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Table of Contents
REPORT
Year : 2010  |  Volume : 12  |  Issue : 1  |  Page : 18-21

KTP laser in hereditary hemorrhagic telangiectasia induced epistaxis


1 Resident of Otolaryngology Head and Neck Surgery, College of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
2 Assistant Professor and Consultant of Otolaryngology, Head and Neck Surgery, Department of Otolaryngology, Head and Neck Surgery.College of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia

Date of Web Publication24-Dec-2019

Correspondence Address:
MBBS Faisal T Zawawi
P.O. Box 10659 Jeddah 21443
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-8491.273968

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  Abstract 


Objective: To demonstrate the effectiveness of Potassium-titanyl-phosphate (KTP) laser therapy in hereditary hemorrhagic telangiectasia (HHT) induced nasal bleeding.
Case Report: 62 years old gentleman with HHT induced recurrent severe epistaxis treated with sessions of KTP laser.
Conclusion: KTP laser should be considered for treating patients with moderate to severe epistaxis. Keywords: Hereditary hemorrhagic telangiectasia, epistaxis, KTP, Laser.

Keywords: Hereditary hemorrhagic telangiectasia, epistaxis, KTP, Laser.


How to cite this article:
Zawawi FT, Al-Ghamdi KB. KTP laser in hereditary hemorrhagic telangiectasia induced epistaxis. Saudi J Otorhinolaryngol Head Neck Surg 2010;12:18-21

How to cite this URL:
Zawawi FT, Al-Ghamdi KB. KTP laser in hereditary hemorrhagic telangiectasia induced epistaxis. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2010 [cited 2022 Dec 2];12:18-21. Available from: https://www.sjohns.org/text.asp?2010/12/1/18/273968




  Introduction Top


Hereditary hemorrhagic telangiectasia (HHT), also know as Rendu-Osler-Weber syndrome is a systemic vasculopathy, which is inherited as autosomal-dominant trait. Different mutations have been found on two gene loci so far. Mutations of endoglin can be found on 9q34.1 (HHT-1) [1],[2] while ACVRL-1 mutations situated on 12q11-q14 (HHT-2) [2]. The most recent detection was recognized on 18q21.1 [2],[3]. The prevalence varies between 1:2351 to 1:39,216 in individuals in different regions [4],[5]. It has recently been formally characterized by Curacao criteria: Epistaxis, telangiectasias, visceral lesions (such as arterio-venous malformations), and a first-degree relative with HHT typify this disorder [6],[7]. Telangientases can principally be found anywhere but predilection sites are the mucous membranes of the nose and gastrointestinal tract and of the outer skin of the face (especially the lower lip) and hands. Epistaxis is the most common symptom of the disease that occurs in 78% to 96% of patients [5],[8]. Nosebleeds often start around puberty and tend to worsen over time leading to anemia and requiring multiple procedures and blood transfusions [5],[7],[9]. Bleeding of the lower gastrointestinal tract is often less obvious but becomes more severe with age [10].

Large vascular malformations (VM) of visceral organs may initially be silent. Pulmonary VM (PVM) can sometimes allow a septic paradoxical embolism that causes visceral infarction and abscesses. A preventative measure is the percutaneous transcatheter embolotherapy of the PVM [11]. Cerebral VM (CVM) is responsible for hemorrhagic strokes with often disabling consequences. Therapeutic options are transcatheter embolotherapy, stereotactic radiotherapy, or surgery [11],[12]. Hepatic VM (HVM) can lead to hepatic cirrhosis and high output heart failure with abdominal pain due to relative ischemia [13]. The overall mortality of HHT patients seems to be elevated, especially in patients younger than 60 years of age [5],[14]. Currently, there is no definitive standard treatment for HHT-associated epistaxis. Numerous treatments have been used in the past, but the recurrent nature of his disease makes it difficult to treat. Monopolar and chemical cautery have been avoided because of the possibility of septal perforations or synechiae formation [7]. Bipolar electrocautery has been used successfully in mild to moderate epistaxis cases [7]. Recently, topical hormonal treatments such as topically applied estrogen haven been observed to decrease the frequency of epistaxis 7, 15, 16]. Various laser therapy modalities have been shown their effectiveness in ablating intranasal telangiectasias [7],[15].

This case report demonstrates the effectiveness of using potassium-titanyl-phosphate (KTP) laser in the treatment of a case of moderate to sever HHT-associated recurrent epistaxis.


  Case Report Top


A 62 years old gentleman who was diagnosed to have HHT 40 years ago, presented to the Otolaryngology clinic at King Abdulaziz University Hospital, Jeddah, Saudi Arabia complaining from recurrent moderate to sever epistaxis attacks that required him to frequently visit the emergency room (ER) at his hometown. Usually these visits end up with blood transfusion. Lately, these attacks became more frequent and severe.

He bled more from the right nostril. The last attack was a day prior to his clinic visit. He had to go to the ER and receive one unit of whole blood (Packed RBCs). He has a brother with a milder form of the disease. On examination the patient was vitally stable and his oxygen saturation was well on room air. He was pale but not jaundiced. An above elbow amputated right arm was noted. He had obvious telagiectasias on the lips, tongue and nasal mucosa (both septum and lateral walls). The lesions in the right nasal cavity had fresh blood clots on them. His blood work up showed low hemoglobin (5.2 g/dL) and good kidney function. His coagulation profile came out normal. The plan was to admit him for thorough investigation and management with the assistance of the hematology team. On admission he received blood transfusion and iron supplement. Over the first few nights he had minimal recurrent blood oozing from his right nasal cavity. He had one episode of sever epistaxis from the right nasal cavity which required nasal packed using Merocel Nasal Pack (Merocel ®) and was put on Amoxicillin Clavulanate 1.2g intravenously every 8 hours. CT scan of the nasal cavity and paranasal sinuses showed no evidence of any other cause or site for his bleeding. Angiography of the carotids showed abnormal capillary bush noted in the nose fed by the facial and internal maxillary arteries bilaterally. Capillary bush was noted on the lips as well.

The patient was taken to the operation room for laser therapy. Intra-operatively, the nasal pack was removed and bleeding was controlled using bipolar cauterization. Endoscopic Potassium-titanyl-phosphate (KTP) laser vaporization was used on the telangiectasias. Laser therapy was performed on the right nasal cavity only. KTP 532 nm was used on a continuous mode powered at level 4 and 2707 Joules were used. Post operatively he was kept in the hospital for observation and during this period he did not develop any episode of epistaxis. He was put on regular nasal ointments and Paracetamol 1g orally as needed in addition to the antibiotic. Nasal aeration and breathing were good as well. Pain was very well tolerated. The patient was discharged with just the nasal ointment and ferrous sulfate tablets to be followed up in the Out Patients Department.

During his first 6 months of follow up, the patient did not suffer from any attack of epistaxis. On the 6th month follow up appointment, nasal endoscopy showed decrease in the telagiectasias in the right nasal cavity (the side that received the laser therapy). No synechiea, crusts or septal perforation were noted. The patient was satisfied with the outcome.

One-year post laser therapy he visited the clinic complaining from mild recurrent epistaxis from both nasal cavities. He also noted black stool. The patient was extremely pale but not jaundiced. Telangiectasias were noted bilaterally on nasal endoscopy. The patient was admitted to the surgical ward for further evaluation and another session of laser therapy to his nasal cavity. Upon admission he was evaluated by the hematology and medical teams and prescribed PRBC transfusion to stabilize him hemodynamically (Hemoglobin was 3.2 g/dl). Echocardiography was performed and was normal.

An upper gastro-intestinal endoscopy was performed by the endoscopic surgeon that revealed multiple telangiectasias in the stomach and the duodenum [Figure 1]. This was followed by a session of Argon Plasma Coagulation (APC). He had a total of 3 sessions of APC during his hospital admission.
Figure 1: Upper Gastro-intestinal Endoscopy showing Telangiectasia in the beginning of the duodenum.

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Afterwards he was taken to the operating room for a session of laser therapy to his nasal cavity. Intraoperative, sever bleeding was noted bilaterally and was controlled by bipolar cauterization [Figure 2]. This was followed by laser therapy to both nasal cavities. KTP 532 nm was used on a continuous mode powered at level 4 and 6534 Joules were used [Figure 3], [Figure 4]. The patient stayed for a week after surgery for observa-tion. During this period he had his hemoglobin checked daily and it was stable at 9.8 g/dl without the need for blood transfusion. He was then discharged on oral analgesics and Iron supplements. His second hospital course lasted for 39 days in which he had received a sum of 40 units of PRBC under the supervision of both medical and hematology teams.
Figure 2: Nasal Endoscopy at the beginning of the therapy showing bleeding from the nasal cavity.

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Figure 3: Nasal Endoscopy showing the KTP laser probe during the therapy session..

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Figure 4: Nasal Endoscopy at the end of the KTP Laser session showing the nasal cavity after controlling the bleeding with KTP Laser

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  Discussion Top


Patients with HHT typically require long-standing treatment regimens to control their epistaxis. Often, the blood loss from recurrent epistaxis experienced by these patients demands lifelong iron supplementations and occasional blood transfusion. In addition to enduring medical costs and potential health complications from the disease, HHT patients suffer from social hindrances from their often-intractable nosebleeds. Because medical treatment of HHT has not yet provided patients with adequate measures to avoid bleeding, surgical therapy has been the mainstay of treatment [7]. The surgical treatment varies from simple measures, such as focused cauterization and packing, to more complex procedures as in septal dermoplasty (SDP) and closure of nasal airway (Young’s procedure). Currently, laser ablation of telagiectasias has been considered a modality of management. While HHT patients may require repeated laser sessions, the technique is associated with relatively minimal morbidity[7]. A variety of different lasers, including CO2, KTP, Nd: YAG, and similar tools like the argon beam coagulator, have established efficacy in ablating HHT-associated telngiectasias [7]. Harvey and colleagues reported their success in managing such cases with combined measures of KTP laser and SDP. They reported that the rate of KTP sessions after SDP has decreased from 1.83 to 0.78 [17]. While these results are impressive, Jovancevic and Mitrovic have concluded that laser therapy alongside 0.1% estriol ointment is an effective treatment for recurrent HHT-associated epistaxis [18].

Our decision to use KTP laser instead of other modalities was for its capability to ablate the specific lesion with minimal harm to the surrounding tissues. Furthermore, the ability of KTP laser in effectively reducing the frequency of epistaxis in patients HHT has been well documented in the literature [19]. Bipolar electrocautery was initially needed to control active bleeding and to give a clearer endoscopic view for performing laser ablation [7].

Although SDP has good results in the literature the decision to go for SDP in this patient would have been premature for this was the first interventional medicine this patient has gone through. Supporting our decision is the study of Werner that demonstrated the effectiveness of using laser therapy in treating HHT-associated epistaxis. In addition, they concluded that initial laser therapy and long-term nasal ointments is the mainstay of management and that SDP should only be reserved for cases which fail the mentioned treatment [20]. Another important point is the approach to HHT patients. This case illustrates that the proper evaluation of HHT patients is a thorough multi-system screen. A team of otolaryngologists, hematologists, gastroenterol- ogists, surgeons as well as urologists is very well needed. In this patient’s second visit the cause of his severe anemia and the need for multiple blood transfusions was not related to his nasal bleeds rather to his gastro-intestinal involvement of the disease. The period between the first session of KTP laser therapy and the return of his nosebleeds was almost a year (11 months). During this period patient was able to maintain a decent quality of life without the need to visit the ER for nosebleeds.


  Conclusion Top


This report shows that KTP laser is a safe technique to be used in patients suffering from recurrent epistaxis. We recommend its use as a first line management for patients with HHT-associated moderate to sever epistaxis.



 
  References Top

1.
McAllister KA, Grogg KM, Johnson DW, et al. Endoglin, a TGF-beta binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1. Nat Genet. 1994;8(4):345-51.  Back to cited text no. 1
    
2.
Folz BJ, Werner JA. Contact endoscopy of the nose in patients with Rendu-Osler-Weber syndrome. Auris Nasus Larynx. 2007;34(1):45-8.  Back to cited text no. 2
    
3.
Gallione CJ, Repetto GM, Legius E, et al. A combined syndrome of juvenile polyposis and hereditary haemor-rhagic telangiectasia associated with mutations in MADH4 (SMAD4). Lancet. 2004;363(9412):852-9.  Back to cited text no. 3
    
4.
Guttmacher AE, Marchuk DA, White RI, Jr. Hereditary hemorrhagic telangiectasia. N Engl J Med. 1995;333(14):918-24.  Back to cited text no. 4
    
5.
Geisthoff UW, Heckmann K, D’Amelio R, et al. Health-related quality of life in hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg. 2007;136(5):726-33.  Back to cited text no. 5
    
6.
Shovlin CL, Guttmacher AE, Buscarini E, et al. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet. 2000;91(1):66-7.  Back to cited text no. 6
    
7.
Ghaheri BA, Fong KJ, Hwang PH. The utility of bipolar electrocautery in hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg. 2006;134(6):1006-9.  Back to cited text no. 7
    
8.
Geisthoff UW, Schneider G, Fischinger J, Plinkert PK. [Hereditary hemorrhagic telangiectasia (Osler’s disease). An interdisciplinary challenge]. HNO. 2002;50(2):114-28.  Back to cited text no. 8
    
9.
Levine CG, Ross DA, Henderson KJ, Leder SB, White RI, Jr. Long-term complications of septal dermoplasty in patients with hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg. 2008;138(6):721-4.  Back to cited text no. 9
    
10.
Longacre AV, Gross CP, Gallitelli M, Henderson KJ, White RI, Jr., Proctor DD. Diagnosis and management of gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia. Am J Gastroenterol. 2003;98(1):59-65.  Back to cited text no. 10
    
11.
White RI, Jr. Pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia: embolotherapy using balloons and coils. Arch Intern Med. 1996;156(22):2627-8.  Back to cited text no. 11
    
12.
Matsubara S, Mandzia JL, ter Brugge K, Willinsky RA, Faughnan ME. Angiographic and clinical characteristics of patients with cerebral arteriovenous malformations associated with hereditary hemorrhagic telangiectasia. AJNR Am J Neuroradiol. 2000;21(6):1016-20.  Back to cited text no. 12
    
13.
Garcia-Tsao G, Korzenik JR, Young L, et al. Liver disease in patients with hereditary hemorrhagic telangiectasia. N Engl J Med. 2000;343(13):931-6.  Back to cited text no. 13
    
14.
Kjeldsen AD, Vase P, Green A. [Hereditary hemorrhagic telangiectasia. A population-based study on prevalence and mortality among Danish HHT patients]. Ugeskr Laeger. 2000;162(25):3597-601.  Back to cited text no. 14
    
15.
Bergler W, Sadick H, Gotte K, Riedel F, Hormann K. Topical estrogens combined with argon plasma coagulation in the management of epistaxis in hereditary hemorrhagic telangiectasia. Ann Otol Rhinol Laryngol. 2002;111(3 Pt 1):222-8.  Back to cited text no. 15
    
16.
Mahoney EJ, Shapshay SM. Nd-YAG laser photocoagulation for epistaxis associated with hereditary hemorrhagic telangiectasia. Laryngoscope. 2005;115(2):373-5.  Back to cited text no. 16
    
17.
Harvey RJ, Kanagalingam J, Lund VJ. The impact of septodermoplasty and potassium-titanyl-phosphate (KTP) laser therapy in the treatment of hereditary hemorrhagic telangiectasia-related epistaxis. Am J Rhinol. 2008;22(2):182-7.  Back to cited text no. 17
    
18.
Jovancevic L, Mitrovic SM. [Epistaxis in patients with hereditary hemorrhagic teleangiectasia]. Med Pregl. 2006;59(9-10):443-9.  Back to cited text no. 18
    
19.
Vickery CL, Kuhn FA. Using the KTP/532 laser to control epistaxis in patients with hereditary hemorrhagic telangiectasia. South Med J. 1996;89(1):78-80.  Back to cited text no. 19
    
20.
Werner JA, Geisthoff UW, Lippert BM, Rudert H. [Treatment of recurrent epistaxis in Rendu-Osler-Weber disease]. HNO. 1997;45(9):673-81.  Back to cited text no. 20
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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