Pediatric Case Report A Rare Case of Neonatal Alloimmune Thrombocytopenia Causing Prolonged Postcircumcision Bleeding Jacob Baber, Steven Kheyfets, and Joel Sumfest Postcircumcision bleeding can be the presenting symptom of a coagulopathy. A blood dyscrasia that is known but not documented in association with circumcision is neonatal alloimmune thrombocytopenia (NAIT). A 2-day-old male infant presented with postcircumcision bleeding. Platelet count was 5000 per microliter of blood. The child was treated conservatively with hematoma improvement. Few studies exist involving patients with coagulopathies who undergo circumcision. Our patient is the first to present with prolonged postcircumcision bleeding as the initial presentation of NAIT. NAIT and other coagulopathies that present as prolonged postcircumcision bleeding can often be managed successfully with conservative management. UROLOGY -: -e-, 2015. 2015 Elsevier Inc.
ale circumcision is one of the oldest and the most common surgical procedures.1 It is estimated that 1 in 3 men are circumcised worldwide for various reasons, including religious, ethnic, social, and health related.2 Postoperative complications are rare; the median frequency of any adverse event for neonatal and infant circumcisions is 1.5%, most commonly bleeding, pain, and infection.3-5 Although bleeding represents the most common complication after circumcision, the literature does not reflect an increased risk in patients with known bleeding disorders.6 However, a paucity of data currently exists analyzing this cohort, mostly representing patients with hemophilia and von Willebrand disease.6-8 Although the bleeding risk in this specific population appears to be equivalent to those without pre-existing bleeding states, prolonged bleeding after a circumcision can serve as a presenting symptom of an existing coagulopathy.7 In the case we describe, prolonged bleeding after circumcision in a neonate leads to the ultimate diagnosis of neonatal alloimmune thrombocytopenia (NAIT). To our knowledge, this represents the first case of NAIT being diagnosed as a result of prolonged bleeding after circumcision.
M
a community hospital for a nonreassuring heart tracing. On the first day of life, the infant underwent a Gomco clamp neonatal circumcision, which led to significant postprocedural bleeding despite manual pressure being applied to the site. Vitamin K was subsequently injected intramuscularly, and a hemostatic agent was applied to the site. Despite these measures, prolonged bleeding ensued, and the patient was transferred to our tertiary care hospital for further evaluation. The patient’s physical examination revealed significant penile edema and substantial ecchymosis surrounding the circumcision site and penile shaft. Laboratory work revealed a profoundly decreased platelet count of 5000 per microliter of blood. The pediatric hematology and oncology service was consulted and diagnosed the patient with NAIT. The child was treated conservatively with intravenous immunoglobulin and platelet transfusions without surgical intervention. On hospital day 2, the bleeding ceased, and the patient was discharged on hospital day 3. At a 9-month follow-up appointment, the patient’s platelet counts were within normal limits, and there was no abnormality of the circumcision site. At the 2-year follow-up appointment, patient continues to do well with no sequelae from the initial penile hematoma and penis remains well healed.
CASE REPORT
COMMENT
A full-term healthy male infant without significant family history was delivered via an emergent cesarean section at
Prolonged bleeding after circumcision may present as the initial symptom of an inherent bleeding disorder.7 We describe the first known case of NAIT in the literature diagnosed as a result of protracted bleeding after circumcision. NAIT was first described in 1953 and is the most common cause of severe neonatal thrombocytopenia with the incidence being approximately 1 per 1000 live
Financial Disclosure: The authors declare that they have no relevant financial interests. From the Department of Urology, Geisinger Medical Center, Danville, PA Address correspondence to: Jacob Baber, M.D., Department of Urology, Geisinger Medical Center, 100 N. Academy Avenue, Danville, PA 17821. E-mail: jababer10@ gmail.com Submitted: February 8, 2015, accepted (with revisions): February 19, 2015
ª 2015 Elsevier Inc. All Rights Reserved
http://dx.doi.org/10.1016/j.urology.2015.02.026 0090-4295/15
1
Figure 1. Algorithm for managing postcircumcision bleeding. BMP, basic metabolic panel; CBC, complete blood count; INR, international normalized ratio; PT, prothrombin time; PTT, partial thromboplastin time.
births.9 NAIT is caused by maternal alloantibodies crossing the placenta directed at fetal platelets. The majority of cases involve maternal antibodies directed against human platelet antigen 1a and 5b. The most common presentations include petechia in an otherwise well child and excessive bruising at an injection site; although, 10% of neonates with NAIT have no presenting symptom at all.9-12 A patient with prolonged bleeding after a circumcision could present to any number of physicians. Currently, no algorithm exists for the workup and management of prolonged bleeding after circumcision. We derived an algorithm based on current practices and thoughts on the subject (Fig. 1). One must always start with a history and physical examination of the patient paying close attention to family history in relation to blood and clotting disorders. Typically, this history is unknown or is negative, which leads the physician to the timing of the bleeding. Currently, there is no standard definition on the length of bleeding, amount of blood loss, or the type of bleeding and varies among articles. This can make assessment difficult, but it is ultimately at the discretion of the clinicians in what they believe is prolonged bleeding or a significant amount of bleeding. If the patient is stable and bleeding has occurred for 24 hours or the patient is bleeding a significant amount, then the patient may have a continued arterial bleed (typically the Frenular artery). The arterial bleeding would be treated with suture ligation or cautery. For those who do not have a discrete vessel, but generalized oozing, then a complete workup including laboratories with a 2
complete blood count and coagulation factors is warranted. Based on these values, the physician will know if additional consulting services are necessary for the evaluation of a blood disorder or for a surgical intervention. We believe this algorithm will allow any physician to start the necessary evaluation and expedite definitive management. In conclusion, we describe the first case of NAIT in the literature diagnosed after a protracted bleeding episode after circumcision. In this clinical scenario, providers must perform a thorough medical investigation of potential causes and have a high index of suspicion for blood disorders. We present an algorithmic approach to treating postcircumcision bleeding.
References 1. Nelson CP, Dunn R, Wan J, Wei JT. The increasing incidence of newborn circumcision: data from the nationwide inpatient sample. J Urol. 2005;173:978-981. 2. WHO/UNAIDS. Male Circumcision: Global Trends and Determinants of Prevalence, Safety, and Acceptability. Available at: http://whqlibdoc.who.int/publications/2007/97892415 96169_eng.pdf?ua=1; Accessed May 1, 2013. 3. Weiss HA, Larke N, Halperin D, Schenker I. Complications of circumcision in male neonates, infants and children: a systematic review. BMC Urol. 2010;10:1-13. 4. Krill AJ, Palmer LS, Palmer JS. Complications of circumcision. ScientificWorldJournal. 2011;11:2458-2468. 5. Palmer JS. Abnormalities of the external genitalia in boys. In: Wein AJ, Kavoussi LR, Novick AC, et al., eds. Campbell-Walsh Urology. 10 ed, vol. 4, chap 31. Philadelphia: Elsevier Co; 2012: 3540-3543. 6. Rodriguez V, Titapiwatanakun R, Moir C, et al. To circumcise or not to circumcise? Circumcision in patients with bleeding disorders. Haemophilia. 2010;16:272-276.
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7. Sasmaz I, Antmen B, Leblebisatan G, et al. Circumcision and complications in patients with haemophilia in southern part of Turkey; Cukurova experience. Haemophilia. 2012;18:426-430. 8. Watts RG, Cook RP. Operative management and outcomes in children with congenital bleeding disorders: a retrospective review at a single haemophilia treatment centre. Haemophilia. 2012;18: 421-425. 9. Rayment R, Birchall J, Yarranton H, et al. Neonatal alloimmune thrombocytopenia. Br Med J. 2003;327:331-332.
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10. Mueller-Eckhardt C, Kiefel V, Grubert A, et al. 348 cases of suspected neonatal alloimmune thrombocytopenia. Lancet. 1989;1:363-366. 11. Williamson LM, Hackett G, Rennie J, et al. The natural history of fetomaternal alloimmunization to the platelet-specific antigen HPA-1a (PlA1, Zwa) as determined by antenatal screening. Blood. 1998;92:2280-2287. 12. Blanchette VS, Johnson J, Rand M. The management of alloimmune neonatal thrombocytopenia. Baillieres Best Pract Res Clin Haematol. 2000;13:365-390.
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ale circumcision is one of the oldest and the most common surgical procedures.1 It is estimated that 1 in 3 men are circumcised worldwide for various reasons, including religious, ethnic, social, and health related.2 Postoperative complications are rare; the median frequency of any adverse event for neonatal and infant circumcisions is 1.5%, most commonly bleeding, pain, and infection.3-5 Although bleeding represents the most common complication after circumcision, the literature does not reflect an increased risk in patients with known bleeding disorders.6 However, a paucity of data currently exists analyzing this cohort, mostly representing patients with hemophilia and von Willebrand disease.6-8 Although the bleeding risk in this specific population appears to be equivalent to those without pre-existing bleeding states, prolonged bleeding after a circumcision can serve as a presenting symptom of an existing coagulopathy.7 In the case we describe, prolonged bleeding after circumcision in a neonate leads to the ultimate diagnosis of neonatal alloimmune thrombocytopenia (NAIT). To our knowledge, this represents the first case of NAIT being diagnosed as a result of prolonged bleeding after circumcision.
M
a community hospital for a nonreassuring heart tracing. On the first day of life, the infant underwent a Gomco clamp neonatal circumcision, which led to significant postprocedural bleeding despite manual pressure being applied to the site. Vitamin K was subsequently injected intramuscularly, and a hemostatic agent was applied to the site. Despite these measures, prolonged bleeding ensued, and the patient was transferred to our tertiary care hospital for further evaluation. The patient’s physical examination revealed significant penile edema and substantial ecchymosis surrounding the circumcision site and penile shaft. Laboratory work revealed a profoundly decreased platelet count of 5000 per microliter of blood. The pediatric hematology and oncology service was consulted and diagnosed the patient with NAIT. The child was treated conservatively with intravenous immunoglobulin and platelet transfusions without surgical intervention. On hospital day 2, the bleeding ceased, and the patient was discharged on hospital day 3. At a 9-month follow-up appointment, the patient’s platelet counts were within normal limits, and there was no abnormality of the circumcision site. At the 2-year follow-up appointment, patient continues to do well with no sequelae from the initial penile hematoma and penis remains well healed.
CASE REPORT
COMMENT
A full-term healthy male infant without significant family history was delivered via an emergent cesarean section at
Prolonged bleeding after circumcision may present as the initial symptom of an inherent bleeding disorder.7 We describe the first known case of NAIT in the literature diagnosed as a result of protracted bleeding after circumcision. NAIT was first described in 1953 and is the most common cause of severe neonatal thrombocytopenia with the incidence being approximately 1 per 1000 live
Financial Disclosure: The authors declare that they have no relevant financial interests. From the Department of Urology, Geisinger Medical Center, Danville, PA Address correspondence to: Jacob Baber, M.D., Department of Urology, Geisinger Medical Center, 100 N. Academy Avenue, Danville, PA 17821. E-mail: jababer10@ gmail.com Submitted: February 8, 2015, accepted (with revisions): February 19, 2015
ª 2015 Elsevier Inc. All Rights Reserved
http://dx.doi.org/10.1016/j.urology.2015.02.026 0090-4295/15
1
Figure 1. Algorithm for managing postcircumcision bleeding. BMP, basic metabolic panel; CBC, complete blood count; INR, international normalized ratio; PT, prothrombin time; PTT, partial thromboplastin time.
births.9 NAIT is caused by maternal alloantibodies crossing the placenta directed at fetal platelets. The majority of cases involve maternal antibodies directed against human platelet antigen 1a and 5b. The most common presentations include petechia in an otherwise well child and excessive bruising at an injection site; although, 10% of neonates with NAIT have no presenting symptom at all.9-12 A patient with prolonged bleeding after a circumcision could present to any number of physicians. Currently, no algorithm exists for the workup and management of prolonged bleeding after circumcision. We derived an algorithm based on current practices and thoughts on the subject (Fig. 1). One must always start with a history and physical examination of the patient paying close attention to family history in relation to blood and clotting disorders. Typically, this history is unknown or is negative, which leads the physician to the timing of the bleeding. Currently, there is no standard definition on the length of bleeding, amount of blood loss, or the type of bleeding and varies among articles. This can make assessment difficult, but it is ultimately at the discretion of the clinicians in what they believe is prolonged bleeding or a significant amount of bleeding. If the patient is stable and bleeding has occurred for 24 hours or the patient is bleeding a significant amount, then the patient may have a continued arterial bleed (typically the Frenular artery). The arterial bleeding would be treated with suture ligation or cautery. For those who do not have a discrete vessel, but generalized oozing, then a complete workup including laboratories with a 2
complete blood count and coagulation factors is warranted. Based on these values, the physician will know if additional consulting services are necessary for the evaluation of a blood disorder or for a surgical intervention. We believe this algorithm will allow any physician to start the necessary evaluation and expedite definitive management. In conclusion, we describe the first case of NAIT in the literature diagnosed after a protracted bleeding episode after circumcision. In this clinical scenario, providers must perform a thorough medical investigation of potential causes and have a high index of suspicion for blood disorders. We present an algorithmic approach to treating postcircumcision bleeding.
References 1. Nelson CP, Dunn R, Wan J, Wei JT. The increasing incidence of newborn circumcision: data from the nationwide inpatient sample. J Urol. 2005;173:978-981. 2. WHO/UNAIDS. Male Circumcision: Global Trends and Determinants of Prevalence, Safety, and Acceptability. Available at: http://whqlibdoc.who.int/publications/2007/97892415 96169_eng.pdf?ua=1; Accessed May 1, 2013. 3. Weiss HA, Larke N, Halperin D, Schenker I. Complications of circumcision in male neonates, infants and children: a systematic review. BMC Urol. 2010;10:1-13. 4. Krill AJ, Palmer LS, Palmer JS. Complications of circumcision. ScientificWorldJournal. 2011;11:2458-2468. 5. Palmer JS. Abnormalities of the external genitalia in boys. In: Wein AJ, Kavoussi LR, Novick AC, et al., eds. Campbell-Walsh Urology. 10 ed, vol. 4, chap 31. Philadelphia: Elsevier Co; 2012: 3540-3543. 6. Rodriguez V, Titapiwatanakun R, Moir C, et al. To circumcise or not to circumcise? Circumcision in patients with bleeding disorders. Haemophilia. 2010;16:272-276.
UROLOGY
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7. Sasmaz I, Antmen B, Leblebisatan G, et al. Circumcision and complications in patients with haemophilia in southern part of Turkey; Cukurova experience. Haemophilia. 2012;18:426-430. 8. Watts RG, Cook RP. Operative management and outcomes in children with congenital bleeding disorders: a retrospective review at a single haemophilia treatment centre. Haemophilia. 2012;18: 421-425. 9. Rayment R, Birchall J, Yarranton H, et al. Neonatal alloimmune thrombocytopenia. Br Med J. 2003;327:331-332.
UROLOGY
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(-), 2015
10. Mueller-Eckhardt C, Kiefel V, Grubert A, et al. 348 cases of suspected neonatal alloimmune thrombocytopenia. Lancet. 1989;1:363-366. 11. Williamson LM, Hackett G, Rennie J, et al. The natural history of fetomaternal alloimmunization to the platelet-specific antigen HPA-1a (PlA1, Zwa) as determined by antenatal screening. Blood. 1998;92:2280-2287. 12. Blanchette VS, Johnson J, Rand M. The management of alloimmune neonatal thrombocytopenia. Baillieres Best Pract Res Clin Haematol. 2000;13:365-390.
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