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SH Kim and H Kim

Finally, we diagnosed the patient with MP hypersensitivity, and recommended avoidance of this drug. During the next 2 years, he did not experience recurrence of anaphylaxis.

Possible cross-reactivity among hydrocortisone, MP, and prednisone has been suggested.10 The present patient had no crossreaction to the other steroids, which was confirmed on oral provocation and allergic skin test.

Discussion

Conclusion

Although corticosteroids are widely used to treat immunological disorders, sensitization to these compounds can occur, ranging in frequency from 0.5% to 5%. Delayed allergic reactions to topical corticosteroids are frequently observed (2.9%). In contrast, immediate hypersensitivity reactions to systemic corticosteroids are very rare (0.3%).1,2 Signs and symptoms of immediate hypersensitivity to glucocorticoids include various manifestations, such as pruritus, rash, angioedema, sneezing, dyspnea, throat tightness, wheezing, nausea, vomiting, hypotension, and anaphylactic shock.1–3 Immediate reactions are generally found following use of i.v. corticosteroid, but they have also been reported following respiratory (nasal or oral inhalation), i.m., intraarticular, and oral use.1–4 In the present case, anaphylaxis was induced by oral MP. Allergic skin test and oral provocation test were positive. This suggests that the pathogenic mechanism is an IgE-medicated reaction. The allergenic moiety in steroids that is responsible for immediate reactions has not been determined. It could be part of the native molecule or a metabolite that acts as a hapten and binds to serum proteins, creating an allergenic complex.3 Data on risk for development of hypersensitivity reactions to corticosteroids are still lacking. Some reports, however, have suggested that renal transplant or asthmatic patients, especially those who are aspirin sensitive, may be at higher risk,5–7 but this could be due to the frequent use of these medications and not to the disease itself.8 The present patient had a history of idiopathic generalized urticaria and had been occasionally exposed to low-dose oral MP. We suggest that the sensitization route in the present case may have been through oral treatment, and even a low dose of oral MP could induce an immediate allergic reaction in exposed patients. Some papers noted that the incidence of hydrocortisone and MP allergy seemed higher than allergy to other glucocorticoids.8,9 This could be related to the frequency with which it is used.

We report a unique case of MP-induced anaphylaxis, in which the patient had a positive oral challenge test result. Clinicians should be aware of this type of hypersensitivity reaction and take it into consideration in the differential diagnosis of patients who receive systemic corticosteroid.

References 1 Baeck M, Marot L, Nicolas JF, Pilette C, Tennstedt D, Goossens A. Allergic hypersensitivity to topical and systemic corticosteroids: A review. Allergy 2009; 64: 978–94. 2 Matura M, Goossens A, Matura M. Contact allergy to corticosteroids. Allergy 2000; 55: 698–704. 3 Rachid R, Leslie D, Schneider L, Twarog F. Hypersensitivity to systemic corticosteroids: An infrequent but potentially lifethreatening condition. J. Allergy Clin. Immunol. 2011; 127: 524–8. 4 Kilpiö K, Hannuksela M. Corticosteroid allergy in asthma. Allergy 2003; 58: 1131–5. 5 Saito R, Moroi S, Okuno H, Ogawa O. Anaphylaxis following administration of intravenous methylprednisolone sodium succinate in a renal transplant recipient. Int. J. Urol. 2004; 11: 171–4. 6 Dajani BM, Sliman NA, Shubair KS, Hamzeh YS. Bronchospasm caused by intravenous hydrocortisone sodium succinate (SoluCortef) in aspirin-sensitive asthmatics. J. Allergy Clin. Immunol. 1981; 68: 201–4. 7 Partridge MR, Gibson GJ. Adverse bronchial reactions to intravenous hydrocortisone in two aspirin-sensitive asthmatic patients. Br. Med. J. 1978; 1: 1521–2. 8 Kamm GL, Hagmeyer KO. Allergic-type reactions to corticosteroids. Ann. Pharmacother. 1999; 33: 451–60. 9 Ventura MT, Calogiuri GF, Matino MG et al. Alternative glucocorticoids for use in cases of adverse reaction to systemic glucocorticoids: A study on 10 patients. Br. J. Dermatol. 2003; 148: 139–41. 10 Calogiuri GF, Muratore L, Nettis E, Ventura MT, Ferrannini A, Tursi A. Anaphylaxis to hydrocortisone hemisuccinate with crosssensitivity to related compounds in a paediatric patient. Br. J. Dermatol. 2004; 151: 707–8.

Parotid lymphangioma associated with facial nerve paralysis Mitsuyoshi Imaizumi, Akiko Tani, Hiroshi Ogawa and Koichi Omori Department of Otolaryngology, School of Medicine, Fukushima Medical University, Fukushima, Japan Abstract

Parotid lymphangioma is a relatively rare disease that is usually detected in infancy or early childhood, and which has typical features. Clinical reports of facial nerve paralysis caused by lymphangioma, however, are very rare. Usually, facial nerve paralysis in a child suggests malignancy. Here we report a very rare case of parotid lymphangioma associated

Correspondence: Koichi Omori, MD PhD, Department of Otolaryngology, School of Medicine, Fukushima Medical University, 1 Hikarigaoka, Fukushima 960-1295, Japan. Email: [email protected] Received 2 September 2013; revised 20 January 2014; accepted 7 February 2014. doi: 10.1111/ped.12340

© 2014 Japan Pediatric Society

Lymphangioma with facial nerve paralysis

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with facial nerve paralysis. A 7-year-old boy was admitted to hospital with a rapidly enlarging mass in the left parotid region. Left peripheral-type facial nerve paralysis was also noted. Computed tomography and magnetic resonance imaging also revealed multiple cystic lesions. Open biopsy was undertaken in order to investigate the cause of the facial nerve paralysis. The histopathological findings of the excised tumor were consistent with lymphangioma. Prednisone (40 mg/day) was given in a tapering dose schedule. Facial nerve paralysis was completely cured 1 month after treatment. There has been no recurrent facial nerve paralysis for eight years. Key words diagnosis, facial nerve paralysis, lymphangioma, malignancy, parotid gland.

Lymphangiomas are localized malformations in the development of the lymphatic system that most frequently affect the head and neck.1 Parotid lymphangioma is a relatively rare disease. It is usually detected in infancy or early childhood,2 and has typical features, thus it is not difficult to reach a diagnosis. The clinical presentation of facial nerve paralysis caused by lymphangioma, however, is very rare. Usually, facial nerve paralysis in a child suggests malignancy.3 Here we report a very rare case of left parotid lymphangioma associated with facial nerve paralysis.

Case report A 7-year-old boy visited hospital with a mass in the left parotid region that had been recognizable from birth. Over the previous year, there had been a history of enlargement of this region. Four days before the current admission, the mass markedly and rapidly increased in size and became painful, and the child presented with dyspnea and difficulty with ingestion. On physical examination the patient had a tense, tender, and non-erythematous mass (10 × 8 cm) in the left parotid region (Fig. 1a). Left peripheraltype facial nerve paralysis was also noted. Right stapedial reflex was positive, but left stapedial reflex was negative. The degree of facial paralysis was evaluated on 40-point Yanagihara grading system.4The method has been widely used for evaluation of facial palsy: the sum of the given rating scores of 10 discrete functional units, such as left–right symmetry, close one eye, whistle and so on, is designated as the paralysis score. A score of 10 points

(almost complete paralysis), which is equivalent to House– Brackmann grade V5, was observed in this patient (Fig. 1b). Laboratory abnormalities included elevated serum amylase (931 IU/L; normal, 70–240) with no clinical evidence of pancreatic disease. The patient had a white blood cell (WBC) count of 10 300/μL. The WBC differential noted 71% neutrophil, 19% lymphocytes, 9% monocytes and 1% eosinophils. Electrolyte profile (Na, 143 mmol/L; K, 4.5 mmol/L; and, Cl 102 mmol/L), hepatic enzymes (aspartate aminotransferase, 21 U/L; alanine aminotransferase, 13U/L), erythrocyte sedimentation rate (9 mm/ h), C-reactive protein (0.1 mg/dL) and interleukin 2 receptor (IL2R; 457 U/mL) were all within normal limits. On computed tomography and magnetic resonance imaging, multiple cystic lesions were seen in the left parotid region (Fig. 2a–d). Ultrasonography of the parotid region showed multiple cystic lesions, with no vascularization (Fig. 2e,f). These images showed features typical of lymphangioma. Given that facial nerve paralysis is rarely caused by benign lesions, we suspected that this clinical presentation of facial nerve paralysis might be caused by a malignant tumor, such as malignant lymphoma or mucoepidermoid carcinoma. For this reason, at 2 days after admission, open biopsy was done under local anesthesia in order to investigate the cause of the facial nerve paralysis. The tumor contained multiple cystic lesions, containing bloody serous liquid. The histopathological findings of the excised tumor were consistent with lymphangioma (Fig. 3). The patient

Fig. 1 Left parotid region and left facial nerve paralysis. (a) Tense, tender, and non-erythematous mass (10 × 8 cm) in the left parotid region. (b) Left facial nerve paralysis. © 2014 Japan Pediatric Society

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M Imaizumi et al. received i.v. cefazolin (15 mg/kg) for 4 days, and predonine 40 mg, 30 mg, 20 mg, 10 mg, 5 mg, for 2 days each. The size of the mass decreased gradually. One month after steroid therapy, facial nerve paralysis was completely cured and the size of the mass was 15 mm × 15 mm. The left parotid lymphangioma has remained unchanged and there has been no recurrent facial nerve paralysis for 8 years.

Discussion

Fig. 2 (a) Axial-enhanced computed tomography showing deviation of the upper airway due to the presence of the parotid tumor. The low-density area without enhancement was partly found in the tumor. (b) Axial, T1-weighted magnetic resonance imaging (MRI) showing a low-intensity mass. (c) Axial, T2-weighted MRI showing a lowintensity mass containing high-intensity areas. (d) Axial, gadolinium (Gd)-enhanced T1-weighted MRI showing enhancement of the dissepiments, without enhancement of the internal fluid. (e) Ultrasonography showing multiple cystic lesions with relatively wide cavities. The cavities of the tumor were easily collapsed by compression of the probe during examination. (f) Color-Doppler ultrasonography showing no vascularization in cystic cavities.

Fig. 3 (a) The excised tumor consisted of connective tissue with lymphocyte infiltration. No salivary gland tissue was observed. (b) Expansion of the cyst by lymphatic vessel-like tissue was marked. Because there was no sign of blood-pooling in the vessels, lymphangioma was diagnosed. Given that there was no constant increase or abnormalities in the infiltrating lymphocytes, the patient was regarded as negative for malignant lymphoma (H&E).

© 2014 Japan Pediatric Society

Parotid lymphangioma is a relatively rare disease. It is usually detected in infancy or early childhood,2 and has typical features, thus it is not difficult to reach a diagnosis. The clinical presentation of facial nerve paralysis caused by lymphangioma, however, is very rare. There has been no report of parotid lymphangioma associated with facial nerve paralysis without iatrogenic procedures, trauma or previous infection. Facial nerve paralysis induced by iatrogenic procedures, such as aspiration, injection of various agents, surgery and so on, is relatively common,2,3,6 therefore the present patient had an extremely rare course. Generally, facial nerve paralysis associated with a parotid tumor in a child suggests a malignant tumor.3 For that reason, open biopsy was done to determine the cause of the facial nerve paralysis in the present case. The histopathological findings of the excised tumor were consistent with lymphangioma. The tumor consisted of multiple cystic lesions, containing bloody serous liquid. The fluid in lymphangiomas is usually watery, serous, clear, or strawcolored.7 Therefore, facial nerve paralysis was considered secondary to the complete compression of the nerve by the hemorrhage-filled lymphangioma. This is a very unusual cause of facial nerve paralysis because this nerve has great resistance to paresis when stretched around or compressed by benign parotid masses.3 It was considered that rapid enlargement of the mass, usually associated with trauma,8 infection8,9 or hemorrhage into the cyst,2,9 induced drastic compression or stretching of the facial nerve, thus causing facial nerve paralysis. Surgical excision is the recommended treatment standard,10 but lymphangioma with multiple cysts is difficult to extirpate completely without damage. In the case of incomplete excision, the rate of disease recurrence is high. Giguere et al. reported OK-432 as primary therapy for lymphangioma,11 but the parents

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Lymphangioma with facial nerve paralysis of the present patient refused additional treatment. The present patient received no treatment for the parotid lymphangioma after the open biopsy and prednisolone treatment. Facial nerve paralysis was completely cured 1 month after medication. The left parotid lymphangioma has remained unchanged and there has been no recurrent facial nerve paralysis for 8 years.

References 1 Brock ME, Smith RJ, Parey SE, Mobley DL. Lymphangioma. An otolaryngologic perspective. Int. J. Pediatr. Otorhinolaryngol. 1987; 14: 133–40. 2 Tsui SC, Huang JL. Parotid lymphangioma. A case report. Int. J. Pediatr. Otorhinolaryngol. 1996; 34: 273–8. 3 Som PM, Zimmerman RA, Biller HF. Cystic hygroma and facial nerve paralysis: A rare association. J. Comput. Assist. Tomogr. 1984; 8: 110–13. 4 Yanagihara N. On standardised documentation of facial palsy (author transl). Nippon Jibiinkoka Gakkai Kaiho 1977; 80: 799– 805 (in Japanese).

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5 House JW, Brackmann DE. Facial nerve grading system. Otolaryngol. Head Neck Surg. 1985; 93: 146–7. 6 Som PM, Lidov M, Lawson W. Hemorrhaged cystic hygroma and facial nerve paralysis: CT and MR findings. J. Comput. Assist. Tomogr. 1990; 14: 668–71. 7 Livesey JR, Soames JV. Cystic lymphangioma in the adult parotid. J. Laryngol. Otol. 1992; 106: 566–8. 8 Ricciardelli EJ, Richardson MA. Cervicofacial cystic hygroma. Patterns of recurrence and management of the difficult case. Arch. Otolaryngol. Head Neck Surg. 1991; 117: 546–53. 9 Kornblut AD, Ilse H, Haubrich J. Parotid lymphangioma: A congenital tumour. ORL J. Otorhinolaryngol. Relat. Spec. 1973; 35: 303–14. 10 Fageeh N, Manoukian J, Tewfik T, Schloss M, Williams HB, Gaskin D. Management of head and neck lymphatic malformations in children. J. Otolaryngol. 1997; 26: 253–8. 11 Giguere CM, Bauman NM, Sato Y et al. Treatment of lymphangiomas with OK-432 (Picibanil) sclerotherapy: A prospective multi-institutional trial. Arch. Otolaryngol. Head Neck Surg. 2002; 128: 1137–44.

Peripheral blood flow monitoring in an infant with septic shock Akio Ishiguro, Sayaka Sakazaki, Ryuta Itakura, Sumie Fujinuma, Shuntaro Oka, Yukiko Motojima, Hisanori Sobajima and Masanori Tamura Department of Pediatrics, Division of Neonatal Medicine, Center for Maternal, Fetal and Neonatal Medicine, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan Abstract

Septic shock is associated with impaired vasoregulation, and treatment includes vasoactive drugs. Therefore, evaluation of vasoregulatory change is important. The present report describes the successful characterization of vasoregulatory change in response to a vasoactive drug during septic shock. A male infant born at 23 weeks’ gestation developed septic shock. Severe hypotension developed, and treatment with colloid fluid and dopamine failed to increase blood pressure. With continuous measurement of skin blood flow using laser Doppler, noradrenaline was started. Based on changes in the blood flow, the dose was increased. At a dose of 1 μg/kg per min, skin blood flow in the foot decreased without any change in blood pressure. Subsequent blood transfusion succeeded in increasing both blood pressure and skin blood flow. It is concluded that decrease in foot blood flow reflects the vasoconstrictive effect of noradrenaline, although this finding must be validated in larger studies.

Key words laser Doppler flowmetry, microcirculation, regional blood flow, sepsis, septic shock.

In infants with septic shock, impaired peripheral vasoregulation can cause circulatory changes. These infants present with either warm shock caused by vasodilation or cold shock caused by vasoconstriction.1 Because vasoregulatory changes can appear before specific evidence of the apparent shock, the detection of

Correspondence: Akio Ishiguro, MD PhD, Department of Pediatrics, Division of Neonatal Medicine, Center for Maternal, Fetal and Neonatal Medicine, Saitama Medical Center, Saitama Medical University, 1981 Kamoda, Kawagoe, Saitama 350-8550, Japan. Email: akio-i @k4.dion.ne.jp Received 27 January 2014; revised 21 February 2014; accepted 3 March 2014. doi: 10.1111/ped.12345

these vasoregulatory changes would facilitate early intervention for septic shock. In addition, because the circulatory support for such conditions usually consists of vasoactive drugs, an accurate method to monitor the vasoconstrictive or vasodilative effects of the drugs would be of benefit. Direct measurement of skin blood flow using a laser Doppler flowmeter2,3 can detect early changes in peripheral circulation. We previously described the effect of dopamine on peripheral circulation in very low-birthweight infants.2 We suspected that the method would be useful for the detection of the effects of the vasoactive drugs in infants with septic shock. This case report describes the successful detection of changes in skin blood flow in an extremely low-birthweight infant who was successfully treated for septic shock. © 2014 Japan Pediatric Society

Parotid lymphangioma associated with facial nerve paralysis.

Parotid lymphangioma is a relatively rare disease that is usually detected in infancy or early childhood, and which has typical features. Clinical rep...
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