improved and he was discharged from the hospital 6. Huang JW, Fang CT, Hung KY, Hsueh PR, Chang Sc, Tsai TJ. 2 weeks later on oral antibiotics. After 1 year he has a Necrotising fasciitis caused by Serratia marcescens in two patients receiving corticosteroid therapy. J Formos Med Assoc residual partial right-sided facial palsy maintaining 1999;98:851-4. adequate closure of his right eye showing no signs of 7. Urschel JD. Necrotising soft tissue infections. Postgrad Med J (Fig. 2). His corrected right visual 1999;75:645-9. acuity is 6/18. 8. Krohel GB, Krass HR, Winnick J. Orbital abscess: presentation, diagnosis, therapy and sequelae. 1982;89:492-8. Comment Aldrin Khan [5!'l Necrotising fasciitis is a severe and potentially fatal soft Nisar Ahmad tissue infection. The skin is initially pale red, tensely Essex County Hospital swollen and accompanied by severe pain, as necrosis Lexden Road spreads in the tissue planes. All authors recommend Colchester C03 3NB, UK prompt diagnosis along with early surgical debridement of necrotic tissue and appropriate parenteral antibiotics. Kronish and McLeishl even recommend hyperbaric Sir, oxygen therapy. Alveolar orbital rhabdomyosarcoma in a patient with Although the diagnosis was made on presentation, no neurofibromatosis type 1 surgical debridement or skin grafting was performed at Patients with neurofibromatosis type 1 (NFl) have an any time, which is usually associated with a poor increased risk of developing malignancy, usually as prognosis. The avascularity of the fascial planes and transformation of a neurofibroma or other tissue of necrotic tissue do not allow penetration of the antibiotics neural crest origin.l Soft tissue sarcomas, including and, therefore, logically these areas should be debrided. rhabdomyosarcomas, also occur, usually in the However, the periorbital area is highly vascular and urogenital system.1.2 We highlight rhabdomyosarcoma in maintains adequate perfusion up to the lid margins. One the differential diagnosis of an mass in patients may suggest that due to limited necrosis and very early with NF1. presentation with prompt diagnosis and institution of appropriate high-dose antibiotics, adequate tissue levels Case report were achieved early to eventually bring the infection A 6-year-old girl presented with a 3 month history of a under control. Arterial arcades of the that lie painless lump in the upper left eyelid. She had NFl, of deep to the pretarsal orbicularis muscle remained which there was a strong family history. Visual acuity unaffected, resulting in adequate perfusion of the lid was 20/20 bilaterally. A mobile, bilobed, rubbery lump margins, and the orbicularis muscle remained intact. was palpated in the medial left upper lid. She had , This patient was closely monitored throughout his congested forniceal conjunctival vessels, and a Lisch acute period of illness and surgical debridement would nodule on her right . Ten days later the mass had have been performed if his condition continued to increased markedly in size (Fig. 1). An MRI scan showed deteriorate. This report is not intended to dispute the fact a large non-enhancing pre-septal mass extending that 'early surgical debridement is the mainstay of superiorly to the orbital margin, indistinguishable from treatment', but merely to outline the importance of early overlying soft tissues, with lateral extension into the diagnosis of this condition with institution of appropriate lacrimal region. antibiotic treatment. Clindamycin has been shown to be Subtotal excision was performed and histological more effective than penicillin G against group A examination showed an alveolar-type streptococcus. This was our first choice in the broad­ rhabdomyosarcoma, confirmed by the regional spectrum cover. Clindamycin possibly controlled the paediatric pathology service and United Kingdom infection initially, thus avoiding muscle involvement/ surgical debridement.

References

1. Kronish JW, McLeish WM. Eyelid necrosis and periorbital necrotising fasciitis. Ophthalmology 1991;98:92-8. 2. Knudson KJ, Gigantelli JW. Necrotising fasciitis of the eyelids and . Arch Ophthalmol 1998;116:1548. 3. Marshall DH, Jordan DR, Gilberg SM, Harvey J, Arthurs BP, Nerad JA. Periocular necrotising fasciitis: a review of five cases. Ophthalmology 1997;104:1857-62. 4. Jordan DR, Mawn L, Marshall DH. Necrotising fasciitis caused by group A streptococcus infection after laser blepharoplasty. Am J Ophthalmol 1998;125:265-6. 5. Ray AM, Bressler K, Davis RE, Gallo JF, Patete ML. Cervicofacial necrotising fasciitis: a devastating complication of blepharoplasty. Arch Otolaryngol Head Neck Surg 1997;123:633-6. Fig. 1. The appearance before treatment.

812 alveolar rhabdomyosarcoma).3-s These molecular studies require fresh tissue rapidly processed, best done in the regional centre.

References

1. McKeen EA, Bodurtha J, Meadows AT, Douglass EC, Mulvihill JJ. Rhabdomyosarcoma complicating multiple neurofibomatosis. J Pediatr 1978;93:992-3. 2. Mosso ML, Castello M, Bellani FF, et al. Neurofibromatosis and malignant childhood cancers: a survey in Italy, 1970-83. Tumori 1987;73:209-12. 3. Kullendorf CM, Donner M, Mertens F, Mandahl N. Chromosomal abnormalities in a consecutive series of childhood rhabdomyosarcoma. Med Pediatr Oneal Fig. 2. The appearance after final treatment. 1998;30:156-9. 4. Biegel JA, Nycum LM, Valentine V, Barr FC, Shapiro DN. Detection of the t(2;13)(q35;q14) and PAX3-FKHR fusion in alveolar rhabdomyosarcoma by fluorescence in situ Childhood Cancer Study Group (UKCCSG) paediatric hybridization. Cenes Chromosomes Cancer 1995;12:186-92. pathology panel. There was no evidence of systemic 5. Reichmuth C, et al. The diagnostic potential of the spread. chromosome translocation (t2;13) in rhabdomyosarcoma: a Treatment comprised sequenced chemotherapy with PCR study of fresh-frozen and paraffin-embedded tumour samples. J Pathol 1996;180:50-7. vincristine, actinomycin D and ifosfamide (IV A) (SlOP / UKCCSG MMT 95 protocol, strategy 953, arm A). She Simon J Glover 1 responded until a local recurrence 8 months later, treated Teresa Parkin2 Martin Mott3 with MMT 5 relapse protocol (multiagent chemotherapy Anthony G. Quinn 1 including carboplatin, epirubicin, etoposide) and 1West of England Eye Unit radiotherapy. She has a residual ptosis (Fig. 2) and upper Royal Devon and Exeter Hospital conjunctival fornix redness due to shortening, with no Exeter, Devon, UK 2 Paediatrict Department mass palpable, marked mucous keratopathy and Royal Devon and Exeter Hospital retained visual acuity of 20/30. Exeter, Devon, UK 3Paediatric Oncology Unit Comment Institute of Child Health Royal Hospital for Sick Children We believe this to be the first report of alveolar-type Bristol, UK orbital rhabdomyosarcoma associated with NFL There Mr A.G. Quinn � are 3 previously reported cases of orbital West of England Eye Unit rhabdomyosarcomas in series of NFl patients, but they Royal Devon and Exeter Hospital were of embryonal or pleomorphic histological type or Barrack Road the histology was not stated, and neither series discussed Exeter, Devon, UK the ophthalmic presentation.1,2 Tel: +44 (0)1392 4-6004 Fax: +44 (0)1392 406022. Orbital rhabdomyosarcomas constitute 10% of e-mail: Anthony. Quin [email protected] childhood rhabdomyosarcomas. As with all paediatric malignancies, treatment is according to national! international protocols supervised by regional paediatric Sir, oncologists. This girl was treated by the UKCCSG Ankyloblepharon filiforme adnatum version of the malignant mesenchymal tumour of Ankyloblepharon is a full-thickness fusion of the eyelids childhood procotol- MMT 95 study. at the lateral canthus (external ankyloblepharon) or the Chemotherapy is the primary treatment but medial canthus (internal ankyloblepharon) producing radiotherapy is indicated if there is residual tumour after shortening of the palpebral fissure. Ankyloblepharon chemotherapy. Long-term sequelae of radiotherapy filiforme adnatum is a benign congenital anomaly in include visual loss, dry eye, facial asymmetry, and which single or multiple strands of fine connective tissue endocrine/growth problems if the hypothalamus or join the upper and lower eyelids. pituitary is involved in the field of radiation. Prognosis and treatment of alveolar Case re ort rhabdomyosarcoma differ substantially from that of p embryonal rhabdomyosarcoma; it is therefore imperative A 4480 g, white male boy was born at 40 weeks gestation that cases are discussed before surgery with the regional to a 21-year-old third gravida. The pregnancy was paediatric oncology team. Histological differentiation normal and the mother denied any drugs, alcohol or between the two types on formalin-fixed tissues is often smoking. The previous tWo siblings, aged 4 and 2 years, difficult so increasing reliance is placed on finding 2;13 both males, were healthy. There was no family history of chromosome translocation (or variants characteristic of congenital anomalies or consanguinity. At birth the baby

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