Crouzon syndrome: posterior fossa volume studies in vestibular orientation.

INTRODUCTION: With the increasing possibilities of surgical treatment addressing the complex spectrum of defects in Crouzon syndrome (CS), it is of utmost importance to obtain accurate assessment of the malformation. Evaluating the volume of the posterior fossa is of great importance since many authors, considering that its volume is always decreased, favor posterior fossa enlargement as a first step in the treatment of CS. MATERIAL AND METHODS: We studied CT scans of children with CS, which were vestibular-oriented (VO) in order to conform with physiological landmarks, and we measured intracranial volume by manual segmentation; these abnormal CT scans were superposed with VO 3D-CT scanners of age-matched controls. We studied the volume index of the posterior fossa for each CS patient defined as the ratio of its volume in CS patients with the normal for age calculated from normal controls. RESULTS: We studied the imaging of 41 children with CS and 70 control. Among CS patients, the volume of the posterior fossa was increased in 10, compared with control. We found closure of the sphenoidal synchondrosis was correlated with age and with the width of the posterior fossa, but not with its length nor with the posterior fossa volume index. CONCLUSION: Segmentation on VO-CT scanner and superposition with CT scanners of normal controls is a powerful tool for the study of the impact of CS or other synostoses on volume and shape. We found that CS is more heterogeneous than previously thought, and surgical strategies should be adapted accordingly.

Three-dimensional study of 31 cases of synostotic anterior plagiocephaly before and after surgical management the Lille protocol.

Synostotic anterior plagiocephaly is a rare pathological cranial malformation. Therapeutic options are rarely studied due to the rarity of the malformation and difficulties in diagnosis and care management. The objective of this study was to analyze the results obtained with the Lille protocol based on 62 CT-scans done before and after surgery in 31 patients. A specific analysis was designed for this work. Nine cephalometric measures enabled to evidence on each CT-Scan the corrections made on the fronto-orbital bandeau and the potential impact of surgery on the craniofacial structures. Results show that surgical symmetry of the fronto-orbital bandeau in the transversal plane, according to the symmetrical axis of the semicircular canals, allows a normalization of the skull's growth and morphogenesis for the surgically affected structures but also adjacent ones.

Brachmann-Cornelia de Lange syndrome with a papilloma of the choroid plexus: analyses of molecular genetic characteristics of the patient and the tumor. A single-case study.

PURPOSE: A 10-month-old girl with a Brachmann-Cornelia de Lange syndrome and a choroid plexus papilloma of the brain was studied at the Hospital Infantil de México Federico Gómez (HIMFG) in Mexico City. METHODS AND RESULTS: Presumptive papilloma of the third ventricle was evidenced on CT and MR images and removed. Pathological analysis confirmed its origin. A posterior radiosurgery was required due to a tumor relapse. Karyotypes (GTG bands) of the patient and her parents undertaken at HIMFG were normal. Array comparative genomic hybridization (array CGH) analyses of blood DNA of the patient and her parents carried out at BlueGnome's Laboratory in Cambridge, UK, set in evidence amplification of genes SPNS2, GGT6, SMTNL2, PELP1, MYBBP1A, and ALOX15 in chromosome 17p of the patient. Since MYBBP1A is a proto-oncogene and ALOX15 participates in the development of cancer and metastases of tumors, further fluorescent in situ hybridization (FISH) analyses of these two genes were implemented at HIMFG. Amplification of the two genes was found in the tumor of the case under study but not in an unrelated papilloma of the choroid plexus. DISCUSSION: Further analyses of the association of choroid plexus papillomas with disorders of psycho-neural development and its relationship to molecular genetic modifications at chromosome 17p are now under way at HIMFG.

Fifteen years of experience with the midfacial distraction without maxillary osteotomy protocol.

PURPOSE: Midfacial distraction for facial stenosis is minimizing the communication between cranial fossa and nasal fossa caused by the Le Fort III osteotomy during frontofacial advancement procedures. There are different types of distractors, such as internal and external devices. The aim of our study is to present a series of 22 consecutive distraction cases operated without any Le Fort osteotomy with external distraction frames. We completely avoid the gap between the skull and nose, thus avoiding related complications. PATIENTS AND METHODS: Between 1997 and 2012, we operated on 22 patients presenting syndromes associating midfacial retrusion, maxillomandibular class III malocclusion and upper airway obstruction. METHODS: We perform a fronto-orbital advancement. We do not perform any maxillary osteotomy. A vertical cut in the lateral orbital wall is done towards the inferior orbital fissure and another cut on the zygomatic arch. We realise the fixation of the frame posteriorly with a folded K-wire and anteriorly with a transmaxillary pin. Aiming overcorrection, we distract on average 1 mm a day for a mean period of 26 days and with a horizontal distraction vector. RESULTS: No deaths or life-threatening complications were reported. All midfacial retrusions were corrected without relapse. The advancement ranged between 6 and 20 mm. Several complications were notified: one sphenopetrous dislocation, one ethmoidonasal dislocation, two device disassemblages and two cases of maxillary sinusitis. Some of these complications caused an incomplete distraction result. CONCLUSIONS: Compared to other techniques, this method is safe, simple and efficient. By sparing major osteotomies, it avoids severe complications.

Trigonocephaly: Lille's surgical technique.

From the early beginning, in 1977, of our experience in the treatment of trigonocephaly and with the follow-up of 263 patients, we have been convinced that it was a condition related to the skull base. It is now proved thank to the comparison between patient's CT scan and normal template (normal skull of the same age) in vestibular orientation. We used to correct this condition by a frontal reshaping and a rotation of the superior and lateral orbital rim along an oblique axis. We call that movement: "valgisation."

Aplasia cutis congenita: review of 29 cases and proposal of a therapeutic strategy.

INTRODUCTION: Aplasia cutis congenita (ACC) is a rare congenital disorder, which most commonly involves the scalp, and can affect the galea, the pericranium, the bone, and the dura mater. ACC thus is at risk of infection and hemorrhage. There is no consensus over the ideal management and the role for plastic surgery. MATERIALS AND METHODS: We reviewed retrospectively our experience with 29 patients treated between 1976 and 2011. RESULTS: The patients were 17 male and 12 female, 25 being referred immediately at birth. The size of the defect ranged from 1 to 192 cm2. Thirteen patients had bone aplasia. Initial conservative treatment was decided in five cases; 15 patients underwent excision-sutures with or without local plasty, 8 underwent pedicled scalp flap, and 1 had skin graft followed by further reconstruction by a free flap. Four patients died in neonatal period because of infection or associated ailments. All others patients achieved complete healing. DISCUSSION: The mortality rate of ACC remains high and increases with the size of bone defect. We propose a therapeutic strategy based on the size of the skin defect and the nature of underlying exposed structures. Cranioplasty is exceptionally necessary because of good spontaneous bone regeneration within few months or years. Cosmetic appearance can be improved later by skin expansion. CONCLUSION: Aplasia cutis congenita is a rare malformation with sometimes a rapid fatal issue. A precise evaluation of surface and depth of the lesion is essential to decide if and how to operate, in order to provide rapid and efficient coverage.

Atypical scaphocephaly: a review.

BACKGROUND AND PURPOSE: Sagittal craniosynostosis (SCS) is common and easily recognized and corrected surgically. However, rare cases of SCS are more complex: these associate closure of the metopic or delayed closure of the coronal suture, uni- or bilaterally. MATERIAL AND METHODS: We reviewed the available literature on atypical sagittal craniosynostosis (ASCS). We also reviewed retrospectively our series of SCS treated since 1980 and selected cases with simultaneous closure of the metopic (leptocephaly) or delayed closure of other sutures (plagiocephaly, oxycephaly, or Crouzon syndrome). RESULTS: ASCS is rare, representing <10 % of SCS. In our series, among 447 cases of SCS followed for a mean duration of 63.7 months, we identified 22 cases of ASCS: 6 with leptocephaly, 9 with non-syndromic oxycephaly, 4 with Crouzon syndrome, and 3 with plagiocephaly. Fourteen patients required a second operation, either planned initially (severe leptocephaly) or because of brain compression. The actuarial incidence of ASCS requiring reoperation was 5.3 % of SCS at 10 years. After a mean follow-up of 113 months, morphological results in ASCS were grade 1 (no defect) in 5, grade 2 (mild defect) in 2, grade 3 (minor reoperation) in 3, and grade 4 (major reoperation) in 12; one patient had visual impairment, and two had learning difficulties. CONCLUSIONS: ACSC can be detected initially or occur with a delay in apparently standard SCS. Leptocephaly is a specific entity. Because of the implications on the management and risk for the patient, preoperative evaluation of patients with SCS with CT scanner and prolonged follow-up are necessary.

Unicoronal synostotic plagiocephaly: surgical correction: Lille's technique.

INTRODUCTION: For 35 years, we have a tight neuro-plastic surgical cooperation for the surgical correction and long-term systematic follow-up of 125 cases of unicoronal synostotic plagiocephaly. METHODS: We have tried to understand why some patients had kept an asymmetrical facial growth pattern in spite of a good fronto orbital correction. Analysis in vestibular orientation which was available from 1993 has demonstrated a discrepancy between the ocular and the vestibular verticality referential system. So we have designed a surgical procedure to try to fix that problem. RESULTS AND CONCLUSION: Preliminary results in 27 cases operated according to this procedure, for which we have pre- and post-CT scan demonstrate significative improvements of our results.

Non-syndromic oxycephaly and brachycephaly: a review.

BACKGROUND: Non-syndromic coronal synostoses oxycephaly and brachycephaly (NSCSOB) are rare. Their natural history, surgical management, and outcome are debated. MATERIAL AND METHODS: We reviewed the available literature on NSCSOB. In addition, we reviewed retrospectively our experience with cases of NSCSOB, managed and operated by our joint craniofacial team since 1984. Newborns underwent perifrontal craniectomy, while infants and older children underwent fronto-orbital advancement with frontoparietal remodeling. Cases with delayed onset of faciosynostosis were excluded. RESULTS: Some cases of NSCSOB present at birth or even prenatally with gross dysmorphism and severe brachycephaly; others present later in life with harmonious restriction of intracranial volume and are at risk for development and vision. Some NSCSOB evolve from initially unisutural synostoses. We treated 61 cases, operated aged 1.4 to 106.7 months, 30 with brachycephaly and 31 with oxycephaly. Twelve of these (19.7 %), had been treated initially for sagittal or unicoronal synostosis, and evolved into NSCSOB. Intracranial hypertension was present initially in 28 (45.9 %), with ophthalmological consequences in 13 (21.3 %) and mental retardation in 6 (9.6 %). No patient had hydrocephalus; only two had asymptomatic Chiari malformation. The mean postoperative follow-up was 113.6 months. At last control, 21 patients had developmental delay and 5 had visual impairment. CONCLUSIONS: NSCSOB are a complex entity. They have in common a high risk of severe intracranial hypertension and rare hydrodynamic complications. Genetic screening is necessary to assert their non-syndromic nature. Prolonged follow-up of all craniosynostoses is necessary because unisutural synostoses can evolve into NSCSOB, and NSCSOB can evolve into craniofaciosynostosis.

Midfacial distraction without osteotomy using a transfacial pin and external devices.

INTRODUCTION: During the 1970s, frontofacial advancement revolutionized the treatment of severe facial stenosis. Unfortunately, this method was associated with significant morbidity due to the Le Fort III osteotomy, which creates a major communication between the frontocranial dead space and the nasal fossae. Midfacial distraction improves the complication rate by diminishing the size of this gap. The aim of our study was to present an original technique that uses external distraction frames and eliminates the need for Le Fort osteotomies. This innovative technique eliminates the gap between the skull and nose, thus avoiding related complications. PATIENTS AND METHODS: Between 1997 and 2008, we operated on 17 patients presenting midfacial retrusion and maxillomandibular class III malocclusion. We performed classic fronto-orbital advancement. The only facial osteotomies are vertical cuts of both the lateral orbital wall and the zygomatic arch. The distraction device is then anchored posteriorly with a K-wire and anteriorly with a transfacial pin through the maxilla. Finally, the distraction is performed horizontally until a class II overcorrection is obtained. RESULTS: No life-threatening complications or mortalities occurred. In all cases, the midfacial retrusion was corrected without relapse. All patients with complications fully recovered. It was observed that most complications were a result of either an overly rapid activation (>1 mm/d). DISCUSSION: Midface distraction using the external transfacial pin is a simple and safe procedure that allows an efficient correction of major facial retrusion. The external transfacial pin acts directly onto the maxilla and allows distraction without Le Fort osteotomy. By eliminating major osteotomies, it reduces the number of severe complications encountered in craniofacial surgery.

Neurocutaneous melanosis and the Dandy-Walker complex: an uncommon but not so insignificant association.

BACKGROUND: Neurocutaneous melanosis represents a rare congenital but nonheritable phakomatosis defined as the association of giant or multiple congenital nonmalignant melanocytic nevi with leptomeningeal melanosis or melanoma of the central nervous system. METHODS: We describe the case of an adolescent with a giant congenital bathing trunk melanocytic nevus who developed progressive intracranial hypertension due to leptomeningeal melanosis confirmed by surgical biopsy. Brain and spine magnetic resonance images showed posterior fossa malformation compatible with the Dandy-Walker complex, hydrocephalus, and extensive enhancement of posterior fossa then spine. Shunt placement, corticotherapy, and chemotherapy were attempted leading to transient relief but the boy died 12 months after the onset of primary neurological symptoms. DISCUSSION: We discuss diagnosis, pathogenesis, management, and prognosis in the light of data from the recent literature. CONCLUSION: Neurocutaneous melanosis is considered to follow from neurulation disorders which could account for associated developmental malformations as the so-called Dandy-Walker complex. Cutaneous lesions are usually recognized at birth whereas neurological manifestations develop later. Numerous neurological symptoms have been reported according to extent of leptomeningeal and parenchymal infiltration. Whether magnetic resonance imaging of the neuroaxis represents the choice radiological exam, definite diagnosis relies upon the histological data obtained by mean of biopsy. Once symptomatic, surgical and medical measures remain palliative since death occurs within 3 years.

Clinical epidemiology for childhood primary central nervous system tumors.

This work was conducted by the French Brain Tumor Data Bank (FBTDB) and aims to prospectively record all primary central nervous system tumors (PCNST), in France, for which histological diagnosis is available. Results concerning children are presented. This study analyzes the childhood cases (0-19 years) of newly diagnosed and histologically confirmed PCNST (during the years 2004-2006) which have been recorded by the FBTDB. All French neuropathology and neurosurgery departments participated in this program. Neurosurgeons and neuropathologists completed a data file containing socio-demographic, clinical, radiologic and anatomopathologic information. The Tumor Registry from Herault was authorized to compile the data files with personal identifiers. About 1,017 cases (533 boys and 484 girls) of newly diagnosed childhood PCNST have been recorded (gliomas: 52%, all other neuroepithelial tumors: 31%, craniopharyngioma: 5%, germ cell tumors, meningioma and neurinoma: approximately 3% each, all histological subtypes have been detailed). Tumor resections were performed in 83.3%, and biopsies in 16.7%. The distributions by histology, cryopreservation of the samples, age, sex, tumor site and surgery have been detailed. To our knowledge, this work is the first databank in Europe dedicated to PCNST that includes the collection of clinical, radiological and histological data (including cryopreservation of the specimen). The long term goals of the FBTDB are to create a national registry and a network to perform epidemiological studies, to implement clinical and basic research protocols, and to evaluate and harmonize the healthcare of children and adult patients affected by PCNST.

Craniopharyngioma and hypothalamic obesity in children.

BACKGROUND AND PURPOSE: Obesity is a major concern in children treated for craniopharyngioma and is caused by hypothalamic damage. The role of aggressive surgical removal has been questioned, leading some authors to recommend a minimalist approach. In order to test this hypothesis, we decided to study obesity in craniopharyngioma and the factors related to it. MATERIALS AND METHODS: We reviewed retrospectively our series of pediatric craniopharyngiomas operated since 1981. The body-mass index (BMI) was calculated for each patient pre- and at several intervals postoperatively and expressed as standard deviations (SD) adjusted for age and gender. RESULTS: We operated on 45 cases, which were followed up for a mean duration of 11.0 years. Initial resection was total in 25 cases (55.6%). No patient died because of surgery or tumor progression; two died with delay presumably because of endocrine failure. At last control, 28 patients (62%) had obesity (BMI over +2SD). Hypothalamic involvement was significantly correlated with preoperative and postoperative BMI. Subtotal tumor resection was significantly associated with obesity at last control. Reoperation for tumor recurrence was associated with a significantly higher BMI. CONCLUSIONS: Our results suggest that obesity results from hypothalamic lesions caused by the tumor rather than by surgery. The postoperative weight gain appears to result from the continued impact of preoperative hypothalamic damage. The high rate of tumor recurrence in children, with the risk of additional damage to the hypothalamus, incites us to recommend total resection whenever it appears safe during initial surgery.

Management of cystic craniopharyngiomas with stereotactic endocavitary irradiation using colloidal 186Re: a retrospective study of 48 consecutive patients.

OBJECTIVE: We retrospectively reviewed the results of stereotactic endocavitary irradiation with colloidal 186Re in the treatment of cystic craniopharyngiomas. METHODS: Among 54 consecutive patients included in a 186Re stereotactic endocavitary irradiation procedure, 42 patients (29 male and 13 female; average age, 38.7 years) received therapeutic injection via intracystic catheter introduced under stereotactic conditions. The mean follow-up period was 43 months (range, 8-148 months). Endocavitary irradiation was the primary treatment for 15 patients and an adjuvant treatment for 27 patients. The mean volume before irradiation was 8.07 mm3, and the mean radiation dose was 381 MBq. RESULTS: Cystic mean volume after irradiation was 2.6 cm3 (P < 0.001 with initial volume). The retraction of the cyst was complete for 17 patients (44%) and partial for 17 (44%). In partial retraction, cystic volume decreased more than 50% for 12 (3%) patients and less than 25% (13%) for 5. Cystic volume remained the same or increased for 5 (13%) patients. Concerning visual functions, 12 (44.5%) patients improved and 2 worsened, despite tumor control. No endocrinological deterioration occurred. Among 17 patients with preoperative cognitive dysfunction, 10 improved and 6 had no change. One patient showed memory alteration 4 months after stereotactic endocavitary irradiation despite cyst volume reduction. No mortality occurred. CONCLUSION: 186Re stereotactic endocavitary irradiation for the treatment of cystic craniopharyngiomas is a safe and effective procedure with a tumor control rate greater than 70%. This treatment can be used for first-intention treatment of pure cystic craniopharyngiomas or for second-intention treatment of cystic recurrences.

Intracranial teratomas in children: the role and timing of surgical removal.

OBJECT: In this study, the authors report their experience with the surgical treatment of intracranial teratomas with an emphasis on the indications for delayed resection after oncological treatment. METHODS: The authors retrospectively reviewed the cases of 14 children with intracranial teratomas. The mean age at diagnosis was 10.5 years (range 2 days-18 years), and 11 patients were male. The final histological analysis revealed pure mature teratoma in 5 cases, mixed teratoma with germinoma in 3 cases, and nongerminomatous malignant germ cell tumor in 6 cases. Thirteen patients underwent tumor resection, and these patients were divided into 2 subgroups according to the timing of surgery. In Group A, 10 patients underwent resection as the primary treatment because no tumor markers were detected in 4 patients, a teratomatous component was revealed on biopsy sampling in 3 patients, and a large tumor volume in 3 patients. In Group B, 3 patients underwent removal of residual pure mature teratoma after oncological treatment. RESULTS: Seven of the 8 patients (87.5%) with pure mature teratomas or with mixed teratoma and germinoma are currently alive (mean follow-up of 9 years); the eighth patient died of postoperative meningitis. Two of the 6 patients (33%) with mixed nongerminomatous malignant germ cell tumors died of tumor progression regardless of the timing of surgery. CONCLUSIONS: The results of this study support the belief that microsurgical removal is the only effective treatment for intracranial teratomas. Surgery may be performed as the primary therapy when there is evidence of a noninvasive teratoma, and as a secondary therapy if there is only a partial response to neoadjuvant therapy or if progression is observed in mixed malignant germ cell tumors.

Disappearance of a middle cerebral artery aneurysm associated with Moyamoya syndrome after revascularization in a child: case report.

INTRODUCTION: Pediatric Moyamoya disease is rarely associated with intracranial aneurysms. We report a case of a 7-year-old girl with an antecedent of persistent craniopharyngeal canal, who presented with a history of choreiform movements. MATERIALS AND METHODS: A Moyamoya disease was found with an unruptured left middle cerebral artery aneurysm on her first angiography. Conservative treatment was chosen for the aneurysm and she underwent indirect revascularization by encephalosynangiosis using the multiple bur-hole technique for her Moyamoya disease. Abnormal movements were improved. Control angiogram at 6 months showed development of intracranial-extracranial anastomoses with complete resolution of the aneurysm. Aneuryms including the major arteries of the basal arterial circle occur as a by-product of the high velocity and blood flow secondary to the arterial stenosis. Blood flow modification after revascularization often lead to spontaneous regression and disappearance of these aneurysms. CONCLUSION: Therefore, a conservative treatment of these proximal aneurysms must be chosen after encephalosynangiosis.

Bifocal mixed germ-cell tumor with growing teratoma syndrome and metachronous mature metastases: case report.

The authors report the case of a 16-year-old male who presented with a bifocal diencephalic tumor arising both in the neurohypophysis and the pineal region with hydrocephalus. The histological diagnosis obtained during endoscopic ventriculocisternostomy was germinoma. MRI revealed an increase of the neoplasm during chemotherapy with recurrent obstructive hydrocephalus. A new ventriculostomy was performed followed by total surgical resection. The final histopathological analysis demonstrated an immature teratoma. Subsequently, this patient developed metachronous cystic metastases in the cerebello-pontine angles, which were resected and identified as mature teratoma, then we observed a lesion of the brachium conjunctivum which stayed stable after 29 consecutive months. The patient is alive and feels well 6 years after the initial diagnosis and 5 years after the first metastasis. To our knowledge, this is the fifth case of the growing teratoma syndrome located in the brain but the first case with simultaneously bifocal location and infratentorial disseminated metastasis. Obviously surgical removal is the treatment of reference for teratomas. Metastases of teratoma can be mature and may be amenable to surgery with a favorable outcome. Primary intracranial germ-cell tumors (CGT) arise in the midline of the brain and are located in the diencephalon. The peak incidence occurs during the second decade of life. Germ cell tumor (CGT) includes germinomas and non-germinomatous tumors, mature and immature teratomas account for 19.6 % [1]. Curiously, teratomas are able to grow during the first weeks of chemotherapy while serum markers remain normal. This situation was originally described and designed as "the growing teratoma syndroma" (GTS) in primary testis tumors by Logothetis in 1982 [2]. Here we report the rare occurrence of a GTS in a teenager who presented metachronous cystic metastases located in posterior fossa which were histologically mature.

Antenatal diagnosis of a third ventricular mass: fetus in fetu or teratoma?

BACKGROUND: Fetus in fetu (FIF) is a rare condition in medicine where an aberrant monozygotic twining results in inclusion of a degenerated fetus inside its twin bearer. Whereas FIF is generally defined as a fetiform vertebrate mass, teratoma is considered as a tumor composed of disorganized tissues derived from the three embryological layers. Recent data plead for a common origin and suggest a continuum between FIF and teratoma. CASE HISTORY: We describe the case of an intraventricular mass diagnosed by prenatal imaging and associated with triventricular hydrocephalus. Surgical removal of a non-vertebrate mass covered by normal skin was performed when the child was 15 months old. Now aged of 30 months, the child continues to develop normally. Histological data were in favor of a FIF. DISCUSSION: We discuss the diagnosis, pathogenesis, and prognosis in the light of data from the recent literature. CONCLUSIONS: Aberrant monozygotic twining leading to inclusion of a degenerate fetus inside its twin bearer results in FIF or teratomas. Surgical removal represents the main treatment. Intracranial FIF remains an extremely rare congenital condition, with a favorable oncological prognosis; the developmental prognosis appears conditioned by the absence of other malformation, the small size of the lesion, and the early management of intracranial hypertension.

Isolated bilateral coronal synostosis: early treatment by peri-fronto-orbital craniectomy.

Early strip craniectomy is commonly used for the treatment of craniosynostosis, but its effect on bilateral coronal synostosis remains disappointing. The technique developed in our institution of early (1-3 months of age) extensive perifrontal craniectomy that provides liberation of the anterior skull base is detailed, and its results in 12 cases of nonsyndromic brachycephaly are presented. Analysis of the growth of the cranial diameters of these children over a mean postoperative period of 10 years revealed a noticeable lengthening of the skull similar to the normal range, whereas no effect was noted on transversal cephalic development. Two patients needed additional surgery for focal correction despite a good morphologic result. The functional outcome was normal in all cases. For a number of carefully selected patients, early peri-fronto-orbital craniectomy provides an excellent result with functional preservation and morphologic improvement in most cases (10/12) in nonsyndromic brachycephaly. Its limited dissection and bleeding, as well as its short operating time, allow to have the procedure at a very young age and do not preclude the possibility of secondary surgery when needed.

Craniopharyngiomas in children: recurrence, reoperation and outcome.

INTRODUCTION: Craniopharyngioma bears a high rate of recurrence and morbidity in childhood. Although the outcome after recurrence and reoperation is an important parameter for the long-term evaluation of craniopharyngioma, it is poorly documented in literature. MATERIALS AND METHODS: We studied children reoperated for recurrent craniopharyngioma in our institution since the advent of computed tomography (CT) scanner. Reoperation for tumor resection was decided whenever the recurrence was solid, with the aim total resection if possible and reasonable. Stereotactic techniques were used in case of cystic recurrence, and external irradiation was used only in case of recurrent tumor not amenable to surgery. RESULTS: From September 1981 to January 2007, we performed one or more reoperations in 20 children presenting with recurrent craniopharyngiomas. The total number of reoperations was 29: resection was total in 12 cases, near-total in 8 cases, partial in 8 cases, and undocumented in 1. In addition, stereotactic procedures were performed in 11 cases, and 5 patients underwent external irradiation. DISCUSSION: No patient died after surgery or because of tumor progression; one patient died abruptly of an undiagnosed cause during external irradiation. The event-free survival after reoperation was 49.9% at 5 years and 40.0% at 10 years. At last control, after a mean follow-up of 70.4 months after the last surgery, nine patients were tumor-free and ten had stable disease. CONCLUSION: Reoperation for recurrent craniopharyngioma is an efficient method for tumor control and should be proposed whenever the recurrent tumor is solid. Morbidity results above all from tumor aggressiveness, rather than from surgical damage.