|Intraneural hemangioma of the median nerve: A case report.|
|Jump to Full Text|
|PMID: 18294368 Owner: NLM Status: PubMed-not-MEDLINE|
|Hemangiomas of the median nerve are very rare and, so far, only ten cases of intraneural hemangioma of this nerve have been reported in the literature. We present a case of 14-year-old girl who had a soft tissue mass in the region of the left wrist with signs and symptoms of carpal tunnel syndrome. Total removal of the mass was achieved using microsurgical epineural and interfasicular dissection. The symptoms were relieved completely, after this procedure, without any neurologic deficit. On follow-up two years later, no recurrence was observed. Whenever a child or young adult patient presents with CTS the possibility of a hemangioma involving the median nerve should be kept in mind in the differential diagnosis.|
|Yunus Doğramaci; Aydiner Kalaci; Teoman Toni Sevinç; Ahmet Nedim Yanat|
Related Documents :
|3040418 - Uncompacted lamellae in three patients with poems syndrome.
20977708 - Oculomotor nerve palsy associated with bortezomib in a patient with multiple myeloma: a...
405868 - Aplasia of the optic nerve and disk.
6599408 - Hereditary motor and sensory neuropathies (hmsn). retinal involvement and erg alterations.
7726368 - Intraductal papillary mucinous neoplasms of the pancreas associated with so-called "muc...
22669878 - Atypical toxoplasmic retinochoroiditis.
|Type: Journal Article Date: 2008-02-22|
|Title: Journal of brachial plexus and peripheral nerve injury Volume: 3 ISSN: 1749-7221 ISO Abbreviation: J Brachial Plex Peripher Nerve Inj Publication Date: 2008|
|Created Date: 2008-04-01 Completed Date: 2010-06-28 Revised Date: 2010-09-21|
Medline Journal Info:
|Nlm Unique ID: 101265114 Medline TA: J Brachial Plex Peripher Nerve Inj Country: England|
|Languages: eng Pagination: 5 Citation Subset: -|
|Dept, of Orthopaedics and Traumatology, Mustafa Kemal University Faculty of Medicine, Hatay, Turkey. firstname.lastname@example.org|
|APA/MLA Format Download EndNote Download BibTex|
Journal ID (nlm-ta): J Brachial Plex Peripher Nerve Inj
Publisher: BioMed Central
Copyright © 2008 Doğramacı et al; licensee BioMed Central Ltd.
open-access: This is an Open Access article distributed under the terms of the Creative Commons Attribution License (), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received Day: 16 Month: 12 Year: 2007
Accepted Day: 22 Month: 2 Year: 2008
collection publication date: Year: 2008
Electronic publication date: Day: 22 Month: 2 Year: 2008
Volume: 3First Page: 5 Last Page: 5
Publisher Id: 1749-7221-3-5
PubMed Id: 18294368
|Intraneural hemangioma of the median nerve: A case report|
|Yunus Doğramacı1||Email: email@example.com|
|Aydıner Kalacı1||Email: firstname.lastname@example.org|
|Teoman Toni Sevinç1||Email: email@example.com|
|Ahmet Nedim Yanat1||Email: firstname.lastname@example.org|
1Dept. of Orthopaedics and Traumatology, Mustafa Kemal University Faculty of Medicine, Hatay, Turkey
The carpal tunnel syndrome (CTS) is the most common neuropathy due to compression seen in adults. There are very few cases in the literature referring to patients of paediatric age . Most of these young patients had a metabolic disorder mucopolysaccharidosis or mucolipidosis. Other unusual causes of CTS in children are fibrolipomas of the median nerve or intraneural perineuroma or haemangioma, haemophilia (secondary to local bleeding), musculotendinous malformation, Klippel-Trenaunay syndrome, Poland's syndrome, scleroderma, benign localised form of gigantism, intensive sports practice, and primary familial CTS . Very rarely Schwannomas of the median nerve can be mistakenly diagnosed and present as carpal tunnel syndrome [2-4]. Lipofibromatous hamartoma of the median nerve at the wrist was reported, and caused macrodactyly of the digits, and also resulted in symptoms of carpal tunnel syndrome [5-7]. Again epithelioid sarcoma of the median nerve may present with symptoms and signs of carpal tunnel syndrome . An isolated malignant peripheral nerve sheath tumor of mild type has also been reported to present with symptoms and signs of carpal tunnel syndrome .
Posttraumatic neuroma-in-continuity of the median nerve causing median nerve compression is rare . Damage to the median nerve after vascular graft placement as a result of an occult mass has been documented in a single case .
Intraneural hemangioma of the median nerve is a rare condition and only ten cases have been described in the literature [12-20]. Due to mechanical compression, carpal tunnel syndrome (CTS) is the main presenting feature [12-18]. Raynaud's phenomenon may be an associated complaint .
Here we present a case of intraneural hemangioma of the median nerve of a 14-year-old female removed surgically by combined interfasicular and epineural resection, no recurrence observed during the two years of postoperative follow-up period.
A 14-year-old female student presented to our outpatient clinic with painful swelling in the volar surface of the right wrist of 3 years duration; associated with tingling and numbness in the thumb, index, middle and radial half of the ring fingers, difficulty in writing long paragraphs. There was no history of trauma and relevant medical condition.
Physical examination revealed a tender, soft mass, 3 × 5 × 2 cm in dimension in the volar aspect of the right wrist. Tinel sign was positive.
Radiographic examination revealed no bony lesion. Ultrasonographic examination done to exclude any vascular lesion of the radial artery, revealed non pulsatile, cystic mass consistent with ganglion. An MR image obtained in another institution revealed a 3 × 2 × 1.5 cm ovoid rapidly enhancing mass in the volar surface of the right wrist region (Fig. 1).
EMG examination was planned for this patient, but the patient refused to cooperate during the test and the test was not completed successfully.
After preoperative assessment, the patient was admitted for surgical treatment under the diagnosis of volar ganglion causing CTS.
The operation was done under general anaesthesia, using a pneumatic tourniquet. Exploration revealed a yellowish brown soft tissue mass with areas of hemorrage and dimensions of approximately 4 × 3 × 1.5 cm, originating from the volar surface of the median nerve with intraneural extension and adhesions to the surrounding tissues (Fig. 2). The mass was removed totally by interfasicular and epineural microsurgical resection technique, without structurally damaging the nerve fibbers.
Histopathologic and microscopic evaluation revealed dilated and congested vascular structures in a fibrocollagenous stroma with areas of bleedings, consistent with histopathologic findings of hemangioma (Fig 3).
The symptoms were relieved in the first three weeks after the operation. On clinical and ultrasound examination, no recurrence was observed in the first two years following the operation.
Benign intraneural hemangioma originating from peripheral nerves is rare. Most patients present in with a painful, soft mass along the path of a nerve with signs and symptoms of nerve compression and entrapment.
A thorough search through the literature revealed ten cases of hemangioma of the median nerve [12-19]. In all the described cases CTS is the presenting feature and in one case Raynaud's phenomenon was an associated presenting feature.
The tumor may not be easily recognised until it becomes painful and it is rarely diagnosed before surgery. In the differential diagnosis, lipoma, lipofibroma, hamartoma and intraneuronal Schwannoma must be considered [20,21].
Ultrasonography may give useful information about the nerve's dynamic relation to the surrounding musculotendinous structures  and nerve conduction studies may reveal non specific features of compressive neuropathies . For appropriate planning of surgical therapy and preoperative diagnosis, MRI is essential and gives useful information regarding tumor location, size, extent and relationship of peripheral nerve.
Hemangioma shows a hyperintense signal on T1- and T2- weighted images with fat suppression sequences. Flow voids are usually apparent and feeding vessels may be visualized; these lesions are also noted to enhance after Gd-addition. On angiography an early and persistent tumoral blush is demonstrated .
Schwannoma is a slightly hypodense, solid tumor with no vascular contrast enhancement on CT. MRI shows intermediate signals on. T1-W, and T2-W imaging shows high signal intensity with some heterogenity . Lipomas exhibit signal characteristics consistent with those of normal adipose tissue: homogeneous hyperintensity on T1- and T2-weighted sequences . MR imaging findings of lipofibromatous hamartoma are pathognomonic which consist of serpiginous T1- and T2-weighted low-intensity structures containing and surrounded by fat (hyperintense on T1- and hypointense on T2-weighted fat suppression sequences), giving the lesion a spaghetti-like appearance on sagittal images, and a "coaxial cable-like" appearance on coronal images .
No certain protocol has been established to manage this difficult condition, however conservative treatment usually fails and surgery is the treatment of choice. When possible total resection of intraneural hemangiomas is curative, partial resection may relieve symptoms but recurrence may occur which may require en-bloc nerve resection and repair with nerve graft .
The longest period of follow-up without recurrence has been reported by Oztekin et al. . They reported a case of CTS due to a cavernous hemangioma of the median nerve, which was successfully removed by epineural resection, and no recurrence was observed over a 6 year follow-up period. Patel et al.  reported two cases of hemangioma of the median nerve which they treated by partial excision and resulted in recurrence in the third year, one of the recurred case managed by resection of median nerve and nerve grafting without recurrence, four years after surgery.
Chatillon et al.  reported the first case of using radiotherapy in the treatment of intraneural hemangioma. Preoperative embolization and postoperative radiotherapy combined with partial resection were beneficial in a case of intraneural hemangioma involving inferior trunk of brachial plexus and resulted in symptomatic relief and radiologic shrinkage in the size of the mass seen on serial follow-up MRI images, with a follow-up period of two years.
In our case, total resection of the hemangioma was achieved by combined epineural resection and interfasicular dissection with microsurgical resection technique, no neurologic complications observed postoperatively and no recurrence observed in the two year follow-up period.
The type of microsurgical dissection and resection should be decided at the time of surgery and careful preoperative planning using MRI, and if needed angiography, is essential for cystic lesions of the volar side of wrist. Excision of the affected nerve and grafting should be the last choice and should only be used in complicated cases and when there are frequent recurrences.
Whenever a child or young adult patient presents with CTS the possibility of a hemangioma involving the median nerve should be kept in mind in the differential diagnosis.
Written informed consent was obtained from the patient for publication of this Case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
|Van Meir N,De Smet L. Carpal tunnel syndrome in childrenActa Orthop Belg 2003;69:387–395. [pmid: 14648946]|
|Aslam N,Kerr G. Multiple Schwannomas of the median nerve: a case report and literature reviewHand Surg 2003;8:249–252. [pmid: 15002106] [doi: 10.1142/S0218810403001741]|
|Padua L,Pazzaglia C,Insola A,Aprile I,Caliandro P,Rampoldi M,Bertolini C,Tonali P. Schwannoma of the median nerve (even outside the wrist) may mimic carpal tunnel syndromeNeurol Sci 2006;26:430–434. [pmid: 16601936] [doi: 10.1007/s10072-006-0527-9]|
|Aydin MD,Kotan D,Keles M. Acute median nerve palsy due to hemorrhaged schwannoma: case ReportJBPPNI 2007;2:19.|
|Bagatur AE. Lipofibromatous hamartoma of the median nerveActa Orthop Traumatol Turc 2002;36:172–176. [pmid: 12510101]|
|Lorenzoni PJ,Lange MC,Kay CS,Silvado CE,Scola RH,Werneck LC. Fibrolipomatous hamartoma of the median nerve: case reportArq Neuropsiquiatr 2005;63:881–884. [pmid: 16258677]|
|Bains R,Kotwal A,Saeed W. Recurrent carpal tunnel syndrome in a child due to fibrolipomatous hamartoma of the median nerve successfully treated by limited excision and decompressionJ Plast Reconstr Aesthet Surg 2006;59:1394–1397. [pmid: 17113528] [doi: 10.1016/j.bjps.2006.01.016]|
|Harish S,Saifuddin A,Fajinmi M. Epithelioid sarcoma of the median nerve mimicking a peripheral nerve sheath tumourAustralas Radiol 2007;51:71–74. [pmid: 17217493] [doi: 10.1111/j.1440-1673.2006.01662.x]|
|Ochsner F,Baumann RP,Kuntzer T. Carpal tunnel syndrome with an unusual cause: a malignant nerve sheath tumor of the median nerveRev Neurol (Paris) 2001;157:1547–1549. [pmid: 11924454]|
|Martinelli P,Poppi M,Gaist G,Padovani R,Pozzati E. Posttraumatic neuroma of the median nerve: a cause of carpal tunnel syndromeEur Neurol 1985;24:13–15. [pmid: 3967671] [doi: 10.1159/000115755]|
|Goldstein LJ,Helfend LK,Kordestani RK. Postoperative edema after vascular access causing nerve compression secondary to the presence of a perineuronal lipoma: case reportNeurosurgery 2002;50:412–414. [pmid: 11844280] [doi: 10.1097/00006123-200202000-00035]|
|Coessens B,De Mey A,Lacotte B,Vandenbroeck D. Carpal tunnel syndrome due to an haemangioma of the median nerve in a 12-year-old childAnn Chir Main Memb Super 1991;10:255–257. [pmid: 1718362] [doi: 10.1016/S0753-9053(05)80292-3]|
|Kojima T,Ide Y,Marumo E,Ishikawa E,Yamashita H. Haemangioma of median nerve causing carpal tunnel syndromeHand 1976;8:62–65. [pmid: 1261900] [doi: 10.1016/0072-968X(76)90063-2]|
|Patel CB,Tsai TM,Kleinert HE. Hemangioma of the median nerve: a report of two casesJ Hand Surg [Am] 1986;11:76–79. [pmid: 3944449]|
|Peled I,Iosipovich Z,Rousso M,Wexler MR. Hemangioma of the median nerveJ Hand Surg [Am] 1980;5:363–365. [pmid: 7419879]|
|Prosser AJ,Burke FD. Haemangioma of the median nerve associated with Raynaud's phenomenonJ Hand Surg [Br] 1987;12:227–228. [pmid: 3624983]|
|Wood MB. Intraneural hemangioma: report of a casePlast Reconstr Surg 1980;65:74–76. [pmid: 7350583]|
|Oztekin HH,Karaarslan AA. Carpal tunnel syndrome due to a cavernous hemangioma of the median nerveActa Orthop Traumatol Turc 2003;37:170–172. [pmid: 12704258]|
|Petrovici V. Cavernous hemangioma of the palm with symptoms resembling carpal tunnel syndromeZ Plast Chir 1980;4:40–47. [pmid: 6250298]|
|Chatillon CE,Guiot MC,Jacques L. Lipomatous, vascular, and chondromatous benign tumors of the peripheral nerves: Representative cases and review of the literatureNeurosurg Focus 2007;22:E18. [pmid: 17613209] [doi: 10.3171/foc.2007.22.6.19]|
|Louis D. Peripheral nerve tumors of the upper extremityHand Clin 1987;3:311–318. [pmid: 3108268]|
|Kuo YL,Yao WJ,Chiu HY. Role of sonography in the preoperative assessment of neurilemmomaJ Clin Ultrasound 2005;33:87–89. [pmid: 15674832] [doi: 10.1002/jcu.20085]|
|MacDonell RAL,Schwartz MS,Swash M. Carpal Tunnel Syndrome: Which finger should be tested? An analysis sensory conduction in digital branches of the median nerveMuscle Nerve 1990;13:601–606. [pmid: 2388658] [doi: 10.1002/mus.880130707]|
|Hems TE,Burge PD,Wilson DJ. The role of magnetic resonance imaging in the management of peripheral nerve tumoursJ Hand Surg 1997;22:57–60. [pmid: 9061527] [doi: 10.1016/S0363-5023(05)80180-7]|
|Chiao HC,Marks KE,Bauer TW,Pflanze W. Intraneural lipoma of the sciatic nerveClin Orthop Relat Res 1987;221:267–271. [pmid: 3608310]|
|Marom EM,Helms CA. Fibrolipomatous hamartoma: pathognomonic on MR imagingSkeletal Radiol 1999;28:260–264. [pmid: 10424331] [doi: 10.1007/s002560050512]|
Previous Document: Cost-utility of an 8-month aquatic training for women with fibromyalgia: a randomized controlled tri...
Next Document: ReAlignerV: web-based genomic alignment tool with high specificity and robustness estimated by speci...