Neurological manifestations of pediatric achondroplasia

1981 ◽  
Vol 54 (1) ◽  
pp. 49-57 ◽  
Author(s):  
Hiroshi Yamada ◽  
Shigetoshi Nakamura ◽  
Masataka Tajima ◽  
Naoki Kageyama

✓ The neurological and neuroradiological manifestations of pediatric achondroplasia are analyzed on the basis of 10 cases. In addition to the classical symptomatology of an enlarging head, with or without increased intracranial pressure, several patients presented symptoms related to a small foramen magnum. Respiratory problems and quadriparesis were also observed in these patients. Six patients who were treated by foramen magnum decompression showed remarkable improvement. Neuroradiological evaluation revealed a significant number of patients with dilated cortical sulci and basal cisterns, and mild dilatation of the ventricles on computerized tomography (CT) and/or ventriculography. Two patients showed signs of obstruction at the fourth ventricle outlets. Some exhibited anomalous dilatation of cerebral veins and dural sinuses, narrowing of the sinuses at the jugular foramen, and enlarged emissary veins. Ventriculoperitoneal or ventriculoatrial shunts were placed in three patients. In the other children with a large head and dilated ventricles, head growth curves paralleled the normal slope, and there was no significant clinical evidence of increased intracranial pressure or enlarging ventricles on follow-up CT scan.

1993 ◽  
Vol 78 (2) ◽  
pp. 297-300 ◽  
Author(s):  
Zain Alabedeen B. Jamjoom ◽  
Vinita Raina ◽  
Abdulfattah Al-Jamali ◽  
Abdulhakim B. Jamjoom ◽  
Basim Yacub ◽  
...  

✓ The authors describe a 37-year-old man with the classic clinical features of Hand-Schüller-Christian disease. He presented with symptoms of increased intracranial pressure due to obstructive hydrocephalus secondary to a huge xanthogranuloma involving falx cerebri and tentorium cerebelli. Immunohistochemical and ultrastructural studies failed to demonstrate Langerhans histiocytes, however. The implication of this finding is discussed in light of the recent relevant literature.


1971 ◽  
Vol 34 (3) ◽  
pp. 405-407 ◽  
Author(s):  
Salvador Gonzalez-Cornejo

✓ The author reports the safe and satisfactory use of Conray ventriculography in 26 patients with increased intracranial pressure and discusses his technique for this procedure.


1982 ◽  
Vol 56 (5) ◽  
pp. 660-665 ◽  
Author(s):  
David D. Weaver ◽  
H. Richard Winn ◽  
John A. Jane

✓ Four patients with unilateral mass lesions are presented in whom bilateral supratentorial subarachnoid pressures were continuously recorded. A significant pressure differential between the ipsi- and contralateral side was documented in each case. The possible relationship of this phenomenon to various factors involved in producing increased intracranial pressure, including cerebrospinal fluid flow dynamics, vascular reactivity, elasticity, and brain tissue pressure, are discussed. This study suggests that supratentorial subarachnoid pressure should be measured ipsilateral to the site of a focal mass lesion.


1985 ◽  
Vol 63 (4) ◽  
pp. 532-536 ◽  
Author(s):  
John R. Ruge ◽  
Leonard J. Cerullo ◽  
David G. McLone

✓ The authors present two cases of pneumocephalus occurring in patients with permanent shunts and review nine previously reported cases. Mental status changes and headache are the most common presenting symptoms. Six of the 11 cases of pneumocephalus occurred in patients with shunt placement for hydrocephalus secondary to aqueductal stenosis. In these patients, thinned cerebrospinal fluid barriers secondary to longstanding increased intracranial pressure may predispose them to pneumocephalus. Temporary extraventricular drainage is an effective method of treatment in this group of patients. Two other etiologies are identified with significance to treatment, and the role of craniotomy is discussed.


2002 ◽  
Vol 97 (3) ◽  
pp. 317-322 ◽  
Author(s):  
Jörg Klekamp ◽  
Giorgio Iaconetta ◽  
Ulrich Batzdorf ◽  
Madjid Samii

Object. Syringomyelia is often linked to pathological lesions of the foramen magnum. The most common cause is hindbrain herniation, usually referred to as Chiari I or II malformation. Foramen magnum arachnoiditis without either Chiari I or II malformation is a rare cause of syringomyelia. The authors undertook a retrospective analysis of 21 patients with foramen magnum arachnoiditis (FMA) and syringomyelia treated between 1978 and 2000 to determine clinical course and optimum management. Methods. In the review of records, 21 patients with FMA and syringomyelia were documented. A stable clinical course was demonstrated in three patients in whom surgery was not performed, and one patient refused surgical intervention. Seventeen patients underwent 23 operations to treat progressive neurological disease. Of these 23 operations, 18 involved opening of the foramen magnum, arachnoid dissection, and placement of a large dural graft. One patient underwent insertion of a ventriculoperitoneal shunt for treatment of accompanying hydrocephalus, one patient received a cystoperitoneal shunt for an accompanying arachnoid cyst; two syringoperitoneal and one syringosubarachnoid shunts were also inserted. Hospital and outpatient files, neuroimaging studies, and intraoperative photographic and video material were analyzed. Additional follow-up information was obtained by telephone interview and questionnaires. Standard and cardiac-gated magnetic resonance imaging studies are the diagnostic procedures of choice in these patients. Sensory disturbances, dysesthesias, and pain were the only symptoms likely to improve after foramen magnum surgery. Motor weakness and gait disturbances, which were severe in a considerable number of patients, and swallowing disorders tended to remain unchanged. As a consequence of the rather severe arachnoid lesions in most patients, clinical recurrences were observed in 57% over a 5-year period. Conclusions. Surgery for FMA and syringomyelia has to provide clear cerebrospinal fluid pathways between the cerebellopontine cisterns, spinal canal, and fourth ventricle. If this can be achieved successfully, the syrinx decreases in size and the clinical course of the patient may even improve. In patients with severe and widespread areas of arachnoiditis, however, multiple operations may be required at least to stabilize the clinical course.


1990 ◽  
Vol 73 (6) ◽  
pp. 927-932 ◽  
Author(s):  
Leslie N. Sutton ◽  
Alan C. McLaughlin ◽  
Stephen Dante ◽  
Mark Kotapka ◽  
Teresa Sinwell ◽  
...  

✓ In order to test the hypothesis that the cerebral arteriovenous oxygen difference (AVDO2) and venous oxygen content (VO2) could be used to monitor brain energy metabolism in the setting of increased intracranial pressure (ICP), 12 cats were studied with 31P-magnetic resonance spectroscopy. Six cats were subjected to intracranial hypertension by cisternal infusion of saline. Energy failure occurred at an average AVDO2 of 8.4 ± 3.2 vol% (± standard deviation) (range 4.7 to 14.7 vol%). The VO2 at the point of metabolic failure averaged 1.45 ± 0.6 vol% and extended over a narrower range (1.0 to 2.9 vol%). In an additional six cats, ICP was raised to the threshold of metabolic failure and hyperventilation was then instituted (pCO2 10 to 18 torr). Five of the six cats experienced a drop in VO2 with hyperventilation. In two of these animals, hyperventilation resulted in a VO2 of 1.1 vol% or less and in metabolic failure as evidenced by a fall in phosphocreatine. It is concluded that a VO2 of less than 2 vol% is correlated with brain ischemia and that the safety of hyperventilation in the setting of increased ICP can be monitored by the use of VO2.


2003 ◽  
Vol 98 (5) ◽  
pp. 1128-1132 ◽  
Author(s):  
Gabriel C. Tender ◽  
Scott Kutz ◽  
Deepak Awasthi ◽  
Peter Rigby

✓ The surgical treatment for cerebral spinal fluid (CSF) fistulas provides closure of the bone and dural defects and prevents the recurrence of brain herniation and CSF fistula. The two main approaches used are the transmastoid and middle fossa ones. The authors review the results of performing a modified middle fossa approach with a vascularized temporalis muscle flap to create a barrier between the repaired dural and bone defects. Fifteen consecutive cases of CSF fistulas treated at the authors' institution were retrospectively reviewed. All patients presented with otorrhea. Eleven patients had previously undergone ear surgery. A middle fossa approach was followed in all cases. The authors used a thin but watertight and vascularly preserved temporalis muscle flap that had been dissected from the medial side of the temporalis muscle and was laid intracranially on the floor of the middle fossa, between the repaired dura mater and petrous bone. The median follow-up period was 2.5 years. None of the patients experienced recurrence of otorrhea or meningitis. There was no complication related to the intracranial temporalis muscle flap (for example, seizures or increased intracranial pressure caused by muscle swelling). One patient developed hydrocephalus, which resolved after the placement of a ventriculoperitoneal shunt 2 months later. The thin, vascularized muscle flap created an excellent barrier against the recurrence of CSF fistulas and also avoided the risk of increased intracranial pressure caused by muscle swelling. This technique is particularly useful in refractory cases.


2010 ◽  
Vol 5 (1) ◽  
pp. 131-135 ◽  
Author(s):  
Matthew A. Adamo ◽  
Ian F. Pollack

Object Sagittal synostosis accounts for the most common form of craniosynostosis, occurring with an incidence of 1 in 2000–5000 live births. In most cases of single-suture, nonsyndromic sagittal synostosis, a single operation is all that is required to achieve a reasonable cosmetic result. However, there are a number of patients who may experience symptomatic postoperative calvarial growth restriction secondary to fibrosis of newly formed bone and pericranium that replace the surgically removed sagittal suture, or due to fusion of other previously open sutures leading to increased intracranial pressure, necessitating a second operation. Methods A retrospective review was conducted of all cases involving infants who had undergone an extended sagittal strip craniectomy with bilateral parietal wedge osteotomies at our institution between 1990 and 2006 for single-suture, nonsyndromic sagittal craniosynostosis. The frequency with which subsequent operations were required for cranial growth restriction was then defined. Results There were a total of 164 patients with single-suture nonsyndromic sagittal synostosis. Follow-up data were available for 143 of these patients. The average age at time of initial operation was 5.25 months, and the mean duration of follow-up was 43.85 months. There were 2 patients (1.5%) who required a second operation for symptomatic postoperative calvarial growth restriction. Conclusions Recurrence of synostosis with resultant increased intracranial pressure in cases of single-suture, nonsyndromic sagittal craniosynostosis is an uncommon event, but does occur sporadically and unpredictably. Therefore, we recommend routine neurosurgical follow up for at least 5 years, with regular ophthalmological examinations to assess for papilledema.


1979 ◽  
Vol 50 (6) ◽  
pp. 823-825 ◽  
Author(s):  
David W. Beck ◽  
Neal F. Kassell ◽  
Charles G. Drake

✓ The authors report a case of glomus jugulare tumor presenting with papilledema and visual loss. The tumor was extremely vascular with significant shunting of arterial blood into venous sinuses. There was no intracranial extension of tumor, and papilledema resolved after removal of the lesion.


2004 ◽  
Vol 101 (3) ◽  
pp. 536-540 ◽  
Author(s):  
Nobuhiro Mikuni ◽  
Takeshi Satow ◽  
Junya Taki ◽  
Namiko Nishida ◽  
Rei Enatsu ◽  
...  

✓ Difficulty swallowing due to damage of the vagus nerve is one of the most devastating complications of surgery in and around the medulla oblongata; therefore, intraoperative anatomical and functional evaluation of this nerve is crucial. The authors applied endotracheal tube surface electrodes to record electromyography (EMG) activity from vocal cords innervated by the vagus nerve. The vagal nucleus or rootlet was electrically stimulated during surgery and vocalis muscle EMG activities were displayed by auditory and visual signals. This technique was used successfully to identify the vagus motor nerve and evaluate its integrity during surgery. The advantages of this method compared with the use of needle electrodes include safe simple electrode placement and stable recording during surgery. In cases involving a pontine cavernoma pressing the nucleus or a jugular foramen tumor encircling the rootlet, this method would be particularly valuable. Additional studies with a larger number of patients are needed to estimate the significance of this method as a means of functional monitoring to predict clinical function.


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