Letters to Editor
20. 21. 22. 23.
ependymoma of the thoracic spine. Case illustration. J Neurosurg 1997;87:643. Lee CH, Moon KY, Chung CK, Kim HJ, Chang KH, Park SH, et al. Primary intradural extramedullary melanoma of the cervical spinal cord: Case report. Spine (Phila Pa 1976) 2010;35:E303‑7. Scotti G, Scialfa G, Colombo N, Landoni L. MR imaging of intradural extramedullary tumors of the cervical spine. J Comput Assist Tomogr 1985;9:1037‑41. Lin YH, Huang CI, Wong TT, Chen MH, Shiau CY, Wang LW, et al. Treatment of spinal cord ependymomas by surgery with or without postoperative radiotherapy. J Neurooncol 2005;71:205‑10. Lee SH, Chung CK, Kim CH, Yoon SH, Hyun SJ, Kim KJ, et al. Long‑term outcomes of surgical resection with or without adjuvant radiation therapy for treatment of spinal ependymoma: A retrospective multicenter study by the Korea Spinal Oncology Research Group. Neuro Oncol 2013;15:921‑9. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.125386
Received: 24‑10‑2013 Review completed: 18‑11‑2013 Accepted: 18‑12‑2013
generalization of the slowing. She underwent left frontotemporal craniotomy with an orbito‑zygomatic osteotomy (FTOZ) and a transsylvian approach using the carotid‑oculomotor triangle as the approach corridor. The hamartoma appeared nearly like normal brain tissue and was shaved off parallel to the floor of the III ventricle. Post‑operatively she developed transient occulomotor nerve paresis, right hemiparesis and transient diabetes insipidus, all of which resolved within a month. She is seizure free 1 year after surgery and is off all medications. Post‑operative MRI brain revealed total excision of the hamartoma with no residue [Figure 2a and b]. Case 2: The second case is about a 3½‑year‑old boy was brought with complaints of increasing penile and scrotal size since 8 months of age with spontaneous erections. He had early development of milestones (started walking at the age of 8 months) and was unusually aggressive. He was above the 97th percentile of height and weight and had acne all over the body. His testicular volume was 8cc bilaterally (pubertal transition volume = 4cc). Biochemical investigations confirmed central precocious puberty with testosterone levels in the adult male
Complete and curative excision of hypothalamic hamartomas via the orbito‑zygomatic approach Sir, Hypothalamic hamartomas (HH) are congenital non‑neoplastic lesions arising from the tuber cinereum, mammillary bodies or the floor of the third ventricle with reported incidence of 1‑2/million. They may present with intractable seizures (gelastic), mental retardation or precocious puberty.[1] We report two patients of HH managed by excision through a skull base approach. Case 1: This was a first case of a 3‑year‑old girl brought with the complaints of episodes of uncontrollable laughter since early childhood. She experienced multiple seizures daily despite being on two anti‑epileptic drugs. Developmental milestones and neuropsychological assessment were age‑appropriate. Magnetic resonance imaging (MRI) revealed a 1.75 cm × 1.5 cm sessile non‑enhancing hamartoma attached to the floor of the third ventricle, just ventral to the left mammillary body [Figure 1a‑d]. Her hormonal profile was in the normal range. Electroencephalography showed frequent random runs and bursts of theta and delta activity, predominantly in the central leads with intermittent 690
a
b
c
d
Figure 1: Case 1 - pre-operative magnetic resonance images. The arrows point to the lesion. The panels show the lesion appearance on (a) T2 axial, (b) post gadolinium-contrast sagittal, (c) fast fluid-attenuated inversion recovery coronal and (d) T1 coronal images
a
b
Figure 2: Case 1 - post-operative magnetic resonance images, contrast enhanced (a) sagittal and (b) coronal images showing complete excision of the lesion
Neurology India | Nov-Dec 2013 | Vol 61 | Issue 6
Letters to Editor
range (488 ng/dl); follicle stimulating hormone (FSH) and luteinizing hormone (LH) levels were 6.44 mIU/ml and 19.12 mIU/ml respectively. MRI brain imaging revealed a 4 mm pedunculated mass between the infundibular stalk and the mammillary bodies, suggestive of a hamartoma of the tuber cinereum [Figure 3a and b]. With leuprolide depot injections at a dosage of 11.25 mg every 3 months, his aggression was controlled over 2 years and testosterone and gonadotropin levels normalized; however, bone age continued to progress and there was no regression of his testicular volume. Hence, he underwent right FTOZ, temporo‑polar approach and complete excision of the hypothalamic hamartoma. He developed bilateral partial ptosis and diabetes insipidus, which resolved within the next 2 weeks. Immediate post‑operative hormone levels were ‑ serum testosterone
20. 21. 22. 23.
ependymoma of the thoracic spine. Case illustration. J Neurosurg 1997;87:643. Lee CH, Moon KY, Chung CK, Kim HJ, Chang KH, Park SH, et al. Primary intradural extramedullary melanoma of the cervical spinal cord: Case report. Spine (Phila Pa 1976) 2010;35:E303‑7. Scotti G, Scialfa G, Colombo N, Landoni L. MR imaging of intradural extramedullary tumors of the cervical spine. J Comput Assist Tomogr 1985;9:1037‑41. Lin YH, Huang CI, Wong TT, Chen MH, Shiau CY, Wang LW, et al. Treatment of spinal cord ependymomas by surgery with or without postoperative radiotherapy. J Neurooncol 2005;71:205‑10. Lee SH, Chung CK, Kim CH, Yoon SH, Hyun SJ, Kim KJ, et al. Long‑term outcomes of surgical resection with or without adjuvant radiation therapy for treatment of spinal ependymoma: A retrospective multicenter study by the Korea Spinal Oncology Research Group. Neuro Oncol 2013;15:921‑9. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.125386
Received: 24‑10‑2013 Review completed: 18‑11‑2013 Accepted: 18‑12‑2013
generalization of the slowing. She underwent left frontotemporal craniotomy with an orbito‑zygomatic osteotomy (FTOZ) and a transsylvian approach using the carotid‑oculomotor triangle as the approach corridor. The hamartoma appeared nearly like normal brain tissue and was shaved off parallel to the floor of the III ventricle. Post‑operatively she developed transient occulomotor nerve paresis, right hemiparesis and transient diabetes insipidus, all of which resolved within a month. She is seizure free 1 year after surgery and is off all medications. Post‑operative MRI brain revealed total excision of the hamartoma with no residue [Figure 2a and b]. Case 2: The second case is about a 3½‑year‑old boy was brought with complaints of increasing penile and scrotal size since 8 months of age with spontaneous erections. He had early development of milestones (started walking at the age of 8 months) and was unusually aggressive. He was above the 97th percentile of height and weight and had acne all over the body. His testicular volume was 8cc bilaterally (pubertal transition volume = 4cc). Biochemical investigations confirmed central precocious puberty with testosterone levels in the adult male
Complete and curative excision of hypothalamic hamartomas via the orbito‑zygomatic approach Sir, Hypothalamic hamartomas (HH) are congenital non‑neoplastic lesions arising from the tuber cinereum, mammillary bodies or the floor of the third ventricle with reported incidence of 1‑2/million. They may present with intractable seizures (gelastic), mental retardation or precocious puberty.[1] We report two patients of HH managed by excision through a skull base approach. Case 1: This was a first case of a 3‑year‑old girl brought with the complaints of episodes of uncontrollable laughter since early childhood. She experienced multiple seizures daily despite being on two anti‑epileptic drugs. Developmental milestones and neuropsychological assessment were age‑appropriate. Magnetic resonance imaging (MRI) revealed a 1.75 cm × 1.5 cm sessile non‑enhancing hamartoma attached to the floor of the third ventricle, just ventral to the left mammillary body [Figure 1a‑d]. Her hormonal profile was in the normal range. Electroencephalography showed frequent random runs and bursts of theta and delta activity, predominantly in the central leads with intermittent 690
a
b
c
d
Figure 1: Case 1 - pre-operative magnetic resonance images. The arrows point to the lesion. The panels show the lesion appearance on (a) T2 axial, (b) post gadolinium-contrast sagittal, (c) fast fluid-attenuated inversion recovery coronal and (d) T1 coronal images
a
b
Figure 2: Case 1 - post-operative magnetic resonance images, contrast enhanced (a) sagittal and (b) coronal images showing complete excision of the lesion
Neurology India | Nov-Dec 2013 | Vol 61 | Issue 6
Letters to Editor
range (488 ng/dl); follicle stimulating hormone (FSH) and luteinizing hormone (LH) levels were 6.44 mIU/ml and 19.12 mIU/ml respectively. MRI brain imaging revealed a 4 mm pedunculated mass between the infundibular stalk and the mammillary bodies, suggestive of a hamartoma of the tuber cinereum [Figure 3a and b]. With leuprolide depot injections at a dosage of 11.25 mg every 3 months, his aggression was controlled over 2 years and testosterone and gonadotropin levels normalized; however, bone age continued to progress and there was no regression of his testicular volume. Hence, he underwent right FTOZ, temporo‑polar approach and complete excision of the hypothalamic hamartoma. He developed bilateral partial ptosis and diabetes insipidus, which resolved within the next 2 weeks. Immediate post‑operative hormone levels were ‑ serum testosterone
![[Hypothalamic hamartomas]].](/assets/img/hold.png)
