Torsion of a lumbar nerve root schwannoma

We report a very rare case of a lumbar nerve root schwannoma presenting with torsion and infarction. The patient was a 16-year-old male presenting with severe low back pain and urinary retention following an aggressive game of hockey. Subsequent MRI of the lumbar spine revealed a nonenhancing lumbar intradural lesion at the level of L3. The patient was taken to the operating room where he underwent a bilateral L2 and L3 laminectomy and gross total resection of an intradural nerve root tumor, which appeared to have undergone torsion and infarction. Subsequent histopathological examination of the surgical specimen verified the diagnosis of infarcted schwannoma. This is a unique case of lumbar nerve root schwannoma, with atypical MRI findings, presenting with infarction due to torsion of the involved nerve root.     

Tako-Tsubo Syndrome in a 12-Year-Old Girl: Exhausted Heart, Not Broken Heart

We report the case of a 12-year-old girl with Tako-tsubo cardiomyopathy. She was successfully treated in our Pediatric Intensive Care Unit for acute left ventricular failure, which occurred after a brief submersion in sea water. At 2-month follow-up she showed an almost complete recovery of cardiac function. We consider Tako-tsubo syndrome, which is rarely reported in the pediatric population, to be the most likely diagnosis.     

Isolated hypoglossal nerve palsy in a child

We report an 11-year-old boy who had isolated hypoglossal nerve palsy one week after symptoms and signs of urticarial lesions. Neuroradiological examinations and other investigations for etiology of hypoglossal nerve palsy and urticaria were normal. We suggest that all patients with hypoglossal palsy must be carefully evaluated for atypical findings and etiologies.     

Isolated hypoglossal nerve palsy in a 14-year-old girl.

Isolated hypoglossal nerve palsy is rare, but occasionally it appears as the initial or solitary sign of an intracranial or extracranial space-occupying lesion or a vascular abnormality of the internal carotid artery. We present a 14-year-old girl who, following an upper respiratory tract infection, presented with isolated right hypoglossal nerve palsy. Anti-streptolysin O titre was increased to >1280 suggesting a preceding streptococcal infection. Magnetic resonance imaging of the brain did not show any intracranial or extracranial abnormality. She had a partial improvement at 3 months. This case emphasizes the value of recognizing the existence of benign self-limiting, post-infectious causes of isolated hypoglossal nerve palsies in children.     

Expanded endoscopic endonasal resection of an olfactory schwannoma

Olfactory schwannomas are rare tumors of the anterior skull base that are possibly derived from ectopic Schwann cells, perivascular neural tissue, or sensory nerves of the meninges. The authors report the case of a 14-year-old boy with an olfactory schwannoma that extended inferiorly through the cranial base and superiorly into the frontal lobe. Because of the growth characteristics of the tumor and the significant overlying frontal lobe edema, the lesion was approached via an endonasal endoscopic route, as a strategy to minimize brain retraction. This tumor was characterized radiographically as contrast-enhancing with cystic areas and erosion into bone. The tumor showed immunoreactivity for S100 protein and leukocyte antigen 7 (CD57) but not epithelial membrane antigen, supporting the diagnosis of olfactory schwannoma. A gross-total resection was achieved. This approach represents a novel application of endoscopic endonasal surgery to the pediatric neurosurgical context, as well as a favorable outcome in an extremely unusual tumor type, that should be applicable to other appropriately selected pediatric brain tumors.     

Pediatric orbital schwannoma originating from the oculomotor nerve

Intraorbital schwannoma is a rare tumor that constitutes approximately 1%-8% of all orbital tumors. The authors report a case of orbital schwannoma in a 5-year-old boy who was admitted to their institute with exophthalmos and ptosis of the right eye. Computed tomography scanning and MR imaging revealed a retroocular mass in the right orbit. The tumor was successfully removed via a transcranial approach. The pathological diagnosis was schwannoma that appeared to originate from the superior branch of the oculomotor nerve. Despite the rarity of these intraorbital extraocular tumors in children, schwannomas should be differentiated from other intraorbital tumors.     

Glandular schwannoma in a 17-month-old child

Glandular schwannoma is an infrequent tumor, especially in the pediatric age group. We report a case in which the tumor developed in the retroperitoneum of a 17-month-old girl without stigmata of Von Recklinghausen's disease (VRD). She presented a local recurrence and bone and pulmonary metastases at 11, 23, and 30 months, respectively, after tumor resection. The pathologic findings in the original tumor did not permit us to predict its potential to metastasize. The ultrastructural characteristics of both glandular and schwannian elements are described. The rarity of this neoplasia in children and the absence of histological parameters of aggressiveness are emphasized.     

Glossopharyngeal schwannoma in childhood

Glossopharyngeal (that is, cranial nerve IX) schwannomas are extremely rare nerve sheath tumors that frequently mimic the more common vestibular schwannoma in their clinical as well as radiographic presentation. Although rare in adults, this tumor has not been reported in a child. The authors report the case of a 10-year-old boy who presented with several months of unilateral hearing loss. He was found to have a large right cerebellopontine angle tumor. Given the boy's primary complaint of hearing loss and the appearance of the lesion on imaging, the tumor was initially believed to be a schwannoma of the vestibular nerve. It was found intraoperatively, however, to originate from the glossopharyngeal nerve. To the authors' knowledge, this is the first reported case of a glossopharyngeal schwannoma in a child.     

Optic glioma with characteristic bilateral optic atrophy in a 3-year-old girl

We report a case of optic glioma with bilateral optic atrophy. A 3-year-old girl presented with vomiting and left hemiparesis. She had hypothalamic dysfunction, right ptosis, right monocular nystagmus, left facial palsy, left hemiparesis, and left pes adductus. Neuroimaging studies showed obstructive hydrocephalus with a large suprasellar calcified tumor with a ring-like enhancement mimicking craniopharyngioma. Visual-evoked potentials showed delayed latency of N75 in the right occipital lead. The tumor, a pilocytic astrocytoma in the right optic tract and chiasma, was partially removed via a right frontotemporal craniotomy. The right optic nerve had shrunk to half the normal diameter and became twisted downwardly. Intracranial pressure (ICP) increased to 40 cm H2O. The fundus had bilateral optic atrophy without disc swelling. To our knowledge, this is the first report of a lamina/dot sign of the optic disc in a small child with a brain tumor and a normal neuroretinal fiber layer. These ocular findings may result from possible interruption of the axonal flow caused by the tumor and not increased ICP.     

Interpeduncular fossa lipoma: a novel cause of oculomotor nerve palsy in childhood

Oculomotor nerve palsy is a rare finding in children and, when reported, is most frequently either congenital or acquired from postnatal trauma, infection, aneurysm, or migraine. Intracranial lipomas also represent an uncommon finding in children, and although their development is not completely understood, they are now thought to be congenital in nature. Here, we describe the case of a 23-month-old boy presenting to the emergency department with left-sided, complete, pupil-involving oculomotor nerve palsy. On magnetic resonance imaging, he was found to have an intracranial lipoma of the left interpeduncular fossa. The patient had gradual and spontaneous improvement of symptoms, with complete resolution reported at the 4-month follow-up visit. However, a second magnetic resonance image at 6 months revealed that the lipoma did not change in size. To our knowledge, intracranial lipomas have been previously reported as a possible cause of partial oculomotor nerve palsy in only one adult and have never been reported in a child. In addition, we did not find any reports of intracranial lipomas as a cause of complete, pupil-involving oculomotor palsy, although they are known to cause other cranial nerve pathology. We conclude that intracranial lipomas, although rare, should be considered in the differential diagnosis for oculomotor nerve palsy in children. Further investigation is needed to determine the true incidence of this association.     

Schwannoma of the tentorium cerebelli in a child. Case report

Intracranial schwannomas not arising from a cranial nerve are very rare. Schwannomas of the dura are even rarer; in the literature, we found only two cases: a schwannoma of the falx and one of the torcula. We report a third case of a 9-year-old girl with a schwannoma of the tentorium cerebelli. The different theories concerning the origin of this tumor in this particular location are discussed.     

Juxta-adrenal malignant schwannoma

A case of malignant schwannoma occurring in an 11-year-old boy is described. To our knowledge, this is the third case of malignant juxta-adrenal schwannoma reported in the literature. It was misdiagnosed at the onset of the disease as a post-traumatic renal hematoma. Angiography revealed that the tumor derived its blood supply from collateral vessels of the aorta, splenic, and left gastric arteries. An analysis of the diagnosis, clinical course, and prognostic factors of this rare tumor is performed.     

Facial palsy associated with mumps parotitis.

Peripheral facial nerve paralysis is relatively common in the pediatric age group. However, facial palsy rarely has been documented in patients with mumps parotitis. This case report describes the findings of a 3-year-old Japanese boy who developed facial palsy immediately after mumps parotitis. This work calls attention to a possible association between mumps parotitis and facial palsy.     

A rare cause of facial nerve palsy in children: Hyperostosis corticalis generalisata (Van Buchem disease). Three new pediatric cases and a literature review

Differential diagnosis of facial nerve palsy in children is extensive. We report on three pediatric cases presenting with facial nerve palsy caused by hyperostosis corticalis generalisata (Van Buchem disease). This autosomal recessive disease is characterized by progressive bone overgrowth, with narrowing of the neuroforamina in the skull causing cranial neuropathies. These three new cases of Van Buchem disease are of interest because of exceptionally early presentation of symptoms. Furthermore, this is the first report describing bilateral papilledema in a child with Van Buchem disease. Head computerized tomography (CT) scan revealed thickened calvarium, skull base and mandible in all three children, with narrowed facial nerve canals. Bone mineral density (BMD) was markedly increased at all measured points and biochemical markers of bone formation were significantly elevated. Diagnosis of Van Buchem disease was genetically confirmed. The cases are unique in that these are the first well-documented pediatric cases of Van Buchem disease.     

Retroclival epidural hematoma in a child affected by whiplash cervical injury: a typical case of a rare condition

Traumatic posterior fossa epidural hematomas are uncommon lesions. Among these lesions, retroclival epidural hematomas (REDH) are particularly rare conditions that usually occur in the pediatric population due to predisposing anatomical features in this patient group. We describe a typical case of traumatic REDH from the mechanism of trauma to outcome. This 8-year-old girl was involved in a motor vehicle accident leading to whiplash cervical injury and cranial nerve palsy. Any children involved in a severe motor vehicle accident with such a sequence of events should raise suspicion for prompt diagnosis.     

Clinical manifestation of Lyme borreliosis in childhood

We report on 4 children with different clinical manifestations of Lyme-Borreliosis. One patient presented with a stage 2 typical aseptic meningitis and 2 others with symptoms of Sch?nlein-Henoch purpura and rheumatic disease respectively. A further case had bilateral palsy of abducens nerve and unilateral palsy of trochlearis nerve which are described for the first time in Lyme-Borreliosis. Diagnosis was established by detection of specific antibodies to Borrelia in all patients. Differential diagnosis of these symptoms should include Lyme-Borreliosis.     

Recurrent pneumonia revealing a bronchial carcinoid tumor: report of two cases]

Carcinoid tumors are the most common endobronchial tumor in the pediatric population, and represent a rare cause of airway obstruction. The authors report two cases of boys aged 10 and 11 years old, who presented with a 12-month history of recurrent pneumonia. Bronchial endoscopy showed an endobronchial tumor. Chest CT-scan identified local extension and lung-associated lesions; octreoscan was performed to detect distant metastases. Histopathological study concluded in typical carcinoid tumor. The outcome after surgical conservative resection is uneventful with a follow-up of 7 and 26 months. Bronchial tumors must be considered in children with recurrent pneumonia or persistant respiratory symptoms, and require CT scan and bronchial endoscopy for their diagnosis.     

A common symptom of an uncommon disease

Cancer of the colon is the second most common visceral cancer in the United States (lung cancer is the first). It is usually diagnosed in patients older than 40 years, with a peak incidence at 70 years of age. Rarely, are cases seen in the pediatric population. In this study, we report a case of a 13-year-old girl with an 11-month history of intermittent abdominal pain whose diagnosis was delayed due to vague symptoms and a low index of suspicion for this condition.     

Penile malignant peripheral nerve sheath tumour (schwannoma) in a three-year-old child without evidence of neurofibromatosis.

Malignant schwannoma (malignant peripheral nerve sheath tumour, MPNST) is a rare high-grade tumour arising from peripheral nerves. We report the case of a 3-year-old male who presented with a non-tender lesion on the dorsum of his penis. The lesion was excised and a formal circumcision performed. Histology of the lesion revealed a spindle cell tumour. Immunohistochemistry showed the tumour cells to be strongly positive for S100 and Vimentin. A diagnosis of intermediate grade malignant peripheral nerve sheath tumour was made. Malignant schwannoma is rare in children and is previously unreported in the penis in the paediatric age group without evidence of neurofibromatosis.     

Temporal bone rhabdomyosarcoma presenting as acute peripheral facial nerve paralysis

Facial palsy is not an uncommon presentation to an emergency department. Whereas most patients will ultimately receive a diagnosis of Bell palsy (idiopathic peripheral seventh cranial nerve palsy), a subset will have an identifiable cause for their facial paralysis. Children are more likely to have an identifiable cause than are adults. We present a case in which a child presented with acute peripheral facial nerve palsy and was found to have temporal bone rhabdomyosarcoma. The key clinical finding was the presence of both 7th and 12th cranial nerve palsy.