Circumcision Procedure in Hemophilia Patients Revision.pptx

Sirkumsisi hemofilua

1. Circumcision in hemophilia patients Saga Aditya Hutama NIM 208072000111002

2. CIRCUMCISION

3. Introduction Male circumcision is one of the oldest surgical procedures known. This procedure usually as a mark of cultural identity or religious importance .

4. Prevalence of Circumcision in The World World Health Organization. 2007. Male circumcision: global trends and determinants of prevalence, safety and acceptability

5. The Anatomy Foreskin of penis The foreskin is a continuation of skin from the shaft of the penis that covers the glans penis and the urethral meatus The word “circumcision” comes from the Latin circumcidere (meaning “to cut around”) The possible function of foreskin: Keeping the glans moist Protecting the developing penis in utero Vella M, Abrate A, Argo A, dan Simonato A. 2017. Circumcision and Sexually Transmitted Disease Prevention: Evidence and Reticence

6. The Vascular and nerve of Foreskin Femoral A. -> External Pudendal A. -> Posterolateral and anterolateral Axial Branch, Interconnecting Branch, Perforating Branch At Subcoronal , Axial Branch -> Preputial Artery and Perforating Branch Brandes , S. B. (2008) ‘Vascular Anatomy of Genital Skin and the Urethra: Implications for Urethral Reconstruction’, in Brandes , S. B. (ed.) Urethral Reconstructive Surgery . Totowa, NJ: Humana Press, pp. 9–18. doi : 10.1007/978-1-59745-103-1_2.

7. The Vascular and nerve of Foreskin Hsu, G. L. et al. (2013) ‘The venous drainage of the corpora cavernosa in the human penis’, Arab Journal of Urology . Arab Association of Urology, 11(4), pp. 384– The venous system there is superficial dorsal vein of the penis that runs along with the arteries and dorsal nervus of the penis drains the prepuce and skin of the penis

8. Indication of Circumcision Medical Indication World Health Organization. 2007. Male circumcision: global trends and determinants of prevalence, safety and acceptability Non Medical Indication

9. World Health Organization. 2007. Male circumcision: global trends and determinants of prevalence, safety and acceptability Hegazy , AA. and Mo, A. (2012) ‘Male circumcision : review and authors perspective’, 3(1), pp. 24–30 . Contraindication of Circumcision ABSOLUTE RELATIVE

10. What is Hemophilia ? Hemophilia is a common hereditary coagulation blood disorder due to deficiency or reduced activity of clotting factor VIII or clotting factor IX . This disorder is a X - linked recessive disorder . Hemophilia is a bleeding disorder that slows down the blood clotting process It’s transmitted via females to men who are sufferers People who have Hemophilia often have longer bleeding after some sort of contact to injury. Hemophilia is more common in males than females. From Massimo study explain why Haemophilia B is clinically less severe than haemophilia A. It determined from l ess severe gene mutations , l ess severe clinical symptoms, less factor consumption. Mehta P, Reddivari AKR. Hemophilia . [Updated 2021 Jun 15]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK551607/ Franchini M, Mannucci PM. Haemophilia B is clinically less severe than haemophilia A: further evidence. Blood Transfus . 2018 Feb;16(2):121-122. doi : 10.2450/2016.0158-16. Epub 2016 Oct 4. PMID: 27723453; PMCID: PMC5839607.

11. Type of Hemophilia and Cascade of Coagulation Hemophilia A Also known as classic hemophilia or Factor VIII Deficiency It’s the most common hemophilia type Hemophilia A is more prevalent (80% to 85% of the total hemophilia population) than hemophilia B 1 in 5000 live male births, Hemophilia B Also known as Christmas disease or Factor IX Deficiency 1 in 30000 live male births Hemophilia C Caused by Factor XI Deficiency It’s the rare hemophilia type Hemophilia C generally occurs in 1 of every 100000 people Mehta P, Reddivari AKR. Hemophilia . [Updated 2021 Jun 15]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK551607/

12. Sign and Symptom of Hemophilia U sually presents as bleeding after minor trauma or as a spontaneous bleed eg : Gum Bleeding, epistaxis, or prolonged bleeding episode than a normal Severe hemophilia usually manifests in the first few months of life, while mild or moderate hemophilia can present later in childhood or adolescence P atients often present with internal bleeding, potentially impacting multiple organs , typical symptoms of presentation are abdominal pain Some Bruises and contusion sign in body especially in extremities. Joints can become painful, swollen, inflamed, warm, and have a restricted range of motion due to bleeding . The most commonly affected joints are knees, elbows Tenderness and distention in abdominal palpation ANAMNESIS Physical Examination Mehta P, Reddivari AKR. Hemophilia . [Updated 2021 Jun 15]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-

13. How is Hemophilia diagnosed ? Hemophilia A&B are diagnosed by measuring clotting factor activity, made incidentally or on routine presurgical laboratory testing. Individuals who have Hemophilia A have low factor VIII clotting activity. Individuals who have hemophilia B have low factor IX clotting activity Platelet count: Normal Bleeding time: Normal PT : Normal Clotting time & PTT: Prolonged Factor VIII or Factor IX assay: Decreased Sivrikaya , Abdullah & Baran, Hatice & Abusoglu , Sedat & Öztürk , B. & Vatansev , Husamettin & Ali, UNLU. 2013. Effect of gender and age on the prothrombin time (PT), activated partial thromboplastin time ( aPTT ) levels and international normalized ratio (INR). International Journal of Mevlana Medical Sciences. 1. 27-29.

14. Incidence in Saiful Anwar General Hospital According Miftahul et al study in Saiful Anwar General Hospital there 23 hemophilia patient gathered and underwent circumcision procedure from October 2012 – March 2016 There is significant association between hemophilia and incidence of post circumcision About 75% patient had bleeding incidence after the procedure was hemophilia A patients Variable Total Percentage Number Of Patient 23 100% Age      5 yo 7 30% 6 – 10 yo 4 18% 11 – 15 yo 10 44% 16 – 20 yo 1 4% > 20 yo 1 4% Hemophilia Type     Hemophilia A 13 56% Hemophilia B 10 44% Anaesthesia Type     Local 1 4% General 22 96% Bleeding Incidence post Circumcision     Hemophilia A 6 26% Hemophilia B 2 9% Post Hemorrage Incidence Procedure     Regular Wound Care 5 22% Re-Circumcision 3 13% Miftahul Firdaus M., Daryanto B., Penta Seputra K. 2018. PERDARAHAN PASKA SIRKUMSISI PADA PENDERITA DENGAN HEMOFILIA. FAKULTAS KEDOKTERAN UNIVERSITAS BRAWIJAYA RUMAH SAKIT Dr. SAIFUL ANWAR MALANG

15. PREPARATION AND Surgical technique

16. A ntihemophilic F actor VIII Concentrate Hemofil M®, Koate ®, Koate -DVI® FVIII Unit = BW(Kg) X % (Desired FVIII Plasma Target – FVIII Plasma Patient) X 0,5 Give per 12 Hours

17. A ntihemophilic F actor IX Concentrate Immunine ®, Octanine - F® FIX Unit = BW(Kg) X % (Desired FIX Plasma Target – FIX Plasma Patient) Give per 24 Hours

18. Recommended plasma factor level and duration of administration Mukul, Sailesh & Agrawal, Amit. 2018. Management of Hemophilia: A Dental consideration. 7. 27-31. Park, Y. S. (2009).  Recent Advance of Pharmacotherapy in Hemophilia. Journal of the Korean Medical Association, 52(12), 1201

19. Skin Preparation Prepare the skin with povidone iodine aqueous solution, starting with the glans and the shaft of the penis, and then moving out to the periphery World Health Organization. 2018. Manual for male circumcision under local anaesthesia and HIV prevention services for adolescent boys and men. ISBN: 978-92-4-151359-3

20. Preparing The Penis a. Dilation of aperture of foreskin b. Retracting the foreskin to fully expose the glans, corona and coronal sulcus, and to separate any adhesion  if adhesion is hard to separate, refer to specialist Marking the line of circumcision A B Abdulwahab -Ahmed A, Mungadi IA. Techniques of male circumcision. J Surg Tech Case Rep. 2013 Jan;5(1):1-7. doi : 10.4103/2006-8808.118588. PMID: 24470842; PMCID: PMC3888996.

21. NON-SURGICAL / CIRCUMCISION DEVICE Peleg D, Steiner A. 1998. The Gomco Circumcision: Common Problems and Solutions . American Family Physician Sept.15 Page 1-10 WHO (2009) ‘Male circumcision under local anaesthesia Version 3.1(Dec09) Page i’, Manual for Male Circumcision under Local Anaesthesia , 1. Ma Qi, Fang Li, Yin Wei-qi et al. 2018. Chinese Shang Ring Male GOMCO CLAMP MOGEN CLAMP PLASTI BELL

22. PREPEX NON-SURGICAL / CIRCUMCISION DEVICE Abdulwahab-ahmed, A. and Mungadi, I. A. (2013) ‘Techniques of Male Circumcision’, (1), pp. 1–8. doi: 10.4103/2006-8808.118588. Jan IA. 2004. Circumcision In Babies and Children With Plastibell Technique: An Easy Procedure With Minimal Complications – Experience of 316 cases . Pak Journal Medical Science July-Sept. Vol. 20 No. 3 Page 175-180 TARA CLAMP SMART CLAMP SHANG RING

23. Circumcision Technique in H emophilia Patients Rodriguez et al used dorsal slit circumcision procedure in 38 Hemophilia A patient, only 2 patient had bleeding complications despite adequate recombinant factor replacement Bawazir et al used sleeve resection (circumference slit) procedure in 31 patients and done under general anesthesia . Only 2 patient had bleeding complications Sasmaz et al retrospectively reviewed 76 patients with hemophilia B underwent circumcision was done by dorsal slit technique. It was carried out under general anaesthesia except for three patients who were given local anaesthesia . These three patients were adolescent. RODRIGUEZ, V., TITAPIWATANAKUN, R., MOIR, C., SCHMIDT, K. A., & PRUTHI, R. K. (2009). To circumcise or not to circumcise? Circumcision in patients with bleeding disorders. Haemophilia, 16(2), 272–276. doi:10.1111/j.1365-2516.2009.02119.x Bawazir OA, Alharbi I. Circumcision in Hemophilia : A Multicenter Experience. J Pediatr Hematol Oncol. 2021 Jan;43(1):e33-e36. doi : 10.1097/MPH.0000000000001960. PMID: 33003145. SASMAZ, I., ANTMEN, B., LEBLEBISATAN, G., ŞAHIN KARAGÜN, B., KILINÇ, Y., & TUNCER, R. (2011).  Circumcision and complications in patients with haemophilia in southern part of Turkey: Çukurova experience. Haemophilia, 18(3), 426–430 Karaman , M. I., Zulfikar, B., Caskurlu , T., & Ergenekon, E. (2004).  Circumcision in hemophilia : A cost-effective method using a novel device. Journal of Pediatric Surgery, 39(10), 1562–1564

24. Circumcision Technique in H emophilia Patients Some operative procedure in hemophilia patient, as well as circumcision, used general anaesthesia considering the child’s cooperation limits, and to minimize postoperative pain and aid patient homeostasis during surgery Nerve block injections usually avoided to lower the risk of hematoma and/or internal bleeding Circumcision in hemophilia often requires the use of large quantities of clotting factor concentrates to ensure proper hemostasis. For these reasons, some haemophiliacs can’t be circumcised in countries where factor concentrated are unavailable or not easily accessible RODRIGUEZ, V., TITAPIWATANAKUN, R., MOIR, C., SCHMIDT, K. A., & PRUTHI, R. K. (2009). To circumcise or not to circumcise? Circumcision in patients with bleeding disorders. Haemophilia, 16(2), 272–276. doi:10.1111/j.1365-2516.2009.02119.x SASMAZ, I., ANTMEN, B., LEBLEBISATAN, G., ŞAHIN KARAGÜN, B., KILINÇ, Y., & TUNCER, R. (2011).  Circumcision and complications in patients with haemophilia in southern part of Turkey: Çukurova experience. Haemophilia, 18(3), 426–430 Karaman , M. I., Zulfikar, B., Caskurlu , T., & Ergenekon, E. (2004).  Circumcision in hemophilia : A cost-effective method using a novel device. Journal of Pediatric Surgery, 39(10), 1562–1564 El Batawi , H. Y. (2013).  Minimizing the Risk of Perioperative Bleeding in a Child with Hemophilia A during Dental Rehabilitation under General Anesthesia: A Case Report.

25. Pre and Post operative care after Circumcision in H emophilia Patients Widjajanto , P., Sugiarto , R., Sutaryo , S. and Ali, K. (2007) “Circumcision in boys with mild Hemophilia A – the Yogyakarta experience”,  Paediatrica Indonesiana , 47(2), pp. 71-3. doi : 10.14238/pi47.2.2007.71-3. HERMANS, C., ALTISENT, C., BATOROVA, A., CHAMBOST, H., DE MOERLOOSE, P., … KARAFOULIDOU, A. (2009).  Replacement therapy for invasive procedures in patients with haemophilia: literature review, European survey and recommendations. Haemophilia, 15(3), 639–658. Pudjo et al used combination of Tranexamic acid and Antihemophilic factor for his Hemophilia A patient who underwent circumcision procedure Herman et al had surveyed some Hemophilia A respondent were from Comprehensive Care Centres in Europe for some operative procedure and Review some circumcision in hemophilia literature

26. Pre and Post operative care after Circumcision in H emophilia Patients In Ridwan et al study, 4 hemophilia A patients underwent circumcision procedure and compare the treatment between continuous drip AHF and IV bolus AHF The protocol are measuring FVIII factor in each patient before procedure and recombinant FVIII concentrate was given at 25 IU/kg IV per 12 hours before , durante , and after the procedure In 4 th day of the treatment, after there is no sign of bleeding, recombinant FVIII concentrate was given at 25 IU/kg drip continuously for each patient and in 5 th day the treatment converted to 25 IU/kg IV bolus Continous drip AHF show clinically less than IV bolus AHF for increasing the plasma FVIII factor DRIP IV BOLUS

27. Complication Seck M, Sagna A, Guéye MS, Faye BF, Sy D, Touré SA, Sall A, Touré AO, Diop S. Circumcision in hemophilia using low quantity of factor concentrates: experience from Dakar, Senegal. BMC Hematol . 2017 Apr 24;17:8. doi : 10.1186/s12878-017-0080-1. PMID: 28451435; PMCID: PMC5402675. Bawazir OA, Alharbi I. Circumcision in Hemophilia : A Multicenter Experience. J Pediatr Hematol Oncol. 2021 Jan;43(1):e33-e36. doi : 10.1097/MPH.0000000000001960. PMID: 33003145. SASMAZ, I., ANTMEN, B., LEBLEBISATAN, G., ŞAHIN KARAGÜN, B., KILINÇ, Y., & TUNCER, R. (2011).  Circumcision and complications in patients with haemophilia in southern part of Turkey: Çukurova experience. Haemophilia, 18(3), 426–430 DURANTE Bawazir et al had bleeding intra-operative complication (6,5%) EARLY Sasmaz e t al had 3,5% penile hematoma incidence after procedure And 22,8% bleeding incidence after procedure Seck et al Bleeding complications were noted in 19.2% of patients LATE Bawazir et al included 31 patients with an earlier diagnosis of hemophilia had infection complication (3 ,2 %) And wound gaping complication (6,5%) Bleeding Infection Penile Hematoma

28. Conclusion 01 Circumcision Male circumcision is one of the oldest surgical procedures known mark of cultural identity or religious importance. Circumcision removes some, or all, of the foreskin from the penis 03 Circumcision in Hemophilia Dorsal slit and Circumference slit are well-known procedure for hemophilia patient. When General Anaesthesia become the choice of pre –procedural Anaesthesia 02 Hemophilia Hemophilia is a X - linked recessive disorder coagulation blood disorder due to deficiency or reduced activity of clotting factor 04 Before– procedure - After management Circumcision in hemophilia often requires the use of large quantities of clotting factor concentrates. The other reference used modalities like tranexamic acid, fibrin glue to ensure proper hemostasis and avoid complication

29. Thank you