Antonio López López-Guerrero

Hydrocephalus and arachnoid cysts

AimThe management of arachnoid cysts (AC) remains controversial. An additional problem derives from the management of hydrocephalus associated with an AC. In this work, we discuss existing procedures proposed in the current literature for their treatment.MethodsWe reviewed selected reports on intracranial ACs placing special interest in those about the association of hydrocephalus and ACs. We also briefly surveyed data of our patients with this association.Results and discussionHydrocephalus is often found in midline and posterior fossa ACs. Interhemispheric lesions may also evolve with ventriculomegaly, while middle fossa lesions rarely produce hydrocephalus. Patients’ age, cyst location and size, and macrocephaly have all been related to the development of hydrocephalus. Some authors remark on the role played by hydrocephalus and hypothesize that some ACs would result from disturbed cerebrospinal fluid (CSF) dynamics. They also propose that ACs might represent a localized form of hydrocephalus. We also comment on hydrocephalus in relation to the diverse locations of ACs. Neuroendoscopic techniques have transformed previous ways of management as cystoperitoneal shunting and open fenestration.ConclusionsACs may be pathogenetically related with hydrocephalus, and conversely, ACs may cause hydrocephalus. In some patients, aberrant CSF dynamics seems to play a major role in the development of both cyst and hydrocephalus. Hydrocephalus and ACs may be treated exclusively with neuroendoscopic procedures, although some patients will still require CSF shunting. The ideal option seems to consist of choosing the method that offers the highest success with a single procedure for treating the hydrocephalus and the AC simultaneously.

Massive hemorrhage in hemangioblastomas

Massive hemorrhage is a very uncommon event among hemangioblastomas. Forty-four cases have been reported before this review. Thorough analysis of all reported cases on literature was accomplished. The majority presented as parenchymatous or subarachnoid bleedings. Subarachnoid hemorrhage was only associated with spinal hemangioblastomas, while parenchymatous bleedings were more, but not only, originated from cranial instances. Ventricular hemorrhage from a hemangioblastoma was exceptional, with only two previous cases bleeding directly into the ventricular compartment. To our knowledge, the illustrative case is the first one of pure tetraventricular hemorrhage from a hemangioblastoma. When hemangioblastoma bleeding occurs, it is usually the very first clinical presentation of a previously undetected tumor. Solid type, large size, and spinal–radicular locations are more frequently related to hemorrhage. Hemorrhage following hemangioblastoma embolization and the association of this tumor with other bleeding lesions, such as arteriovenous malformations and aneurysms, is also discussed.Massive hemorrhage is a very uncommon event among hemangioblastomas. Forty-four cases have been reported before this review. Thorough analysis of all reported cases on literature was accomplished. The majority presented as parenchymatous or subarachnoid bleedings. Subarachnoid hemorrhage was only associated with spinal hemangioblastomas, while parenchymatous bleedings were more, but not only, originated from cranial instances. Ventricular hemorrhage from a hemangioblastoma was exceptional, with only two previous cases bleeding directly into the ventricular compartment. To our knowledge, the illustrative case is the first one of pure tetraventricular hemorrhage from a hemangioblastoma. When hemangioblastoma bleeding occurs, it is usually the very first clinical presentation of a previously undetected tumor. Solid type, large size, and spinal–radicular locations are more frequently related to hemorrhage. Hemorrhage following hemangioblastoma embolization and the association of this tumor with other bleeding lesions, such as arteriovenous malformations and aneurysms, is also discussed.

Syringomyelia with quadriparesis in CSF shunt malfunction: a case illustration

BackgroundShunt malfunction typically presents with headaches, vomiting, and impaired consciousness. Paraparesis has been rarely reported as the initial manifestation of valve dysfunction.Case illustrationA 17-year-old boy was admitted with invalidating quadriparesis that after neuroimaging evaluation was found to be produced by communicating syringomyelia attributed to shunt malfunction.Discussion and conclusionPersistence of the central canal in association with communicating hydrocephalus may lead to the development of communicating syringomyelia. In this context, insufficient drainage of CSF produced by shunt dysfunction may evolve exclusively with symptoms and signs of spinal cord involvement in the absence of the usual cerebral manifestations related to shunt failure.

Bilateral ethmoidal dural arteriovenous fistula: Unexpected surgical diagnosis

Dural arteriovenous fistulae (DAVFs) are infrequent lesions, the most common locations of which are the cavernous, sigmoid and transverse sinuses. The cribiform plate is one of the less frequent sites for DAVFs, where they entail a high hemorrhage risk. Feeding arteries for ethmoidal DAVFs can be uni- or bilateral. However, the draining fistulous system has classically been described as unilateral. The authors report the second case in literature of bilateral ethmoidal DAVF, which is defined as that with bilateral draining veins. The present case was diagnosed only after surgical exploration of both cribiform plates. No preoperative radiological test could detect the presence of a bilateral venous draining system from the ethmoidal DAVF. Possible reasons for that lack of presurgical diagnosis are discussed. Bilateral surgical exploration of the anterior cranial fossa is recommended when dealing with ethmoidal DAVFs, even when they seem to be unilateral on preoperative studies.

Acute cholecystitis complicating ventriculo-peritoneal shunting: report of a case and review of the literature

CaseA 3-year-old boy underwent emergency external ventricular drainage and excision of a fourth ventricle anaplastic ependymoma. A week later, the child was given a ventriculo-peritoneal shunt. Fourteen days after shunting, the child developed a subphrenic abscess and acute cholecystitis that required surgery.ResultsA Staphylococcus epidermidis was isolated both from the ventricular catheter and CSF and from the subphrenic abscess and the gallbladder. To our knowledge, this is the first report of cholecystitis evolving as a descending shunt infection. The current literature related with this unique complication is briefly reviewed.

Chiari type 1 anomaly in pseudohypoparathyroidism type Ia: pathogenetic hypothesis

AimThis study aims to report a patient with Chiari type 1 malformation (CM1) occurring in the context of pseudohypoparathyroidism type 1a (PHP-Ia) that we believe represents the first instance of this association in the current literature.Case reportThe authors describe the case of a 6-year-old girl diagnosed with PHP-Ia who presented an associated tonsillar descent. During the follow-up, the skull vault and the occipital squama became extremely thickened at the same time as the tonsillar herniation showed a marked regression.DiscussionChronic tonsillar descent has been reported in diverse genetic and metabolic diseases of bone. A constant finding in PH-Ia consists of changes that mainly involve the bones of the patients’ hands and feet. Cerebral anomalies have also been documented in PHP-Ia, especially cerebral calcifications, but in contrast involvement of the skull bones has seldom been described in this condition. The authors briefly discuss the probable role played by the observed skull changes in the origin and subsequent regression of the tonsillar descent in this child.ConclusionsWe suggest that CM1 may develop in patients with PHP-Ia and that it should be actively sought, especially in individuals diagnosed with PHP-Ia presenting with neurological manifestations. Probably, the seeming rarity of chronic tonsillar descent in PHP-Ia is due to the fact that many patients with this condition are rarely investigated with magnetic resonance.

Severe spinal cord injury in craniocervical dislocation. Case-based update

BackgroundCraniocervical distraction injuries, including atlanto-axial dislocation (AAD) and atlanto-ocipital dislocation (AOD), are often associated with severe spinal cord involvement with high morbidity and mortality rates. Many patients with these injuries die at the accident scene, but advances in emergency resuscitation and transport permit that many patients arrive alive to hospitals.DiscussionChildren with craniocervical distraction injuries usually present with a severe cranioencephalic traumatism that is the most relevant lesion at admission. After resuscitation and hemodynamic stabilization, the spinal cord damage appears as the main lesion. Apnea and quadriparesis, or quadriplegia, are usually present at the onset. Early diagnosis and management perhaps decrease life-threatening manifestations of the spinal lesion. But even so, the primary spinal cord insult is often irreversible and precludes obtaining a satisfactory functional outcome.Patients and methodsWe report the findings of four children with craniocervical distraction injuries (AOD and AAD) who presented with severe spinal cord damage. All patients were admitted with respiratory distress or apnea together with significant brain injuries. The medical records pertaining to these patients are summarized in regard to clinical features, management, and outcome.ConclusionsIn spite of timely and aggressive management, craniocervical injuries with spinal cord involvement continue to have a dismal prognosis. Outcome is closely related to the severity of the initial brain and spinal cord damage and is nearly always fatal in cases of complete spinal cord transection. Priority should be given to life-threatening complications. Ethic issues on indications for surgery deserve a detailed discussion with the children’s parents.

Clinical Manifestations of CSF Shunt Complications

Hydrocephalus and hydrocephalus-related problems occupy a large part of current neurosurgical activity, accounting for approximately 35–50 % of pediatric neurosurgical practice. At present, the most frequent treatment for hydrocephalus consists of cerebrospinal fluid (CSF) shunting, especially with ventriculoperitoneal (VP) shunts. Other types of CSF derivations, such as lumboperitoneal, ventriculopleural, ventriculo-gallbladder, subgaleal shunting, etc., continue to be in use, although they are less often utilized. Ventriculoatrial valves have almost totally been abandoned due to the severity of their complications. On the contrary, neuroendoscopic procedures, especially endoscopic third ventriculostomy (ETV), are increasingly being used in daily practice for avoiding shunt complications.

Intracranial hemorrhage following surgery for occult spinal dysraphism. Reply to E.M.J. Cornips and J. van Aalts’ letter to the editor

We very much appreciate the Letter to the Editor written by Dr. Cornips and Dr. van Aalst regarding our paper “Intracranial hemorrhage following surgery for occult spinal dysraphism: a case-based update” (Childs Nerv Syst 31:837–842, 2015) [1, 2]. We acknowledge their thoughtful and authoritative comments on the pathogenesis of this rare complication of spinal procedures that involve the purposeful or unintentional opening of the dura mater with the subsequent escape of CSF either during the surgery proper or after it [1]. Also, we mainly appreciate their suggestions for undertaking further possible measures of prevention that will undoubtedly contribute to improve the value of our paper [1]. CSF depletion in variable amounts occurs after lumbar puncture, spinal anesthesia, and a variety of spinal surgical procedures [2]. CSF losses are an almost constant feature in surgeries that involve durotomy. However, significant symptoms related to CSF depletion develop in few instances. The proposed mechanism for the production of intracranial hygromas or hematomas after some spinal operations seems to consist primarily of the loss of CSF when the dura mater is open (intentionally as part of the technique or accidentally as a complication of the operation) [2]. The true incidence of these collections is presently unknown, as cranial CT scans are not routinely performed in patients submitted to these procedures. The immediate consequence of the fluid loss is the diminution of the intracranial CSF volume. The fluid loss may originate either from within the cerebral ventricles or from the subarachnoid spaces surrounding the brain. The physiological response for counteracting the decrease of CSF consists of (1) an increase in the production of CSF and of (2) venous ingurgitation aimed at compensating for the reduced CSF volume in certain cases [2]. Depending on the intensity and/or duration of the escape of CSF, the resulting pathological condition would be the formation of a subdural hygroma or more rarely the production of a subdural hematoma [2]. As pointed out by Drs. Cornips and van Aalts, several factors might also contribute to the development of these collections as are the patients’ age, the presence of brain atrophy, the coexistence of shunted hydrocephalus, and others [1]. Drs. Cornips and van Aalst suggest the possibility of an associated head injury in our patient [1]. However, this occurrence can be ruled out because the child’s syncope was witnessed by us that permitted to hold the patient and avoided that the child could hit his head onto the floor. Regarding prevention, we agree with the measures suggested by Drs. Cornips and van Aalst, namely (1) placing the child in Trendelenburg position before dural opening, (2) plugging the subdural space by placing cottonoid patties on the exposed cord for avoiding the outflow of fluid during the surgery, and (3) using frequent irrigation of the surgical field with abundant warm saline [1]. We usually follow these measures too, except for the one regarding the Trendelenburg position. We instead insist on positioning the child as to avoid undue pressure on the abdomen and on avoiding excessive turning of the child’s head that would compromise the venous return and result in increased intracranial and intraspinal pressure, which might further increase the CSF loss. Before closing the durotomy, we also try to fill the spinal subarachnoid space with warm saline. At the end of the dural closure, we ask the anesthetist to perform a few Valsalva maneuvers to prove the impermeability of the sutured durotomy. Performing this maneuver seems * Juan F. Martinez-Lage juanf.martinezlage@gmail.com

Wind instruments and headaches

The authors illustrate the cases of two children with headaches, one diagnosed with Chiari type 1 malformation and the other with hydrocephalus, who played wind instruments. Both patients manifested that their headaches worsened with the efforts made during playing their musical instruments. We briefly comment on the probable role played by this activity on the patients’ intracranial pressure and hypothesize that the headaches might be influenced by increases in their intracranial pressure related to Valsalva maneuvers. We had serious doubts on if we should advise our young patients about giving up playing their music instruments.