an effective and often inevitable treatment option, especially for most large and symptomatic SDHs; a certain number of complications could be reduced with careful and precise surgical techniques. Close observation for detection of risks is mandatory, and seizure control is essential to prevent further brain injury that may result in large subdural fluid collections that are difficult to treat.

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Jessica Ternier, Alison Wray, Stéphanie Puget, Nathalie Bodaert, Michel Zerah and Christian Sainte-Rose

Object

The authors characterized the clinical course of tectal plate lesions in a group of pediatric patients to identify the prognostic factors at presentation that predict progression, in an attempt to differentiate tectal hamartomas from tumors.

Methods

A retrospective review was conducted of the management of tectal plate lesions in children since the advent of magnetic resonance (MR) imaging at the authors’ hospital (1984–2003). The lesion volume seen on MR images, the clinical and radiological features at presentation, and the clinical course of the population were analyzed for correlations.

Forty children with tectal lesions presented in the typical delayed fashion (mean 8.5 months) with symptoms referable to hydrocephalus (93%). Fourteen children whose tumors demonstrated radiological progression (enlargement, contrast enhancement, or cystic change) were treated surgically. Histologically, 80% of the surgically treated lesions were low grade (with the other 20% consisting of one dysplasia, one high-grade tumor, and one unidentified tumor). Five patients required a second operation and one required a third. One patient died of a high-grade astrocytoma after undergoing surgery and radiotherapy; the other 39 patients remain clinically stable. The only factor predictive of tumor enlargement was lesion volume at presentation (p = 0.002). Distribution analysis revealed three subgroups based on lesion volume (< 4, 4–10, and > 10 cm3), which correlated with the clinical course of the disease.

Conclusions

Children with tectal lesions should undergo contrast-enhanced MR imaging and volume assessment at the time of presentation. After hydrocephalus has been managed with endoscopic third ventriculostomy, these children require prolonged, close clinical and radiological surveillance. Lesions with a volume less than 4 cm3 were likely to be hamartomas and followed a predominantly benign course, with few atypical cases progressing. All large lesions, defined as having a volume greater than 10 cm3 at presentation, eventually required treatment, and all were histologically determined to be tumors. An argument is made for earlier treatment of larger lesions with the aim of improving outcome.

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Anne Morice, Sylvie Fraitag, Catherine Miquel, Christian Sainte Rose and Stéphanie Puget

Juvenile xanthogranuloma (JXG) is a rare disease that belongs to the non-Langerhans cell histiocytoses. It presents a wide clinical spectrum, usually occurs before 5 years of age, and is commonly confined to the skin; however, it can affect multiple sites, including the nervous system, and can lead to severe disorders. Although JXG is a benign disease that usually regresses spontaneously, several curative treatments have been proposed in cases of organ involvement. Treatment options include corticosteroids, chemotherapy, and radiotherapy; however, these can have severe, long-term adverse effects in children.

The authors here describe the first case of spontaneous resolution of an intramedullary spinal cord lesion of JXG associated with cerebral and cutaneous lesions in a young boy with 9 years of follow-up. The initial neurological symptoms resolved without any surgical or medical treatment. This case shows that extracutaneous lesions of JXG, including those with intramedullary spinal cord involvement, can regress without curative treatment—like cutaneous lesions—although both multidisciplinary care and close follow-up should be implemented.

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Pierre-Aurelien Beuriat, Stephanie Puget, Giuseppe Cinalli, Thomas Blauwblomme, Kevin Beccaria, Michel Zerah and Christian Sainte-Rose

OBJECTIVE

Hydrocephalus remains one of the more common pathologies managed in pediatric neurosurgical units. Endoscopic third ventriculostomy (ETV) has an advantage over ventriculoperitoneal shunting as it enables patients to remain device free. Multiple shunt devices with various valve designs exist, with no one valve proven to be superior to another. The aim of this study was to describe the management of hydrocephalus and its long-term outcome.

METHODS

The authors retrospectively reviewed the medical records of all patients who had been treated for hydrocephalus at the Hôpital Necker-Enfants Malades in the period from 1985 to 1995.

RESULTS

Nine hundred seventy-five children had been treated for hydrocephalus. The mean follow-up was 11 ± 7.4 years (mean ± standard deviation). The most common cause of hydrocephalus was tumor related (32.3%), followed by malformative (24.5%) and inflammatory (20.9%) causes. Two hundred eighty patients underwent ETV as the first-line treatment. The procedure was effective in controlling hydrocephalus due to posterior fossa tumors and aqueductal stenosis. Six hundred ninety-five children had initial shunt insertion, with the majority receiving an Orbis-Sigma valve (OSV). The overall OSV shunt survival was 70% at 1 year, 58% at 10 years, and 49% at 20 years. The most common cause for mechanical shunt failure was obstruction (50.7%). Overall shunt survival was statistically different between the OSV and the differential-pressure valve (p = 0.009).

CONCLUSIONS

Endoscopic third ventriculostomy is effective in the management of childhood hydrocephalus. Its success is directly related to the underlying pathology. In the long term, the OSV has significantly higher event-free shunt survival than the classic differential-pressure valve systems

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Stéphanie Puget, Charles Kondageski, Alison Wray, Nathalie Boddaert, Thomas Roujeau, Federico Di Rocco, Michel Zerah and Christian Sainte-Rose

✓ The authors describe the case of a 12-year-old girl with Marfan syndrome, sacral dural ectasia, and tonsillar herniation, who presented with headache. Initially, it was hypothesized that the headaches were secondary to the tonsillar herniation, and the patient consequently underwent surgical decompression of the foramen magnum. Postoperatively, the patient's condition did not improve, and additional magnetic resonance (MR) imaging demonstrated evidence of a cerebrospinal fluid (CSF) leak at the level of the dural ectasia. It was surmised that the girl's symptoms were due to spontaneous intracranial hypotension (SIH) and that the tonsillar herniation was caused by the leakage. The patient responded well to application of a blood patch at the level of the demonstrated leak, and her headache resolved. This appears to be the first reported case of a patient with Marfan syndrome presenting with a symptomatic spontaneous CSF leak complicated by tonsillar herniation. In this rare association of SIH and connective tissue disorders, recognition of the clinical signs and typical MR imaging features of SIH may lead to more appropriate and less invasive treatment, potentially avoiding surgery.

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Christian Sainte-Rose, Ricardo Oliveira, Stéphanie Puget, Liana Beni-Adani, Nathalie Boddaert, John Thorne, Alison Wray, Michel Zerah and Marie Bourgeois

Object

The authors’ aim in this study was to review their experience in the use of indirect revascularization alone in a series of 14 children with moyamoya disease, in which numerous bur holes and arachnoid openings were made over each affected hemisphere.

Methods

Revascularization through multiple bur holes and arachnoid openings was performed in 14 children (mean age at diagnosis 6.5 years [range 3–15 years]) who suffered from progressive moyamoya disease. The authors performed surgery in a total of 24 hemispheres during 18 procedures. Ten children underwent bilateral multiple bur hole procedures, three underwent a unilateral procedure in the more severely affected hemisphere, and one child had previously undergone an encephaloduroarteriomyosynangiosis on the contralateral side. Ten to 24 bur holes were made in the frontotemporoparietooccipital area of each hemisphere, depending on the site and extent of the disease. Early postoperative perfusion magnetic resonance imaging studies, performed in the five most recent cases, showed restoration of cortical perfusion as early as 3 months, which was confirmed on subsequent angiography studies (performed between 8 and 12 months postoperatively) that showed excellent revascularization of the ischemic brain by external carotid artery collateral vessels. None of the children sustained further ischemic attacks postoperatively. Motor improvement was noted in those who had presented with paresis. A single seizure episode occurred in two patients at 2 weeks and 5 months after surgery; both children had presented with epilepsy. There were no postoperative deaths, and only one complication (an infected lumbar shunt in the patient who required cerebrospinal fluid [CSF] drainage). Five of the 18 procedures were complicated by subcutaneous CSF collections, which resolved with tapping and compressive head dressings; a transient lumbar drain was necessary in one case.

Conclusions

The results obtained in this series suggest that in children with moyamoya disease this simple technique is both effective and safe. Furthermore, it is effective as a sole treatment without supplementary revascularization procedures.

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Federico Di Rocco, Matthew R. Garnett, Stephanie Puget, Francisco Pueyerredon, Thomas Roujeau, Francis Jaubert and Christian Sainte-Rose

✓Rosai–Dorfman disease (RDD) is a rare idiopathic histiocytic disorder that only occasionally involves the central nervous system (CNS). Previous cases of RDD involving the CNS were generally seen in adults. Pediatric cases of RDD are rare, and the disease in these cases typically has an indolent clinical course. In this report, the authors describe a pediatric case of intracranial RDD with rapid clinical and radiological progression.

A previously healthy 13-year-old girl presented with a 15-day history of progressive left-sided headaches, vomiting, and fever. On examination she was pyrexial but otherwise normal. Neuroimaging results demonstrated an extraaxial left frontal lesion with peripheral enhancement. A bur hole was drilled over the lesion to obtain a tissue sample and de-bulk the lesion. The initial histological results showed a nonspecific inflammatory lesion. Postoperatively, the patient was asymptomatic, and neuroimaging results confirmed a significant reduction in the size of the lesion. Repeated neuroimaging 3 months later, however, revealed a large recurrence of the lesion, which was removed macroscopically by a craniotomy. Histological analysis of the tissue confirmed the RDD diagnosis. At the latest follow-up (12 months) the patient had remained asymptomatic with no evidence of recurrence on neuroimaging. This is the first reported case of intracranial RDD with an aggressive clinical course.

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Thomas Blauwblomme, Federico Di Rocco, Marie Bourgeois, Kevin Beccaria, Giovanna Paternoster, Juliette Verchere-Montmayeur, Christian Sainte-Rose, Michel Zerah and Stéphanie Puget

OBJECT

The ideal treatment for subdural hematomas (SDHs) in infants remains debated. The aim of this study was to analyze the safety and efficiency of subduro-subgaleal drainage in SDH.

METHODS

The authors conducted a single-center open-label study between August 2011 and May 2012. Data were prospectively collected in a database and retrospectively analyzed.

RESULTS

Eighteen patients (male/female ratio 1.25) with a median age of 5 months were surgically treated. All had preoperative symptoms of intracranial hypertension or seizures. The SDH was bilateral in 16 cases, with a median width of 12 mm. Success of the procedure was noted in 14 of the 18 patients. There was no intraoperative complication or postoperative infection. Drainage failure was attributable to suboptimal positioning of the subdural drain in 2 cases and to migration in 1 case.

CONCLUSIONS

Subduro-subgaleal drainage is an efficient treatment that could be proposed as an alternative to external subdural drainage or subduroperitoneal drainage.

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José Roberto Tude Melo, Federico Di Rocco, Marie Bourgeois, Stephanie Puget, Thomas Blauwblomme, Christian Sainte-Rose, Philippe G. Meyer and Michel Zerah

Object

Subdural hematoma (SDH) is the most common finding on cranial CT in pediatric victims of abusive head trauma (AHT). The hematomas are commonly bilateral and sometimes associated with interhemispheric hyperdensity and/or convexity hemorrhages. There is no consensus regarding the best surgical treatment in such cases nor are there standardized surgical protocols. The authors report their experience and discuss the routine surgical options in the management of traumatic SDH at a Level 1 Pediatric Trauma Center.

Methods

In this paper, the authors describe a cross-sectional study with consecutive revision of data described in the medical records of Hôpital Universitaire Necker–Enfants Malades between January 2008 and January 2013. During this period, all children younger than 2 years of age who were admitted with a traumatic SDH identified on CT scans were included in this study.

Results

One hundred eighty-four children who had SDH and were younger than 2 years of age were included. Their median age was 5.8 months (range 5 days–23 months), and 70% of the children were male. On admission CT scans, the SDH was bilateral in 52% of cases and homogeneously hypodense in 77%. Neurosurgical treatment was undertaken in 111 children (60%) with an admission Glasgow Coma Scale score of 12 or less, bulging fontanels, or other signs suggestive of intracranial hypertension. The first surgical option was craniotomy in 1.8% (2) of these 111 cases, decompressive craniectomy in 1.8% (2), transcutaneous subdural puncture in 15% (17), external subdural drainage in 16% (18), subdural-subgaleal shunt placement in 17% (19), and subdural-peritoneal shunt placement in 48% (53). In 82% of the children initially treated with transcutaneous subdural puncture and in 50% of those treated with external subdural drainage, increase or persistence of the SDH, CSF or skin infection, or shunt system malfunction was observed and further surgical intervention was required. There was a 26% rate of complications in patients initially treated with a subdural-peritoneal shunt. Although 52% of the patients had bilateral SDH, bilateral drainage was only required in 9.4%.

Conclusions

The choice of treatment should be determined by the clinical and radiological characteristics of the individual case. Although effective on an emergency basis, subdural puncture and external subdural drainage are frequently insufficient to obtain complete resolution of SDH, and temporary placement of a subdural-peritoneal shunt is needed in most cases.