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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 59  |  Issue : 1  |  Page : 88-90

Triple procedure in blood-stained cornea of a hemophilia patient


Department of Cornea, RIO, GOH, Chennai, Tamil Nadu, India

Date of Submission10-Nov-2020
Date of Acceptance18-Jan-2021
Date of Web Publication27-Mar-2021

Correspondence Address:
Dr. Rajesh Mimmithi
Department of Cornea, RIO GOH, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_166_20

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  Abstract 


A 16-year-old male Type A hemophiliac patient has been reported with complaints of defective vision in the right eye (OD), following ocular trauma 9 months back for which he took treatment outside. On examination, he had blood staining of cornea and cataract in his right eye. His vision was perception of light + and projection of rays intact. After hematologist opinion, under peribulbar anesthesia, he underwent penetrating keratoplasty with pupillary membranectomy and cataractous lens removal with posterior chamber intraocular lens implantation under the cover of Factor VIII (antihemophilic factor). On day 21, the best-corrected visual acuity in OD was 6/6P. This case report emphasizes the fact that hemophiliac patients can be operated safely with appropriate hematological support.

Keywords: Blood stained cornea, Hemophilia A, penetrating keratoplasty, triple procedure, Factor VIII transfusion


How to cite this article:
Mimmithi R, Gandhi N, Babu M A, Vadivelu SD, Meenakshi B. Triple procedure in blood-stained cornea of a hemophilia patient. TNOA J Ophthalmic Sci Res 2021;59:88-90

How to cite this URL:
Mimmithi R, Gandhi N, Babu M A, Vadivelu SD, Meenakshi B. Triple procedure in blood-stained cornea of a hemophilia patient. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2021 Apr 23];59:88-90. Available from: https://www.tnoajosr.com/text.asp?2021/59/1/88/312300




  Introduction Top


Hemophilia A is an X-linked recessive disorder caused by the deficiency of clotting factor VIII characterized by hemorrhages which may occur spontaneously or after trauma [Table 1]. According to the US Centers for the Disease Control and Prevention, hemophilia A occurs in approximately 1 in 5000 live births.[1] Ophthalmic surgeries done on a case of hemophilia are rare. We wish to report the first case of penetrating keratoplasty (PKP) with pupillary membranectomy and cataractous lens removal with posterior chamber intraocular lens implantation in a case of hemophilia A. The surgery was done under the cover of antihemophilic factor.[2]
Table 1: Patient symptoms and levels of factor VIII according to severity of hemophilia A[1]

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  Case Report Top


A 16-year-old male came to our hospital with complaints of defective vision in the right eye (OD). He gave a history of ocular trauma to the right eye 9 months ago for which he took treatment elsewhere. He also gave a history of hemophilia Type A from birth. He had undergone anterior chamber paracentesis for traumatic hyphema elsewhere. Hyphema was not resolving due to hemophiliac status. The patient was managed conservatively with antiglaucoma medications and topical steroids. He had no other significant systemic illness. There was no family history of hemophilia. Examination of the right eye showed blood staining of cornea. Cataract was seen through peripheral clear cornea. The vision was perception of Light + and Projection of rays was intact. Other details were not made out due to blood staining of cornea [Figure 1]. There was no view in the fundus due to blood staining of cornea. Intraocular pressure in OD by Goldmann applanation tonometry was 8 mm Hg. Right eye B scan showed an intact posterior capsule with a clear vitreous cavity, and the retina was attached. Left eye examination was within the normal limits, and the uncorrected visual acuity (UCVA) was 6/6.
Figure 1: Preoperative picture showing blood staining of cornea

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Triple procedure was planned. Hematologist opinion was sought. Prothrombin time (PT) was 13 s, activated partial thromboplastin time (aPTT) was 57 s which was prolonged.[3] The activated partial thromboplastin time (PTT) test is used to evaluate the clotting factors of the intrinsic and common pathways of the clotting cascade by measuring the time (in seconds) it takes a clot to form after adding calcium and phospholipid emulsion to a plasma sample. After administering Factor VIII, aPTT becomes normal. The normal range of PT is 12.7–15.4 s, aPTT is 30–40 s, and PTT is 60–70 s.[3] The levels of Factor VIII in blood was 2 % and it was classified as a case of moderate hemophilia A.

Under peribulbar anesthesia, PKP with graft size 8.5 mm over 8.0 mm with pupillary membranectomy and cataractous lens removal with posterior chamber intraocular lens implantation (IOL) was done. Anti-hemophilic factor (AHF, Factor VIII) was given according to hematologist opinion [Table 2]. On post operative day 1, patient had OD vision of 4/60 NIG NIP. On POD 7, patient's UCVA was 6/60, and it improved with pinhole to 6/24. On POD 21 OD best-corrected visual acuity was 6/6P with-1.00Dcyl 140° [Figure 2].
Table 2: Dosage of drugs administered pre- and postsurgery for hemophilia A

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Figure 2: Postoperative picture showing clear graft, formed anterior chamber with pseudophakic reflex

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Fundus examination revealed a clear media with normal disc and vessels.


  Discussion Top


Hemostasis can be achieved in simple bleeding episodes of hemophilia A by the use of fresh frozen or lyophilized plasma. In the event of major trauma or surgical procedures sustained levels of 20%–30% of Factor VIII are necessary to provide hemostasis. Such levels are often impossible to achieve with the use of plasma, and attempts to maintain hemostasis with repeated infusions may lead to congestive heart failure owing to the resulting overexpansion of total plasma volume. Concentrated forms of factor VIII (antihemophilic factor, AHF) provide safer means of Factor VIII replacement.[4] HEMOFIL M, antihemophilic factor (Human), Method M, Monoclonal Purified (Hemophil M, Baxalta US Inc.,1700 Rancho Conejo Boulevard, Thousand Oaks, CA 91320, USA) is a sterile, nonpyrogenic, dried preparation of AHF. The half-life of HEMOFIL M administered to Factor VIII deficient patients has been shown to be 14.8 ± 3.0 h. The expected in vivo peak AHF level expressed as IU/dL of plasma or percentage of normal can be calculated by multiplying the dose administered per kg body weight (IU/kg) by two.[2] This calculation is based on the clinical finding by Abildgaard et al.[4] [Table 3].
Table 3: Recommended antihemophilic factor dosage schedule for Hemophilia A[2]*

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Bilateral cataract extraction with peripheral iridectomy without IOL implantation with retrobulbar anesthesia in hemophilia patient was reported by Rothkoff et al. in 1977.[5] In recent times IOL implantation, trabeculectomy and vitrectomy in hemophilia patients were reported by Jijina et al.[6] A case of traumatic hyphema in a patient with severe hemophilia A which was medically managed successfully is also reported by Belviranli et al.[7] Topical deferiprone has been reported to have the potential of speeding up the process of clearance of the blood stain from the cornea while avoiding further surgical complications.[8] A case of delayed suprachoroidal hemorrhage after PKP in a neonate as the first manifestation of hemophilia was also reported.[9] The diagnosis of hemophilia in an elderly patient made after noticing excessive intraoperative bleeding during attempted PKP which was not successful was also reported by Choi et al.[10]

Our case had a successful visual outcome due to preoperative planning. When cornea is removed, mechanical barrier to vitreous expansion is lost. Eyes undergoing PKP have increased risk of expulsive hemorrhage due to sudden globe decompression with an open sky communication with atmosphere.[11] Cataract removal during an open sky procedure is very risky and requires meticulous technique, and it has never been reported before in a hemophilia A patient.


  Conclusion Top


Hemophilia A patients in case of trauma or spontaneous hemorrhage are mostly managed medically and conservatively. Elective procedures are a rarity due to enigma and fear of complications. This case report emphasizes the fact that elective ophthalmic procedures in hemophilia A patients can be done safely with concentrated forms of Factor VIII and also with careful preoperative planning and coordination with the hematology team.

Declaration of patient consent

The authors certify that they had obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hemophilia A. National Hemophilia Foundation; 2014. Available from: https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Hemophilia-A. [Last accessed on 2019 Dec 18].  Back to cited text no. 1
    
2.
DailyMed - HEMOFIL M- Antihemophilic Factor Human Kit. Available from: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=b8953ff7-3bba-4a0b-a486-f26fb81f05d9. [Last accessed on 2019 Dec 18].  Back to cited text no. 2
    
3.
Partial Thromboplastin Time, Activated: Reference Range, Interpretation, Collection and Panels; 2019. Available from: https://emedicine.medscape.com/article/2085837-overview#a4. [Last accessed on 2019 Dec 20].  Back to cited text no. 3
    
4.
Abildgaard CF, Simone JV, Corrigan JJ, Seeler RA, Edelstein G, Vanderheiden J, et al. Treatment of hemophilia with glycine-precipitated factor VIII. N Engl J Med 1966;275:471-5.  Back to cited text no. 4
    
5.
Rothkoff L, Biedner B, Shoham K. Bilateral cataract: Extraction in classic haemophilia with retrobulbar anaesthesia and peripheral iridectomy. Br J Ophthalmol 1977;61:765-6.  Back to cited text no. 5
    
6.
Jijina F, Ghosh K, Madkaikar M, Mohanty D. Ophthalmic surgery in haemophilia. Haemophilia 2001;7:464-7.  Back to cited text no. 6
    
7.
Belviranli S, Ozkagnici A, Tokgoz H, Bitirgen G, Caliskan U. Traumatic hyphema in a patient with severe hemophilia A: Clinical features and management. European Journal of Ophthalmology 2019:1120672119856515. doi: 10.1177/1120672119856515. Epub ahead of print. PMID: 31187640.  Back to cited text no. 7
    
8.
Chan T, White A, Meades K, Bala C. Novel topical therapy for corneal blood staining. Clin Exp Ophthalmol 2017;45:416-8.  Back to cited text no. 8
    
9.
Al-Sharif E, AlEnezi SH, Sharif HA, Osman EA. Ocular bleeding in an undiagnosed hemophiliac neonate causing irreversible loss of vision: A case report with review of the literature. Eur J Ophthalmol 2020;30:NP62–NP65. doi: 10.1177/1120672119841188. Epub 2019 Apr 2. PMID: 30938185.  Back to cited text no. 9
    
10.
Choi DM, Goldstein MH, Driebe WT Jr. Diagnosis of hemophilia made after intraoperative bleeding during attempted penetrating keratoplasty in an elderly patient. The CLAO Journal: Official Publication of the Contact Lens Association of Ophthalmologists, Inc, CLAO J 2001;27:53-4.  Back to cited text no. 10
    
11.
Brightbill FS. Corneal Surgery: Theory, Technique and Tissue. Mosby, Inc.; Westline Industrial Drive St. Louis, Missouri 63146. 1999. 942 p. 11830  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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