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Severe recurrent epistaxis in children: A case report involving two complex underlying conditions
International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 335–338
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology Extra journal homepage: www.elsevier.com/locate/ijporl
Case report
Severe recurrent epistaxis in children: A case report involving two complex underlying conditions ˜ iga d,* Constanza Beltra´n a, Raimundo Jose´ Garcı´a b, Jose´ Tevah c, Ximena Fonseca a, Pamela Zu´n a
Departamento de Otorrinolaringologı´a, Facultad de Medicina, Pontificia Universidad Cato´lica de Chile, Santiago, Chile Escuela de Medicina, Pontificia Universidad Cato´lica de Chile, Santiago, Chile c Departamento de Radiologı´a, Facultad de Medicina, Pontificia Universidad Cato´lica de Chile, Santiago, Chile d Departamento de Pediatrı´a - Hematologı´a, Facultad de Medicina, Pontificia Universidad Cato´lica de Chile, Santiago b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 2 September 2010 Received in revised form 12 February 2011 Accepted 15 February 2011 Available online 16 April 2011
Epistaxis is very frequent in pediatrics affecting almost 60% of children. It is generally a benign condition, but occasionally an underlying disease such as a systemic coagulopathy or a local tumor may be present. We report a case of a child with severe recurrent epistaxis, in whom both a mild platelet function disorder (PFD) and an intranasal infantile hemangioma (IH) were diagnosed after several diagnostic efforts. Both conditions acted synergistically in the genesis of epistaxis. PFD involves a group of hereditary defects of primary hemostasis that are frequently under-diagnosed. IH is the most common tumor of childhood and is preferently localized in the head and neck areas causing unilateral epistaxis when located in the nose. We address the place of complementary hematological or local evaluations in children presenting severe recurrent epistaxis. ß 2011 Elsevier Ireland Ltd. All rights reserved.
Keywords: Recurrent epistaxis Children Coagulopathies Platelet function disorders Infantile hemangioma
1. Clinical case The patient is an 8-year-old boy with a medical history of bilateral recurrent epistaxis for the last 2 years, without any other sign of mucocutaneous bleeding. During the past two months bleeding episodes increased in frequency and severity determining multiple consultations at the emergency department, receiving treatment with nasal packing and cauterization. In the current episode, he is admitted to our hospital after several hours of active bleeding and with a severe anemia of 5.5 g/ dL Hb. He undergoes anterior nasal packing and repeated cauterization attempts without success. Rhinoscopy shows chronic adhesions between the septum and the inferior turbinate, probably secondary to previous repeated cauterizations. He is treated with 2 packed blood cell transfusion, endovenous tranexamic acid and anterior packing with Merocel1. 2. Recurrent epistaxis Epistaxis is a common pediatric symptom, affecting 30–64% of children [1]. It is more frequently a benign and self-limited condition, but also a major cause of medical consultation and parental concerns.
* Corresponding author. Tel.: +56 2 3543273; fax: +56 2 6395123. ˜ iga). E-mail addresses: [email protected], [email protected] (P. Zu´n 1871-4048/$ – see front matter ß 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2011.02.009
Most of epistaxis episodes are originated in the anteroinferior part of the nasal septum, specifically at the Kiesselbach’s plexus or Little’s area. This is explained anatomically by the confluence and anastomosis of branches of the sphenopalatine, anterior ethmoidal, superior labial and greater palatine arteries. These vessels follow a superficial layer on the nasal mucosa, thus predisposing to epistaxis. Some local or environmental conditions that lead to vascular congestion, or mucosal irritants such as cold or dry air, S. aureus colonization, anatomical alterations, upper respiratory tract infections, rhinitis and trauma can increment the bleeding risk at this zone. Less frequently, an underlying systemic coagulopathy or a tumor, can be involved in the genesis of recurrent epistaxis [2]. Post transfusion Hb increases to 9 g/dL. The hemogram shows a normal platelet count, with microcytosis and macroplatelets at the peripheral smear evidencing the chronic process. Bleeding time (BT), partial thromboplastin time (PT) and activated partial thromboplastin time (PTT) were in normal range. He is discharged with scheduled controls with an otolaryngologist and a hematologist. Specific tests to rule out von Willebrand disease (VWD) and also platelet secretion–aggregation tests, are ordered. A week later he restarts with a left nostril epistaxis after retiring the anterior bilateral packing, for which he has to be rehospitalized and retamponated. The coagulation tests show a defect of platelet aggregation and secretion to different agonists, compatible with a primary alteration of platelet secretion. With this finding he receives desmopressin (DDAVP) with partial results, and a four-platelet unit
336
C. Beltra´n et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 335–338
Fig. 1. Embolization of the left internal maxillary artery. A and B show the facial arteries prior embolization. C shows the embolization of the left internal maxillary artery. D shows the result of the procedure.
transfusion thereafter, with bleeding resolution and subsequent hospital discharge.
3. Platelet function disorders Primary hemostatic disorders affect the interaction of blood platelets with the vessel wall, which normally concludes with the formation of the platelet plug. This process is influenced primarily by the level and function of both von Willebrand factor (VWF) and platelets, and is pathologically expressed by VWD disease and platelet function disorders (PFD), respectively [3]. PFD are a heterogeneous group of diseases characterized by some alteration in the platelet aggregation or secretion to different agonists. Although PFD are at least as frequent as VWD [4], they are frequently subdiagnosed, mainly due to the lack of recognition and difficulties in the standardization of laboratory diagnosis [5]. Recent developments in genetic background and physiopathology are increasing our understanding of the complexity and heterogeneity of this entity [6]. Clinically PFD are indistinguishable from other primary hemostatic disorders like VWD. The symptoms are mainly of mucocutaneous bleeding, especially ecchymosis, epistaxis, menorrhagia; and excessive bleeding after minor injuries or invasive procedures such as surgery or dental extractions. PFD are grouped according to their clinical manifestations into mild and severe, being most of them mild with slight bleeding, generally secondary to hemostatic challenges. In severe PFD the hemorrhagic manifestations can be spontaneous, of greater severity and starting earlier in life. This group includes better-
characterized disorders such as Glanzmann’s thrombasthenia and Bernard-Soulier syndrome [7]. Pediatric cases of mild PFD involve a greater diagnostic challenge and unfortunately, sometimes diagnosis is made only after a life-threatening bleeding event secondary to a surgical procedure. Moreover, there is a subgroup of patients presenting as mucocutaneous bleeders but with negative laboratory tests. These patients are known as bleeders of unknown cause (BUC), and in our experience, their prevalence is higher than VWD and PFD taken together [4]. To add more complexity, recommendations for treatment and prophylaxis for these patients are not supported by strong evidence [6]. Therapeutic approaches include mainly DDAVP, antifibrinolytic drugs and platelet transfusions as needed [7]. During follow-up, the patient is readmitted twice for severe recurrent unilateral episodes with basically the same treatment. Because of the change in epistaxis behavior and that the bleeding was considered abnormal for the diagnosed PFD, it is decided to investigate a vascular malformation. A CT scan of the paranasal sinuses shows no extravasation of contrast media and only local inflammatory changes at the ethmoid and maxillar sinuses. A selective sphenopalatine angiography does not show contrast media extravasation nor vascular malformation signs, which can happen in vascular malformations, and because of the high suspicion index a successful left internal maxillary artery embolization with 150–250 mm PVA particle is performed (Fig. 1). Six months later, a new massive unilateral episode leads to another hospitalization. Due to this torpid evolution a surgical exploration is decided. During the procedure, bleeding from the anterior zone of the middle turbinate is noted. A tumor of vascular appearance in the
C. Beltra´n et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 335–338
337
earlier and more active management of IH to avoid psychological and social repercussions may be suitable. The patient evolves satisfactorily and is discharged without active bleeding. A 3-year follow-up shows no new epistaxis episodes, normal Hb and a healthy growth and development.
5. Discussion
Fig. 2. Hemangioma in the anterior ethmoid. Hemangioma (A) between the septum (B) and the sectioned middle turbinate (C).
anterior ethmoid is found and extracted after the section of the middle turbinate. The biopsy shows an infantile hemangioma (Fig. 2). 4. Infantile hemangioma Infancy vascular lesions refer to both vascular malformations and tumors. These lesions, although with different characteristics and manifestations, share a common origin in the vascular system. Vascular malformations are classified according to the original vessel into lymphatic, venous, arterial, capillary and mixed. Vascular tumors include infantile hemangiomas (IH), congenital hemangiomas and other vascular tumors [8,9]. IH are the most common tumors of childhood. Classically, an incidence of 10% in Caucasian infants was described [10,11], but more recent studies and classifications reduced the estimate to 5% [12]. They are 2–3 times more common among females than males [13]. Histologically, these hemangiomas are vascular tumors formed by the clonal proliferation of endothelial cells [14,15], resulting in the formation of new blood vessels from angioblasts, a process known as vasculogenesis [16]. Generally, they are clinically evident from the first weeks of life and exhibit a fast proliferation phase characterized by endothelial proliferation and hypercellularity, followed by a spontaneous slow involution phase in which the vascular component is replaced by collagen or fat over time [9]. More than the 60% of hemangiomas appear in the head and neck regions [17] and approximately 16% occur in the nose [18]. Specifically, 63% is localized in the septum, 18% in the lateral wall and 16% in the vestibule [19], being most of them of the capillary type [20]. In most cases hemangiomas can be diagnosed with the clinical history and physical examination [17] but there are cases when the tumor is not visible and can only be suspected by the symptoms that appear depending on the area involved. The management of the IH is conservative in most cases, reserving treatment for complications, such as massive bleeding. Treatment may include high-dose steroids, intralesional steroids, interferon, propranolol, laser-resection, tumor embolization or surgery [21]. Even when completely involutioned, IH can induce considerable anatomic alterations because of atrophic skin changes, telangiectasias, and fibro fatty tissue deposition, especially in the visible zones of the head and neck [22]. Considering the development of new local therapies with few adverse effects (lesion-specific lasers, interventional radiology, surgery) [23], an
Although most cases of epistaxis in children are mild, selflimited and respond to usual therapy, there is a subgroup of patients presenting recurrent, severe or even life-threatening episodes. In these cases, it is important to acknowledge the possibility of a hidden systemic or local underlying condition. Eventually, disclosure of these underlying conditions may lead to an adequate or complete resolution of bleeding episodes. The questions are in whom should these conditions be suspected and what is the best diagnostic approach to unravel them. It is not clear whether the severity of epistaxis can predict the presence of more complex systemic or local pathologies [4,24–28]. Nevertheless, it seems prudent to consider that severe recurrent epistaxis episodes requiring repetitive cauterizations or transfusions should be further studied. Up to 33% of children who consult for recurrent epistaxis present some bleeding disorder [24]. The presence of other symptoms of mucocutaneous bleeding in the patient or close relatives should arise suspicion. In that case, besides basic coagulation screening, specific tests to rule out VWD or PFD should be ordered. Concerning local pathologies, children with unilateral recurrent epistaxis associated to nasal obstruction or headache should be studied to rule out a nasopharinx neoplasia [29]. Diagnostic options after rhinoscopy include CT scan, NMR or angiography depending on available resources [30]. When these methods fail because of the characteristics of the lesion, and there is a high index of suspicion or a poor clinical evolution that justifies it, blinded surgical exploration may be a last but useful option as illustrated by this report. In summary, when approaching children with recurrent epistaxis, additional studies should be performed in those with a personal or family history of other mucocutaneous bleeding episodes, presenting severe or life-threatening episodes, or with unilateral epistaxis associated to local symptoms. This appears to be particularly true in 2-year-old children or younger who exhibit a higher and more troublesome incidence [31,32].
Conflict of interest The authors declare no conflict of interest. References [1] B. Petruson, Epistaxis in childhood, Rhinology 17 (1979) 83–90. [2] J.L. Guarisco, H.D. Graham, Epistaxis in children: causes, diagnosis, and treatment, Ear Nose Throat J. 68 (1989) 522–538. [3] E.J. Favaloro, Investigating people with mucocutaneous bleeding suggestive of primary hemostatic defects: a low likelihood of a definitive diagnosis? Haematologica 92 (2007) 292–296. [4] T. Quiroga, M. Goycoolea, O. Panes, et al., High prevalence of bleeders of unknown cause among patients with inherited mucocutaneous bleeding. A prospective study of 280 patients and 299 controls, Haematologica 92 (2007) 357–365. [5] T. Quiroga, M. Goycoolea, V. Matus, et al., Diagnosis of mild platelet function disorders. Reliability and usefulness of light transmission platelet aggregation and serotonin secretion assays, Br. J. Haematol. 147 (2009) 729–736. [6] P.H.B. Bolton-Maggs, E.A. Chalmers, P.W. Collins, et al., A review of inherited platelet disorders with guidelines for their management on behalf of the UKHCDO, Br. J. Haematol. 135 (2006) 603–633. [7] A. Coppola, G. Di Minno, Desmopressin in inherited disorders of platelet function, Haemophilia 14 (S1) (2008) 31–39.
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[8] J.B. Mulliken, J. Glowacki, Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics, Plast. Reconstr. Surg. 69 (1982) 412–422. [9] D.M. Adams, A.W. Lucky, Cervicofacial vascular anomalies. I. Hemangiomas and other benign vascular tumors, Semin. Pediatr. Surg. 15 (2) (2006) 124–132. [10] A.H. Jacobs, R.G. Walton, The incidence of birthmarks in the neonate, Pediatrics 58 (1976) 218. [11] J.C. Alper, L.B. Holmes, The incidence and significance of birthmarks in a cohort of 4,641 newborns, Pediatr. Dermatol. 1 (1983) 58. [12] C. Kilcline, I.J. Frieden, Infantile hemangiomas: how common are they? A systematic review of the medical literature, Pediatr. Dermatol. 25 (2008) 168. [13] R.E. Bowers, E.A. Graham, K.M. Tomlinson, The natural history of the strawberry nevus, Arch. Dermatol. 82 (1960) 667–680. [14] E. Boye, Y. Yu, G. Paranya, et al., Clonality and altered behavior of endothelial cells from hemangiomas, J. Clin. Invest. 107 (2001) 745. [15] J.W. Walter, P.E. North, M. Waner, et al., Somatic mutation of vascular endothelial growth factor receptors in juvenile hemangioma, Genes Chromosomes Cancer 33 (2002) 295. [16] V.A. Nguyen, C. Furhapter, N. Romani, et al., Infantile hemangioma is a proliferation of beta 4-negative endothelial cells adjacent to HLA-DR-positive cells with dendritic cell morphology, Hum. Pathol. 35 (2004) 739. [17] M.C. Finn, J. Glowacki, J.B. Mulliken, Congenital vascular lesions: clinical application of a new classification, J. Pediatr. Surg. 18 (1983) 894. [18] M. Waner, P.E. North, K. Scherer, I.J. Frieden, A. Waner, M.C. Mihm, The nonrandom distribution of facial hemangiomas, Arch. Dermatol. 139 (7) (2003) 869–875. [19] D.A. Osborn, Hemangiomas of the nose, J. Laryngol. Otol. 73 (1959) 174–179. [20] J.G. Batsakis, D.H. Rice, The pathology of head and neck tumors: vasoformative tumors, part 9A, Head Neck Surg. 3 (1981) 231–239.
[21] F.M. Tucci, G.C. De Vincentiis, E. Sitzia, L. Giuzio, M. Trozzi, S. Bottero, Head and neck vascular anomalies in children, Int. J. Pediatr. Otorhinolaryngol. 73 (S1) (2009) S71–S76. [22] M. Waner, J.Y. Suen, Treatment of hemangiomas of the head and neck, Laryngoscope 102 (1992) 1123–1132. [23] M. Very, M. Nagy, M. Carr, S. Collins, L. Brodsky, Hemangiomas and vascular malformations: analysis of diagnostic accuracy laryngoscope 112 (2002) 612–615. [24] C. Sandoval, S. Dong, P. Visintainer, et al., Clinical and laboratory features of 178 children with recurrent epistaxis, J. Pediatr. Hematol. Oncol. 65 (2002) 47–49. [25] V. Kiley, J.J. Stuart, C.A. Johnson, Coagulation studies in children with isolated recurrent epistaxis, J. Pediatrics 100 (1982) 579–581. [26] E. Katsanis, L. Koon-Hung, E. Hsu, et al., Prevalence and significance of mild bleeding disorders in children with recurrent epistaxis, J. Pediatrics 113 (1988) 73–76. [27] F.J. Garcia Callejo, M.M. Velert Vila, J. Marco Algarra, Recurrent epistaxis in children as a predictor for hemostatic disorders, An. Esp. Pediatr. 49 (1998) 475–480. [28] J.F. Damrose, J. Maddalozzo, Pediatric epistaxis, Laryngoscope 116 (2006) 387– 393. [29] J.K. Roberts, G.K. Korones, H.L. Levine, et al., Results of surgical management of nasopharyngeal angiofibromas: the Cleveland Clinic experience, 1977–1986, Cleve Clin. J. Med. 56 (1989) 529–533. [30] A. Alvi, N. Joyner-Tripplet, Acute epistaxis. How to spot the source and stop the flow, Postgrad. Med. 99 (1996) 83. [31] N. McIntosh, Y.Q. Jacqueline, J.Y.Q. Mok, A. Margerison, Epidemiology of oronasal hemorrhage in the first 2 years of life: implications for child protection, Pediatrics 120 (2007) 1074–1078. [32] N. McIntosh, J. Chalmers, Incidence of oronasal haemorrhage in infancy presenting to general practice in the UK, Br. J. Gen. Pract. 58 (557) (2008) 877–879.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology Extra journal homepage: www.elsevier.com/locate/ijporl
Case report
Severe recurrent epistaxis in children: A case report involving two complex underlying conditions ˜ iga d,* Constanza Beltra´n a, Raimundo Jose´ Garcı´a b, Jose´ Tevah c, Ximena Fonseca a, Pamela Zu´n a
Departamento de Otorrinolaringologı´a, Facultad de Medicina, Pontificia Universidad Cato´lica de Chile, Santiago, Chile Escuela de Medicina, Pontificia Universidad Cato´lica de Chile, Santiago, Chile c Departamento de Radiologı´a, Facultad de Medicina, Pontificia Universidad Cato´lica de Chile, Santiago, Chile d Departamento de Pediatrı´a - Hematologı´a, Facultad de Medicina, Pontificia Universidad Cato´lica de Chile, Santiago b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 2 September 2010 Received in revised form 12 February 2011 Accepted 15 February 2011 Available online 16 April 2011
Epistaxis is very frequent in pediatrics affecting almost 60% of children. It is generally a benign condition, but occasionally an underlying disease such as a systemic coagulopathy or a local tumor may be present. We report a case of a child with severe recurrent epistaxis, in whom both a mild platelet function disorder (PFD) and an intranasal infantile hemangioma (IH) were diagnosed after several diagnostic efforts. Both conditions acted synergistically in the genesis of epistaxis. PFD involves a group of hereditary defects of primary hemostasis that are frequently under-diagnosed. IH is the most common tumor of childhood and is preferently localized in the head and neck areas causing unilateral epistaxis when located in the nose. We address the place of complementary hematological or local evaluations in children presenting severe recurrent epistaxis. ß 2011 Elsevier Ireland Ltd. All rights reserved.
Keywords: Recurrent epistaxis Children Coagulopathies Platelet function disorders Infantile hemangioma
1. Clinical case The patient is an 8-year-old boy with a medical history of bilateral recurrent epistaxis for the last 2 years, without any other sign of mucocutaneous bleeding. During the past two months bleeding episodes increased in frequency and severity determining multiple consultations at the emergency department, receiving treatment with nasal packing and cauterization. In the current episode, he is admitted to our hospital after several hours of active bleeding and with a severe anemia of 5.5 g/ dL Hb. He undergoes anterior nasal packing and repeated cauterization attempts without success. Rhinoscopy shows chronic adhesions between the septum and the inferior turbinate, probably secondary to previous repeated cauterizations. He is treated with 2 packed blood cell transfusion, endovenous tranexamic acid and anterior packing with Merocel1. 2. Recurrent epistaxis Epistaxis is a common pediatric symptom, affecting 30–64% of children [1]. It is more frequently a benign and self-limited condition, but also a major cause of medical consultation and parental concerns.
* Corresponding author. Tel.: +56 2 3543273; fax: +56 2 6395123. ˜ iga). E-mail addresses: [email protected], [email protected] (P. Zu´n 1871-4048/$ – see front matter ß 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2011.02.009
Most of epistaxis episodes are originated in the anteroinferior part of the nasal septum, specifically at the Kiesselbach’s plexus or Little’s area. This is explained anatomically by the confluence and anastomosis of branches of the sphenopalatine, anterior ethmoidal, superior labial and greater palatine arteries. These vessels follow a superficial layer on the nasal mucosa, thus predisposing to epistaxis. Some local or environmental conditions that lead to vascular congestion, or mucosal irritants such as cold or dry air, S. aureus colonization, anatomical alterations, upper respiratory tract infections, rhinitis and trauma can increment the bleeding risk at this zone. Less frequently, an underlying systemic coagulopathy or a tumor, can be involved in the genesis of recurrent epistaxis [2]. Post transfusion Hb increases to 9 g/dL. The hemogram shows a normal platelet count, with microcytosis and macroplatelets at the peripheral smear evidencing the chronic process. Bleeding time (BT), partial thromboplastin time (PT) and activated partial thromboplastin time (PTT) were in normal range. He is discharged with scheduled controls with an otolaryngologist and a hematologist. Specific tests to rule out von Willebrand disease (VWD) and also platelet secretion–aggregation tests, are ordered. A week later he restarts with a left nostril epistaxis after retiring the anterior bilateral packing, for which he has to be rehospitalized and retamponated. The coagulation tests show a defect of platelet aggregation and secretion to different agonists, compatible with a primary alteration of platelet secretion. With this finding he receives desmopressin (DDAVP) with partial results, and a four-platelet unit
336
C. Beltra´n et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 335–338
Fig. 1. Embolization of the left internal maxillary artery. A and B show the facial arteries prior embolization. C shows the embolization of the left internal maxillary artery. D shows the result of the procedure.
transfusion thereafter, with bleeding resolution and subsequent hospital discharge.
3. Platelet function disorders Primary hemostatic disorders affect the interaction of blood platelets with the vessel wall, which normally concludes with the formation of the platelet plug. This process is influenced primarily by the level and function of both von Willebrand factor (VWF) and platelets, and is pathologically expressed by VWD disease and platelet function disorders (PFD), respectively [3]. PFD are a heterogeneous group of diseases characterized by some alteration in the platelet aggregation or secretion to different agonists. Although PFD are at least as frequent as VWD [4], they are frequently subdiagnosed, mainly due to the lack of recognition and difficulties in the standardization of laboratory diagnosis [5]. Recent developments in genetic background and physiopathology are increasing our understanding of the complexity and heterogeneity of this entity [6]. Clinically PFD are indistinguishable from other primary hemostatic disorders like VWD. The symptoms are mainly of mucocutaneous bleeding, especially ecchymosis, epistaxis, menorrhagia; and excessive bleeding after minor injuries or invasive procedures such as surgery or dental extractions. PFD are grouped according to their clinical manifestations into mild and severe, being most of them mild with slight bleeding, generally secondary to hemostatic challenges. In severe PFD the hemorrhagic manifestations can be spontaneous, of greater severity and starting earlier in life. This group includes better-
characterized disorders such as Glanzmann’s thrombasthenia and Bernard-Soulier syndrome [7]. Pediatric cases of mild PFD involve a greater diagnostic challenge and unfortunately, sometimes diagnosis is made only after a life-threatening bleeding event secondary to a surgical procedure. Moreover, there is a subgroup of patients presenting as mucocutaneous bleeders but with negative laboratory tests. These patients are known as bleeders of unknown cause (BUC), and in our experience, their prevalence is higher than VWD and PFD taken together [4]. To add more complexity, recommendations for treatment and prophylaxis for these patients are not supported by strong evidence [6]. Therapeutic approaches include mainly DDAVP, antifibrinolytic drugs and platelet transfusions as needed [7]. During follow-up, the patient is readmitted twice for severe recurrent unilateral episodes with basically the same treatment. Because of the change in epistaxis behavior and that the bleeding was considered abnormal for the diagnosed PFD, it is decided to investigate a vascular malformation. A CT scan of the paranasal sinuses shows no extravasation of contrast media and only local inflammatory changes at the ethmoid and maxillar sinuses. A selective sphenopalatine angiography does not show contrast media extravasation nor vascular malformation signs, which can happen in vascular malformations, and because of the high suspicion index a successful left internal maxillary artery embolization with 150–250 mm PVA particle is performed (Fig. 1). Six months later, a new massive unilateral episode leads to another hospitalization. Due to this torpid evolution a surgical exploration is decided. During the procedure, bleeding from the anterior zone of the middle turbinate is noted. A tumor of vascular appearance in the
C. Beltra´n et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 335–338
337
earlier and more active management of IH to avoid psychological and social repercussions may be suitable. The patient evolves satisfactorily and is discharged without active bleeding. A 3-year follow-up shows no new epistaxis episodes, normal Hb and a healthy growth and development.
5. Discussion
Fig. 2. Hemangioma in the anterior ethmoid. Hemangioma (A) between the septum (B) and the sectioned middle turbinate (C).
anterior ethmoid is found and extracted after the section of the middle turbinate. The biopsy shows an infantile hemangioma (Fig. 2). 4. Infantile hemangioma Infancy vascular lesions refer to both vascular malformations and tumors. These lesions, although with different characteristics and manifestations, share a common origin in the vascular system. Vascular malformations are classified according to the original vessel into lymphatic, venous, arterial, capillary and mixed. Vascular tumors include infantile hemangiomas (IH), congenital hemangiomas and other vascular tumors [8,9]. IH are the most common tumors of childhood. Classically, an incidence of 10% in Caucasian infants was described [10,11], but more recent studies and classifications reduced the estimate to 5% [12]. They are 2–3 times more common among females than males [13]. Histologically, these hemangiomas are vascular tumors formed by the clonal proliferation of endothelial cells [14,15], resulting in the formation of new blood vessels from angioblasts, a process known as vasculogenesis [16]. Generally, they are clinically evident from the first weeks of life and exhibit a fast proliferation phase characterized by endothelial proliferation and hypercellularity, followed by a spontaneous slow involution phase in which the vascular component is replaced by collagen or fat over time [9]. More than the 60% of hemangiomas appear in the head and neck regions [17] and approximately 16% occur in the nose [18]. Specifically, 63% is localized in the septum, 18% in the lateral wall and 16% in the vestibule [19], being most of them of the capillary type [20]. In most cases hemangiomas can be diagnosed with the clinical history and physical examination [17] but there are cases when the tumor is not visible and can only be suspected by the symptoms that appear depending on the area involved. The management of the IH is conservative in most cases, reserving treatment for complications, such as massive bleeding. Treatment may include high-dose steroids, intralesional steroids, interferon, propranolol, laser-resection, tumor embolization or surgery [21]. Even when completely involutioned, IH can induce considerable anatomic alterations because of atrophic skin changes, telangiectasias, and fibro fatty tissue deposition, especially in the visible zones of the head and neck [22]. Considering the development of new local therapies with few adverse effects (lesion-specific lasers, interventional radiology, surgery) [23], an
Although most cases of epistaxis in children are mild, selflimited and respond to usual therapy, there is a subgroup of patients presenting recurrent, severe or even life-threatening episodes. In these cases, it is important to acknowledge the possibility of a hidden systemic or local underlying condition. Eventually, disclosure of these underlying conditions may lead to an adequate or complete resolution of bleeding episodes. The questions are in whom should these conditions be suspected and what is the best diagnostic approach to unravel them. It is not clear whether the severity of epistaxis can predict the presence of more complex systemic or local pathologies [4,24–28]. Nevertheless, it seems prudent to consider that severe recurrent epistaxis episodes requiring repetitive cauterizations or transfusions should be further studied. Up to 33% of children who consult for recurrent epistaxis present some bleeding disorder [24]. The presence of other symptoms of mucocutaneous bleeding in the patient or close relatives should arise suspicion. In that case, besides basic coagulation screening, specific tests to rule out VWD or PFD should be ordered. Concerning local pathologies, children with unilateral recurrent epistaxis associated to nasal obstruction or headache should be studied to rule out a nasopharinx neoplasia [29]. Diagnostic options after rhinoscopy include CT scan, NMR or angiography depending on available resources [30]. When these methods fail because of the characteristics of the lesion, and there is a high index of suspicion or a poor clinical evolution that justifies it, blinded surgical exploration may be a last but useful option as illustrated by this report. In summary, when approaching children with recurrent epistaxis, additional studies should be performed in those with a personal or family history of other mucocutaneous bleeding episodes, presenting severe or life-threatening episodes, or with unilateral epistaxis associated to local symptoms. This appears to be particularly true in 2-year-old children or younger who exhibit a higher and more troublesome incidence [31,32].
Conflict of interest The authors declare no conflict of interest. References [1] B. Petruson, Epistaxis in childhood, Rhinology 17 (1979) 83–90. [2] J.L. Guarisco, H.D. Graham, Epistaxis in children: causes, diagnosis, and treatment, Ear Nose Throat J. 68 (1989) 522–538. [3] E.J. Favaloro, Investigating people with mucocutaneous bleeding suggestive of primary hemostatic defects: a low likelihood of a definitive diagnosis? Haematologica 92 (2007) 292–296. [4] T. Quiroga, M. Goycoolea, O. Panes, et al., High prevalence of bleeders of unknown cause among patients with inherited mucocutaneous bleeding. A prospective study of 280 patients and 299 controls, Haematologica 92 (2007) 357–365. [5] T. Quiroga, M. Goycoolea, V. Matus, et al., Diagnosis of mild platelet function disorders. Reliability and usefulness of light transmission platelet aggregation and serotonin secretion assays, Br. J. Haematol. 147 (2009) 729–736. [6] P.H.B. Bolton-Maggs, E.A. Chalmers, P.W. Collins, et al., A review of inherited platelet disorders with guidelines for their management on behalf of the UKHCDO, Br. J. Haematol. 135 (2006) 603–633. [7] A. Coppola, G. Di Minno, Desmopressin in inherited disorders of platelet function, Haemophilia 14 (S1) (2008) 31–39.
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