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Multiple extractions in a patient with factor VII deficiency
382
ORAL
illary tooth socket: An intraosseous leiomyoma presenting as an odontogenic lesion. South Med J 69:493. 1976 49. Combos F. Mazzarella G, De Rosa G: A case of vascular leiomyoma of the mouth floor. Arch Stomatol (Napoli) 17:205. 1976
LEIOMYOMA
50. Goldblatt L. Edesess R: Central leiomyoma of the mandible. Oral Surg 43:5Y I. 1977 5 I. Nakamura H. Kanai M. Takita M: Two cases of angiomyoma of the palate region. Tsurumi Shigaru 4: 133. 1978 52. Ioannidis H. Mollegraft F: Leiomyoma of the cheek. Dentist 2:115. 1982
J Oral Maxillofac Surg 43:382-394. 1985
Multiple YASUNORI
Extractions in a Patient with Factor VII Deficiency
SUMI, DDS,* MICHIO SHIKIMORI, DDS,* TOSHIO KANEDA, DDS,* AND TADASHI KITAJIMA, DDSt
The first case of congenital factor VII deficiency was reported by Alexander et al. in 1951.’ It is a relatively rare disorder compared with hemophilia A or von Willebrand’s disease, and there have been only a few reports of this deficiency in the oral surgery literature.2 In this study, a case of factor VII deficiency is presented, and the successful management of dental extractions using a prothrombin complex concentrate containing factors II, VII, IX, and X is discussed.
normal range. At this time. the patient’s youngest brother, who had been born of a consanguineous marriage. also received a diagnosis of factor VII deficiency. They both had considerable prolongation of one stage FAMILY
PEDIGREE
Report of Case A 4%year-old man came to the oral surgery clinic at Nagoya University Hospital on September 27, 1982, for dental treatment after a long history of odontogenic pain in the upper right second premolar, the upper right first molar, and the upper left third molar. Physical examination revealed no abnormalities, and there were no purpura or petechiae. Clinical and radiographic examinations revealed six severely carious teeth with bone resorption requiring extraction. The patient’s neglect of dental hygiene and his dentists’ fear of bleeding after dental treatment may have been directly contributory to the poor condition of his teeth. The patient had experienced recurrent epistaxis and easy bruising until he was 20 years old, when he started to become less hemorrhagic. At the age of 46, he was referred to the hematology clinic at Nagoya University Hospital for evaluation of his bleeding tendencies because dental treatment had become necessary. A diagnosis of factor VII deficiency was made by a hemostatic test.3 The levels of other clotting factors were within the * Department of Oral Surgery,
School
of Medicine.
Male,Female
n q q q
I 3
Nagoya
University, Nagoya, Japan. i The Second Department of Oral Surgery. Gifu College of Dentistry, Gifu, Japan. Address correspondence and reprint requests to Dr. Sumi: Department of Oral Surgery, School of Medicine, Nagoya University, 6.5 Tsuruma-cho, Showa-ku, Nagoya, 466. Japan.
I
I
TIMEAFTER FIGURE ence 3).
1 (ub~re).
FIGURE 2 (below~). prothrombin complex
Family
l tested abnormal Q tested normal @ deceased a~ not tested propor1ta /
I 6
1 , 30 hours
,
INFUSION
pedigree
of patient
Plasma factor VII levels concentrate infusion.
(see
refer-
in response
to
383
SUM1 ET AL.
prothrombin time, whereas activated partial thromboplastin time and Stypven clotting time were within normal limits. Their factor VII levels were 5.2% (patient) and 6.0% (youngest brother), respectively. Including these patients, 11 members of the family were investigated. Three had normal factor VII activity and 6 had 36-54% of the normal level. They were thought to be heterozygotes (Fig. 1).3 The hemostatic tests at admission are summarized in Table 1. They showed that the prothrombin time was prolonged and that the activated partial thromboplastin time was slightly decreased. The factor VII level was 9.4% at this time. The level of antithrombin III was within the normal range. Results of liver function tests and urine analysis were normal. The patient was admitted to Nagoya University Hospital on October 5. 1982, and three days later he underwent routine surgical removal of six teeth under local anesthesia. Because some patients with factor VII deficiency have had extractions without replacement therapy, no preoperative therapy was given. Although severe bleeding was not observed, moderate bleeding resulted, and the generated clots were fragile. Therefore, just after the operation, 30 ml of prothrombin complex concentrate, which contained 1,200 units of factor VII, was infused. The factor VII activity reached its peak (31%) 30 minutes after infusion and then gradually fell to the preinfusion level after 30 hours (Fig. 2). According to these data, the half life of factor VII in vivo was estimated to be about five hours. Before the operation, the antithrombin III level was checked to prevent adverse effects of prothrombin complex concentrate. After infusion, oozing was minimum, and it ceased completely within one hour. Firm clots were noted, but six hours later they developed into large masses (Fig. 3). These separated during the next 24 hours, and then healing progressed satisfactorily and uneventfully. The patient was discharged six days after the extractions, and no abnormality was observed. Discussion
Almost all the patients with factor VII deficiency reported in the literature have shown a mild to moderately severe hemorrhagic diathesis.2-4 The symptoms reported most frequently were epistaxis, easy bruising, and prolonged bleeding time after dental extraction. In contrast, several patients with factor VII deficiency have undergone surgical procedures, such as appendectomy or dental extractions,
Table 1.
Admission Hemostatic Tests Tests
Patient
Normal Range
Platelet count (Fonio. lo3 mm3) Prothrombin time (set) Activated partial thromboplastin time Csec) Fibrinogen (mgidl) Prothrombin (%,) Antithrombin 111(%) Plasminogen (%)
388 24.6
190-240 13.2
31.2 209 94 109 124
48.0 200-400 80-120 80- 120 80- 120
FIGURE 3. large mass.
Six hours after extraction,
the clot has formed a
without transfusion, and had no bleeding problems.3,4 However, profuse bleeding after routine dental extraction is not uncommon, so one cannot expect a freedom from bleeding after dental extraction. Because whole blood or fresh plasma contains a low level of factor VII, and the total infusion volume is high when either is used for replacement therapy, the infusion of factor VII concentrate or prothrombin complex concentrate containing large amounts of factor VII seems advisable. The level required for local hemostasis after dental extraction varies in relation to local factors such as wound size, inflammation, and apical lesion. In recent years prothrombin complex concentrate has been used successfully not only for treating patients with hemophilia B but also for those having liver disease with decreased multiple clotting factors, or with hemophilia A patients who have developed inhibitory antibody to factor VIII as a means of bypassing the normal pathway through factor VIII activation, Unfortunately, the concentrate has serious side effects, with complications such as venous thrombosis, pulmonary embolism, myocardial infarction, and disseminated intravascular coagulation. j-’ The cause of the hypercoagulable state after the infusion of prothrombin complex concentrate is not fully understood. It is clear that although the concentrate is useful and even life saving, it must be administered with the greatest care. Checking the antithrombin III level before infusion is indispensable. Summary
Multiple extractions were carried out in a patient with factor VII deficiency treated with prothrombin complex concentrate. Hemostasis and healing were completely satisfactory. The side effects of prothrombin complex concentrate are discussed.
384
FACTOR VII DEFICIENCY
References
4. Hall CA. et al: A clinical and family study of hereditary proconvertin (factor VII) deficiency. Am J Med 37: 172, 1964 5. Marassi A. et al: Thromboembolism following prothrombin complex concentrates and major surgery in severe liver disease. Thromb Haemostas (Stuttgl 39:248, 1978 6. Fuerth JH. Mahrer P: Myocardial infarction after factor IX therapy. JAMA 245:1455. 1981 7. Campbell EW, et al: Therapy with factor IX concentrate resulting in DIC and thromboembolic phenomena. Transfusion 18:94. 1978
1. Alexander B, et al: Congenital SPCA deficiency: A hitherto unrecognized coagulation defect with hemorrhage rectified by serum and serum fraction. J Clin Invest 30:596. 1951 2. Perhavec JC, Goldberg JS: Management of a patient with factor VII deficiency. Oral Surg 50: 17, 1980 3. Takamatsu J et al: A family of congenital factor VII deficiency. Japan J Clin Hematol 21:834. 1980
J Oral Maxillofac 43364-385,
Surg
1995
Giant Submandibular VIJAY NARAYNSINGH,
More than 80% of salivary calculi occur in the submandibular gland or its duct.*,2 These may be single or multiple but are usually less than 3 cm long. Giant sialoliths are very uncommon. A case of an extremely large submandibular sialolith is reported here.
Gland Calculi FRCS
and gland inflammation. Since the calculi are usually symptomatic, patients often present to the clinician long before the stone becomes very large. This patient had no pain, although he had palpated the mass three years before and noticed it in-
Report of Case In 1979, a 28-year-old East Indian man presented with a hard left submandibular swelling (Fig. 1). When he had first noticed it three years before, it had been much smaller. Though painless, it had gradually increased in size, and he sought medical attention for this reason. On examination, there was a nontender, stony-hard swelling in the left submandibular area. Another large mass could be palpated bimanually, immediately anterior to this. Radiographs confirmed the presence of two submandibular calculi (Fig. 2). With the patient under general anesthesia, a curved incision was made over the cervical mass. There were no signs of acute inflammation, but the lingual nerve, stretched over the submandibular swelling, was very adherent to it. By careful dissection, the nerve was preserved, and the gland and stones were excised. The patient made an uneventful recovery with good preservation of lingual nerve function.
Discussion Salivary calculi are found more commonly in the duct than in the gland parenchyma.3 Thus, they frequently produce recurrent bouts of duct obstruction FIGURE 1 (above, left). by large calculi. Received from the General Hospital, Port-of-Spain, Trinidad, West Indies. Address correspondence and reprint requests to Dr. Naraynsingh.
FIGURE 2 (above, right). mandibular calculi.
Left submandibular
swelling caused
Radiograph showing two giant sub-
FIGURE 3 (below). Submandibular calculi after excision. Rule measurement is shown in inches.
ORAL
illary tooth socket: An intraosseous leiomyoma presenting as an odontogenic lesion. South Med J 69:493. 1976 49. Combos F. Mazzarella G, De Rosa G: A case of vascular leiomyoma of the mouth floor. Arch Stomatol (Napoli) 17:205. 1976
LEIOMYOMA
50. Goldblatt L. Edesess R: Central leiomyoma of the mandible. Oral Surg 43:5Y I. 1977 5 I. Nakamura H. Kanai M. Takita M: Two cases of angiomyoma of the palate region. Tsurumi Shigaru 4: 133. 1978 52. Ioannidis H. Mollegraft F: Leiomyoma of the cheek. Dentist 2:115. 1982
J Oral Maxillofac Surg 43:382-394. 1985
Multiple YASUNORI
Extractions in a Patient with Factor VII Deficiency
SUMI, DDS,* MICHIO SHIKIMORI, DDS,* TOSHIO KANEDA, DDS,* AND TADASHI KITAJIMA, DDSt
The first case of congenital factor VII deficiency was reported by Alexander et al. in 1951.’ It is a relatively rare disorder compared with hemophilia A or von Willebrand’s disease, and there have been only a few reports of this deficiency in the oral surgery literature.2 In this study, a case of factor VII deficiency is presented, and the successful management of dental extractions using a prothrombin complex concentrate containing factors II, VII, IX, and X is discussed.
normal range. At this time. the patient’s youngest brother, who had been born of a consanguineous marriage. also received a diagnosis of factor VII deficiency. They both had considerable prolongation of one stage FAMILY
PEDIGREE
Report of Case A 4%year-old man came to the oral surgery clinic at Nagoya University Hospital on September 27, 1982, for dental treatment after a long history of odontogenic pain in the upper right second premolar, the upper right first molar, and the upper left third molar. Physical examination revealed no abnormalities, and there were no purpura or petechiae. Clinical and radiographic examinations revealed six severely carious teeth with bone resorption requiring extraction. The patient’s neglect of dental hygiene and his dentists’ fear of bleeding after dental treatment may have been directly contributory to the poor condition of his teeth. The patient had experienced recurrent epistaxis and easy bruising until he was 20 years old, when he started to become less hemorrhagic. At the age of 46, he was referred to the hematology clinic at Nagoya University Hospital for evaluation of his bleeding tendencies because dental treatment had become necessary. A diagnosis of factor VII deficiency was made by a hemostatic test.3 The levels of other clotting factors were within the * Department of Oral Surgery,
School
of Medicine.
Male,Female
n q q q
I 3
Nagoya
University, Nagoya, Japan. i The Second Department of Oral Surgery. Gifu College of Dentistry, Gifu, Japan. Address correspondence and reprint requests to Dr. Sumi: Department of Oral Surgery, School of Medicine, Nagoya University, 6.5 Tsuruma-cho, Showa-ku, Nagoya, 466. Japan.
I
I
TIMEAFTER FIGURE ence 3).
1 (ub~re).
FIGURE 2 (below~). prothrombin complex
Family
l tested abnormal Q tested normal @ deceased a~ not tested propor1ta /
I 6
1 , 30 hours
,
INFUSION
pedigree
of patient
Plasma factor VII levels concentrate infusion.
(see
refer-
in response
to
383
SUM1 ET AL.
prothrombin time, whereas activated partial thromboplastin time and Stypven clotting time were within normal limits. Their factor VII levels were 5.2% (patient) and 6.0% (youngest brother), respectively. Including these patients, 11 members of the family were investigated. Three had normal factor VII activity and 6 had 36-54% of the normal level. They were thought to be heterozygotes (Fig. 1).3 The hemostatic tests at admission are summarized in Table 1. They showed that the prothrombin time was prolonged and that the activated partial thromboplastin time was slightly decreased. The factor VII level was 9.4% at this time. The level of antithrombin III was within the normal range. Results of liver function tests and urine analysis were normal. The patient was admitted to Nagoya University Hospital on October 5. 1982, and three days later he underwent routine surgical removal of six teeth under local anesthesia. Because some patients with factor VII deficiency have had extractions without replacement therapy, no preoperative therapy was given. Although severe bleeding was not observed, moderate bleeding resulted, and the generated clots were fragile. Therefore, just after the operation, 30 ml of prothrombin complex concentrate, which contained 1,200 units of factor VII, was infused. The factor VII activity reached its peak (31%) 30 minutes after infusion and then gradually fell to the preinfusion level after 30 hours (Fig. 2). According to these data, the half life of factor VII in vivo was estimated to be about five hours. Before the operation, the antithrombin III level was checked to prevent adverse effects of prothrombin complex concentrate. After infusion, oozing was minimum, and it ceased completely within one hour. Firm clots were noted, but six hours later they developed into large masses (Fig. 3). These separated during the next 24 hours, and then healing progressed satisfactorily and uneventfully. The patient was discharged six days after the extractions, and no abnormality was observed. Discussion
Almost all the patients with factor VII deficiency reported in the literature have shown a mild to moderately severe hemorrhagic diathesis.2-4 The symptoms reported most frequently were epistaxis, easy bruising, and prolonged bleeding time after dental extraction. In contrast, several patients with factor VII deficiency have undergone surgical procedures, such as appendectomy or dental extractions,
Table 1.
Admission Hemostatic Tests Tests
Patient
Normal Range
Platelet count (Fonio. lo3 mm3) Prothrombin time (set) Activated partial thromboplastin time Csec) Fibrinogen (mgidl) Prothrombin (%,) Antithrombin 111(%) Plasminogen (%)
388 24.6
190-240 13.2
31.2 209 94 109 124
48.0 200-400 80-120 80- 120 80- 120
FIGURE 3. large mass.
Six hours after extraction,
the clot has formed a
without transfusion, and had no bleeding problems.3,4 However, profuse bleeding after routine dental extraction is not uncommon, so one cannot expect a freedom from bleeding after dental extraction. Because whole blood or fresh plasma contains a low level of factor VII, and the total infusion volume is high when either is used for replacement therapy, the infusion of factor VII concentrate or prothrombin complex concentrate containing large amounts of factor VII seems advisable. The level required for local hemostasis after dental extraction varies in relation to local factors such as wound size, inflammation, and apical lesion. In recent years prothrombin complex concentrate has been used successfully not only for treating patients with hemophilia B but also for those having liver disease with decreased multiple clotting factors, or with hemophilia A patients who have developed inhibitory antibody to factor VIII as a means of bypassing the normal pathway through factor VIII activation, Unfortunately, the concentrate has serious side effects, with complications such as venous thrombosis, pulmonary embolism, myocardial infarction, and disseminated intravascular coagulation. j-’ The cause of the hypercoagulable state after the infusion of prothrombin complex concentrate is not fully understood. It is clear that although the concentrate is useful and even life saving, it must be administered with the greatest care. Checking the antithrombin III level before infusion is indispensable. Summary
Multiple extractions were carried out in a patient with factor VII deficiency treated with prothrombin complex concentrate. Hemostasis and healing were completely satisfactory. The side effects of prothrombin complex concentrate are discussed.
384
FACTOR VII DEFICIENCY
References
4. Hall CA. et al: A clinical and family study of hereditary proconvertin (factor VII) deficiency. Am J Med 37: 172, 1964 5. Marassi A. et al: Thromboembolism following prothrombin complex concentrates and major surgery in severe liver disease. Thromb Haemostas (Stuttgl 39:248, 1978 6. Fuerth JH. Mahrer P: Myocardial infarction after factor IX therapy. JAMA 245:1455. 1981 7. Campbell EW, et al: Therapy with factor IX concentrate resulting in DIC and thromboembolic phenomena. Transfusion 18:94. 1978
1. Alexander B, et al: Congenital SPCA deficiency: A hitherto unrecognized coagulation defect with hemorrhage rectified by serum and serum fraction. J Clin Invest 30:596. 1951 2. Perhavec JC, Goldberg JS: Management of a patient with factor VII deficiency. Oral Surg 50: 17, 1980 3. Takamatsu J et al: A family of congenital factor VII deficiency. Japan J Clin Hematol 21:834. 1980
J Oral Maxillofac 43364-385,
Surg
1995
Giant Submandibular VIJAY NARAYNSINGH,
More than 80% of salivary calculi occur in the submandibular gland or its duct.*,2 These may be single or multiple but are usually less than 3 cm long. Giant sialoliths are very uncommon. A case of an extremely large submandibular sialolith is reported here.
Gland Calculi FRCS
and gland inflammation. Since the calculi are usually symptomatic, patients often present to the clinician long before the stone becomes very large. This patient had no pain, although he had palpated the mass three years before and noticed it in-
Report of Case In 1979, a 28-year-old East Indian man presented with a hard left submandibular swelling (Fig. 1). When he had first noticed it three years before, it had been much smaller. Though painless, it had gradually increased in size, and he sought medical attention for this reason. On examination, there was a nontender, stony-hard swelling in the left submandibular area. Another large mass could be palpated bimanually, immediately anterior to this. Radiographs confirmed the presence of two submandibular calculi (Fig. 2). With the patient under general anesthesia, a curved incision was made over the cervical mass. There were no signs of acute inflammation, but the lingual nerve, stretched over the submandibular swelling, was very adherent to it. By careful dissection, the nerve was preserved, and the gland and stones were excised. The patient made an uneventful recovery with good preservation of lingual nerve function.
Discussion Salivary calculi are found more commonly in the duct than in the gland parenchyma.3 Thus, they frequently produce recurrent bouts of duct obstruction FIGURE 1 (above, left). by large calculi. Received from the General Hospital, Port-of-Spain, Trinidad, West Indies. Address correspondence and reprint requests to Dr. Naraynsingh.
FIGURE 2 (above, right). mandibular calculi.
Left submandibular
swelling caused
Radiograph showing two giant sub-
FIGURE 3 (below). Submandibular calculi after excision. Rule measurement is shown in inches.