Prenatally Symptomatic Suprasellar Arachnoid Cyst: When to treat? A case-base update.

INTRODUCTION: Suprasellar Arachnoid Cysts (SAC) are rare heterogeneous entities. Though prenatally diagnosed, they are rarely treated pre-birth. Symptomatic cases are mainly seen in infants. CASE DESCRIPTION: We describe a case of a prenatally symptomatic suprasellar arachnoid cyst treated postnatally. The cyst was diagnosed on a routine ultrasound at 22 weeks, was rapidly evolving in the ultrasounds and the MRI of the 29th week. It then became symptomatic at 30 weeks with episodes of fetal bradycardia, independent to the uterine contractions. Antenatal treatment was discussed but delivery decided in emergency despite the prematurity via C-section. Though well tolerated postnatally, the cyst continued to grow. Endoscopic ventriculo-cysto-cisternostomy was performed on the 5th day of birth. Despite progressive reduction of the cyst, residual brainstem compression and evolving ventriculomegaly lead to a transient extrathecal internal shunting. DISCUSSION/CONCLUSION: Our case suggests that prenatally diagnosed cysts require a close follow-up. Treatment options and timing should be adapted to anatomy, cyst evolution and symptoms whether it is before or after birth.

Partial hybrid cranial vault remodeling in late correction of scaphocephaly and revision surgery. A monocentric retrospective study of nine consecutive cases.

There is currently no consensus on the surgical attitude to be adopted for late management of sagittal synostosis or for revision scaphocephaly surgeries without prolapse of the cerebellar tonsils. We present here a monocentric retrospective study of nine patients operated consecutively by a fixed expansion method of the cranial vault which may be associated with a fronto-orbital remodeling. The procedure consists in the realization of multiple parietal tongue-in-groove osteotomies fixed by resorbable plates. Simultaneous fronto-orbital remodeling have been performed when needed. No intraoperative complication was noted. The average operating time was 141min. Six patients (66.7%) had a blood transfusion during the perioperative period. The average hospital stay was 4.8days. With a mean follow-up of 26,7months, no surgical revision was noted. In all patients with clinical or ophthalmologic signs of intracranial hypertension, we highlighted a disappearance of signs within 4months. No protective helmet has been used. The craniofacial remodeling was judged very good by the family and the surgical team. Multiple tongue-in-groove tenons remolding cranioplasty associated or not with a fronto-orbital advancement is a safe technique. It seems to us to be a good alternative to floating or fixed bi-parietal or bi-parieto-frontal cranial flaps because of the very large increase in endocranial volume and the possibility of physiologically and aesthetically remodeling the parietal and fronto-orbital regions.

Sphenoidal sinogenic extradural empyema associated with juvenile myelomonocytic leukemia.

Intracranial empyema is a rare but serious complication of sinusitis in children. Myelodysplastic/myeloproliferative syndromes (MMS), including juvenile myelomonocytic leukemia (JMML), can lead to immunosuppression, thus favouring infections. We report the case of a sphenoid sinogenic retro-clival extradural empyema in a 14-year-old female patient associated with JMML. Treatment consisted in an endonasal transphenoidal drainage of the empyema associated with intravenous antibiotherapy. The patient was thereafter enrolled in chemotherapeutic treatment with Azacitidine. The disease progressed to blast phase, indicating bone marrow graft. This is the first reported case of an endocranial complication of bacterial sinusitis associated with MMS in a child.

[Diagnosis, evolution and prognosis of prenatally diagnosed suprasellar cysts]. / Diagnostic, évolution et pronostic des kystes supra-sellaires de découverte anténatale.

Suprasellar arachnoid cysts (SAC) in children are considered rare, but the incidence is increasing due to the improvement of prenatal diagnosis. We present 15 cases of SAC diagnosed during the antenatal period between 2005 and 2015. The records were reviewed retrospectively by specifying the radiological characteristics, treatment modalities, outcomes, and long-term monitoring. Mean follow-up was 71 months. The forms (SAC-1) accounted for 2 cases (13%) with hydrocephalus. We observed 8 (53%) lower forms (SAC-2) with interpeduncular cistern expansion without hydrocephalus. The 5 (33.5%) remaining patients showed asymmetrical forms (SAC-3). Six patients (40%) were treated by ventriculo-cysto-cisternostomy, 1 by fetoscopy, 1 (6.5%) by ventriculo-peritoneal shunt, 2 (13.5%) by pterional craniotomy, and 6 (40%) were simply followed. The surgical outcomes were initially favorable in 9 cases (100%), 1 patient (13%) had to be re-treated later. Non-operated patients were all type 2 and showed no radiological changes. In the long-term, 1 patient (6.5%) had endocrine disruption, 1 had delayed development, 2 (13.5%) had minor neuropsychological impairments, and 1 had epilepsy. Combined monitoring with prenatal MRI and ultrasound can be used to distinguish three subtypes of SAC. SAC-1 and SAC-3 have an excellent prognosis after treatment in the perinatal period. SAC-2 can benefit from simple monitoring and remain asymptomatic in their majority. This classification allows a better prognosis estimation and better treatment decision.

[Infant rhabdoid tumors: a diagnostic emergency]. / Tumeurs rhabdoïdes du nourrisson : une urgence diagnostique.

Rhabdoid tumors are a heterogeneous family of aggressive tumors affecting young children. Their grouping within a single entity is recent, following the discovery of a bi-allelic inactivation of the hSNF5/INI1 tumor suppressor gene in tumoral cells. This bi-allelic inactivation of the hSNF5/INI1 gene found at the constitutional level in up to one-third of cases has led to the identification of a predisposal syndrome to rhabdoid tumors. Herein we report extrarenal rhabdoid tumors observed in three infants between 3 and 6 months of age, underlining the misleading feature of the clinical presentation and the aggressiveness of the disease. Finally, we also report the genetic patient care management strategy.

[Takotsubo cardiomyopathy in an adolescent girl]. / Cardiomyopathie de type tako-tsubo chez une adolescente.

We report the case of a 14-year-old girl who presented with symptoms of left ventricular systolic failure following the rupture of a cerebellar arteriovenous malformation. Takotsubo cardiomyopathy needs to be recognized by pediatricians because the triggering factors, such as physical or emotional stress, are common during childhood. Echocardiography showed a typical dyskinesia of the left ventricular apical or midventricular segments with a hyperkinetic basal region. Symptomatic treatment may be necessary in cases of hypotension, arrhythmias, or acute heart failure. As the prognosis is generally good, reassuring information can be given and cardiologic investigations limited.

Nonaccidental head injury.

Nonaccidental head injury in children (NAHI), most often due to abusive head trauma (AHT), is not uncommon and carries a high risk of mortality and morbidity. Intracranial lesions encountered are mainly subdural hematomas. Despite heterogeneous clinical presentation, symptoms are related to brain edema with intracranial hypertension and/or seizures that should prompt early diagnosis and treatment. Two main differential diagnoses are glutaric aciduria type 1 and Menkes disease, two inborn errors of metabolism that require specific diagnostic procedures and treatment. The aim of emergency treatment is to control seizures, prevent seizure recurrence, and decrease intracranial pressure. Because NAHI/AHT carries a worse prognosis than accidental head traumatism - with specific risk factors such as initial clinical status, age, aspect of initial CT scan, associated retinal hemorrhage, seizure recurrence - these children need close clinical, neurological, and radiological monitoring in neurointensive care units. It is therefore important to identify the nonaccidental origin of the injury at the earliest opportunity so that the appropriate intervention, including social evaluation and reporting suspected cases to judicial authorities, can be made to protect the child from further harm.

[Skin markers of communicating dermal sinus: report of two cases]. / Anomalies cutanées des sinus dermiques communicants: à propos de 2 cas.

Skin abnormalities in the spinal lumbosacral region of infants are not infrequent. Physicians should look for underlying spinal malformations. We report 2 cases of dermal sinus in infants revealed by severe, acute meningitis. The dermal sinus is an abnormal tract between the skin and underlying neural structures, most often located in the lumbosacral region. It may end blindly in the subcutaneous tissues, or it may extend into the medullary space. The diagnosis should be suspected in patients with other skin findings: localized hypertrichosis, subcutaneous lipomas, hyperpigmented lesions, etc. It must be distinguished from the very frequent coccygeal pits (4% of newborns), which require no treatment. Magnetic resonance imaging should be done every time the diagnosis is suspected. Recurrent bacterial meningitis or meningitis caused by unusual bacteria is frequently associated with dermal sinus. A variety of neurologic abnormalities have been described: sensory changes, motor weakness, reflex changes, abnormal bowel and bladder function, etc. Infants generally have few neurologic symptoms, such as delayed walking or sphincter control. Orthopaedic abnormalities are rare. The dermal sinus tract needs to be detected at an early stage in order to avoid these complications, which can be prevented by early surgery.

[Idiopathic intracranial hypertension: indication for cerebrospinal fluid shunting]. / Hypertension intracrânienne idiopathique: indication d'une dérivation du liquide cérébrospinal.

Idiopathic intracranial hypertension is clearly defined by the Friedmann and Jacobson criteria, followed by the McGirt et al. criteria. Several therapeutic options are still being debated. Cerebrospinal fluid shunting is the most common treatment, because it is simple, non invasive, and has a low rate of complications.

[Passage from childhood to adulthood and craniovertebral malformations]. / Passage de l'enfant à l'adulte et malformations de la charnière crâniorachidienne.

Craniovertebral malformations are infrequent in children. Their causes are numerous (Chiari, congenital bone diseases, metabolic diseases, and genetic anomalies). When symptomatic (seldom), these malformations require surgical decompression and fixation. Progress in therapy probably will postpone the occurrence of these signs and require cooperation between pediatric and adult physicians.

[Transition from childhood to adulthood and lumbosacral dysraphism]. / Passage de l'enfant à l'adulte et dysraphisme lombosacré.

The diagnosis of lumbosacral dysraphism is now made during the first year of life. Although problems due to filum lipomas are solved when the patient becomes an adult, the lipomas of the lumbar spinal cord can induce late complications. It is therefore necessary to organize a neurological, urological, and obstetrical follow-up to: screen for late retethering and/or deterioration ; preserve kidney function and monitor the consequences of the different procedures performed during childhood ; support pregnancies (delivery, prenatal screening for Currarino syndromes).

[Transition from childhood to adulthood and management of spasticity]. / Passage de l'enfant à l'adulte et prise en charge de la spasticité.

The authors report specific concerns on the transition to adulthood in cerebral palsy patients, with particular attention devoted to spasticity treatment follow-up.

[Epilepsy and focal lesions in children. Surgical management]. / Epilepsie et lésions focales chez l'enfant. Traitement chirurgical.

Epilepsy surgery has gained a large role in the treatment of intractable seizures in the last few decades because of the development of operative techniques and better identification of the cerebral anomalies using electrophysiological recordings and neuroimaging. A series of 419 children, aged from five months to 15 years, with epilepsy (medically refractory in 85.5% of them) associated with focal cortical lesions, who underwent surgery between 1986 and 2006 was analyzed to identify the factors that correlated with outcome. Mean follow-up was 5.2 years. According to Engel's classification; 75.8% of the children were seizure-free. When the lesion was well defined, correlations between clinical data, radiological features and electrophysiological features, suggesting a zone of seizure onset around (or even in) the lesion, was the best guarantee of achieving good seizure control by lesionectomy. Nevertheless, seizure outcome was also determined by other factors: the duration of the epilepsy and the surgery. Persistence of seizures was found to be significantly associated with the preoperative duration of epilepsy, the completeness of the lesional resection and de novo brain damage induced by the surgical procedure itself. Early surgery must be considered in children because of the benefits of seizure control on the developing brain and the risk of secondary epileptogenesis.

[Adult supratentorial oligodendrogliomas. Surgical treatment: indications and techniques]. / Oligodendrogliomes supratentoriels de l'adulte. Traitement chirurgical: indications et techniques.

Surgical resection is the first step in the treatment of adult supratentorial oligodendrogliomas (OLG). However, the role of resection on prognosis, the most appropriate time for surgery along the natural history of those tumors, and the best operative strategy remain debated. Survival curves after resection vary greatly among reported series, in particular as a result of a persisting confusion in identification and classification of cerebral OLG. Surgical or stereotactic biopsy is the first surgical procedure which enables confirmation of the diagnosis suggested on imaging, assessment of extension of tumor cell infiltration beyond abnormalities limit described an imaging, and currently available molecular biology studies. Biopsies may be the only surgical procedure in patients having a deep-seated tumor with minimal mass effect, or prior to a surgical resection or a "wait and watch" strategy. Surgical resection is indicated for the other patients. However, it has not been demonstrated that time for resection has an influence on survival, excepted in patients with rapidly growing tumors, with mass effect causing increased intracranial pressure. A wait and watch strategy is therefore warranted in patients with a tumor aspect suggestive of a grade A OLG; surgical resection may be indicated later. There is a current trend for maximal safe resection, preserving functional cerebral areas, since truly complete resection of the tumor including infiltration is exceptional. However, from the contradictory results reported to date, one cannot ascertain whether large or complete resection based on imaging is associated with significantly longer survival. Neuronavigation guidance, intraoperative imaging, and cortical stimulation techniques are helpful neurosurgical techniques enabling maximal safe resection with preservation of functional areas.

[Results of the Sainte-Anne - Lyons series of 318 oligodendroglioma in adults]. / Résultats de la série Sainte-Anne - Lyon de 318 oligodendrogliomes intracrâniens de l'adulte.

INTRODUCTION: Incidence of cerebral oligodendrogliomas is increasing because of better recognition made possible by improved classifications. We studied a homogeneous series using the Sainte-Anne grading scale in order to better understanding the history of these tumors with or without treatment and to assess prognosis and associated factors. PATIENTS AND METHODS: A retrospective series of 318 adult patients with oligodendroglioma (OLG) treated at Hôpital Sainte-Anne, Paris (SA) and Hôpital Neurologique, Lyons (L) between 1984 and 2003 was analyzed: 182 grade A OLG (SA + L), 136 grade B among which a homogenous series of 98 (SA) were included. For grade A: age at diagnosis ranged from 21 to 70 (mean: 41), sex ratio was 1.28. For grade B: age at diagnosis ranged from 12 to 75 (mean: 45.5), sex-ratio was 1.58. The main first symptoms were: epilepsy (A: 91.5%; B: 76%), intracranial hypertension (A: 7.9%; B: 14.6%), neurological deficit (A: 5.1%; B: 17.7%). The most frequent locations were: frontal, insular and central for both A and B. Mean size was 55 mm for grade A, 62 mm for B. Calcifications were found in 20% of A, 48.5% of B. No tumor was enhanced on imaging (CT/MRI) in grade A, all but 7 in grade B. All patients underwent surgery either for biopsy (A: 47.2%; B: 53%), or removal which was partial (A: 26.4% vs B: 19.4%) or extended (A: 36.3% vs B: 37.8%). Fifty-six patients underwent 2 procedures and 12 three procedures. Radiotherapy was performed in 76.9% of grade A, and 91% of B patients, in the immediate postoperative period for 71% A and 82.7% B. Chemotherapy was delivered for 36% of grade A (in the event of transformation to grade B or failure of radiotherapy) and 67.5% of B patients. Among grade A tumors, 38% transformed into grade B within a mean delay of 51 months with a mean follow-up of 78 months. RESULTS: Median survival was 136 months for grade A and 52 for grade B. Survival at 5, 10 and 15 was 75.5%, 51% and 22.4% for grade A vs 45.2%, 31.3% and 0% for grade B respectively. In univariate and multivariate analysis, grade A survival was associated with age at diagnosis, tumor size, large removal and response to radiotherapy. Grade B survival was associated with age at diagnosis, wide removal and sharply defined limits of the tumor on imaging. CONCLUSIONS: Analysis of both published data and this series underlines many prognostic parameters. It shows that OLG are heterogeneous tumors even in each grade (A and B). Treatment should consequently progress towards more targeted procedures for patients mainly with postoperative radiotherapy and chemotherapy.

Intraventricular haemorrhage from a renal cell carcinoma pituitary metastasis.

Symptomatic pituitary metastasis and intraventricular haemorrhage from a cerebral metastasis are exceptional events in the natural history of a renal cell carcinoma. We report the first case of a metastatic renal cell carcinoma to the pituitary gland presenting with intraventricular haemorrhage. The origin of intraventricular haemorrhage and its association with renal cell carcinoma pituitary metastasis are discussed.

Long term course of the H reflex after selective tibial neurotomy.

OBJECTIVES: This study was conducted to evaluate the long term clinical and electrophysiological outcome by recording the H reflex in a consecutive series of six patients treated by selective tibial neurotomy for spastic equinus foot. METHOD: The amplitudes of Hmax reflexes, Mmax responses, and Hmax:Mmax ratio were recorded in six patients with chronic lower limb spasticity, before and after surgery, at day 1 and 8 months and 24 months after selective tibial neurotomy. The passive range of movement, the stretch reflex score according to the Tardieu scale, the osteoarticular and tendon repercussions, and the quality of motor control of dorsiflexion were evaluated preoperatively and postoperatively. RESULTS: At the end of the study, all patients presented a reduction of equines. Gait and Tardieu's score of spasticity had improved in all patients. Active dorsiflexion of the ankle was unchanged in four patients, but two improved by 5 degrees to 12 degrees. In five cases, fascicular resection of the superior nerve to soleus was, alone, sufficient to reduce spastic equinus foot, without recurrence, for a mean follow up of 28 months. Two patients were reoperated on, one for remaining spasticity related to an underestimated spasticity of the gastrocnemius muscles, and the other for painful claw toes. Hmax, Mmax, and Hmax:Mmax ratios were significantly lower the day after surgery. The reduction of Hmax and Hmax/Mmax ratio remained stable over time and was still statistically significant two years after the operation. However, the value of Mmax eight months postoperatively was no longer significantly different from the preoperative value. CONCLUSION: This study shows the long term efficacy of the selective tibial neurotomy as treatment of spastic equinus foot. Neurotomy confined to fibres supplying the soleus muscle is sufficient in most cases and acts by decreasing sensory afferents without significant long term motor denervation.