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J Meikai Dent Med 362, 205 216, 2007 205 1 Dubin-Johnson 1 1 1 2! 2 2 2 2 2 1 1 2 Dubin-Johnson 23 1 2003 23 2003 10 12 Dubin-Johnson A Case of Severe Skeletal Mandibular Protrusion Indicating Simultaneous Corrective Surgery of the Maxilla and Mandible Including Discussion on Perioperative Management of the Dubin-Johnson Syndrome Satoru KOBAYASHI 1, Kimiko YASUI 1, Mikiko MANO 1, Hisao SHIGEMATSU 2, Una CHO 2, Keiichi HOSOKAWA 2, Hizuru MIYAMOTO 2, Seiji SUZUKI 2, Hideaki SAKASHITA 2 and Haruhide KANEGAE 1 1 Division of Orthodontics, Department of Human Development & Fostering, Meikai University School of Dentistry 2 Division of Oral and Maxillofacial Surgery, Department of Therapeutic & Diagnostic Sciences, Meikai University School of Dentistry Abstract : Herein, we present a favorable outcome obtained following corrective surgery for a patient with jaw deformities complicated by the Dubin-Johnson syndrome. In addition to summarizing the case, we discuss the perioperative management of patients complicated by constitutional jaundice. The patient was a male 23 years and 1 month of age. Although he had been aware of his mandibular protrusion for many years, he did not seek any medical assistance until 2003. At that timeat the age of 23 years, thepatient presented

206 36, 2007 at a nearby dental clinic with chief complaints of mandibular protrusion and asymmetry. The patient was referred to the Department of Orthodontics of Meikai University Dental Hospital, and in October 2003, he underwent thorough testing and treatment. Maxillary underdevelopment and mandibular overgrowth were confirmed as skeletal problems. Although the maxillary underdevelopment was mild, corrective surgery was indicated because of marked mandibular overgrowth, tilting of the maxillary occlusal plane in the left inferior direction, and excessive lower face height. Prior to orthognathic surgery, the patient s systemic condition was assessed, and simultaneous corrective surgery of the maxilla and mandible was planned in a conference involving the orthodontics and oral surgery departments. At present12 months post, thepostoperative orthodontic therapy has been completed, the patient s symptoms have improved, and a tight occlusal relationship has been achieved. When performing corrective surgery on patients with jaw deformities complicated by systemic diseases, it is essential to ascertain the patient s systemic condition. Furthermore, when planning therapy and orthognathic surgery, it is very important for orthodontics, oral surgery, anesthesiology, and related departments to work closely with each other. Key words : Dubin-Johnson syndrome, congenital jaundice, dentofacial deformity, orthognathic surgery 1 2, 3 Dubin-Johnson 1954 Dubin Johnson 4 Sprintz Nelson 5 6, 7 8 10 Dubin-Johnson 23 1 350-0283 1-1 2003 10 24 Dubin-Johnson 10 20 Dubin-Johnson 2003 23 2003 10 concave type Fig 1 Angle overjet 8.5 mm, overbite 1.5 mm 4.0 mm square taper coronal arch width 30.8 mm Arch length discrepancy 3.0 mm3.0 mm Fig 2 X

1 207 Fig 1 Facial views at initial. Fig 2 Intraoral views at initial. Fig 3 X 11 4.0 mm 2.5 11 X Fig 4Tables 1, 2 Facial angle 91.7Convexity 13.1SNA 75.0SNB 81.0ANB 6.0 Fig 3 Panoramic tomography at initial.

208 36, 2007 Fig 4 Lateral and frontal cephalograms at initial. Table 1 Cephalometric analysis1 Table 2 Cephalometric analysis2 Facial angle Convexity A-B plane Mandibular plane Y-axis Occlusal plane Interincisal L-1 to Mandibular FH to SN SNA SNB ANB U-1 to FH plane U-1 to SN plane Gonial angle Ramus inclination Mean 85.1 5.6 5.1 26.3 65.7 9.5 129.7 94.7 6 81.8 78.6 108.9 103.1 111.4 87.4 SD 5.8 4.3 3.3 6.3 3.3 4 9 7.2 3.4 3.1 3.1 5.6 5.5 5.8 4.1 Initial 91.7 13.1 7.1 30.1 61.3 9.9 134.1 79.9 9.8 75 81 6 116 106.1 132.4 77.7 91.7 12.7 7.2 30.5 61.5 7.5 118.8 85.1 9.8 75.3 81.4 5.9 125.6 115.9 1324 78.4 88.9 0.4 3.2 31.8 63.2 7 122.9 82.7 9.8 78.8 77.5 3.3 122.6 11.72 131.7 80.1 Fig 5 Gonial angle 132.4Gn-Cd 150.2 mm, Pog -Gn 92.9 mm N-Me 150.1 mm, ANS-Me 89.5 mm FH dental compensation N-S N-Me N-ANS ANS-Me S -Ptm A -Ptm Ptm -Ms A -Ms Is-Is Mo-Ms Is-Mo Gn-Cd Pog -Go Cd-Go Ii-Ii Mo-Mi Ii-Mo S-S FH Mean 73.9 136.1 60 77.2 19.8 51 22 27.6 32.6 27 33.8 128.5 82.1 69.6 48.7 37 30.4 22.9 SD 2.6 5.7 2.6 4.5 3 2.6 2.9 2.2 2.8 2 2.8 4.4 3.8 4.9 1.6 2.2 2.4 3.3 Initial 73.3 150.1 60.5 89.5 15.7 48.9 34.2 14.7 38.8 33.4 20 150.2 92.9 73.4 48.4 37.5 28.5 19.8 73.2 150.7 60 90.7 15.3 49.1 33 16 37.5 34.4 23.4 150.8 91.9 74.8 49.9 37 30.6 20.1 Preoperation Postoperation Preoperation Postoperation 72.9 146 56.8 90.3 20.8 49.2 21.5 27.8 37.6 32.4 35.9 141.8 88 68.9 51.1 36.6 30.7 19.7 49 mm click

1 209 Fig 5 Profilogram. 1 2 Angle Le Fort SSRO 1 2 Arch length discrepancy 3 4 MFT Fig 6 Treatment goalpaper surgery

210 36, 2007 Fig 7 Facial views at pre-operation. Fig 8 Intraoral views at pre-operation. Fig 9 Panoramic tomography at pre-operation. 2004 3 018x 025edgewise bracket MFT

1 211 Fig 10 Lateral and frontal cephalograms at pre-operation. Table 3 Result of blood test. Table 4 Change of bilirubin level. Value Unit Normal value Total protein A/G Total bilirubin Direct bilirubin ASTGOT ALTGPT γ-gtp ALP LDH Cholinesterase APTT PT 7.3 1.92 5.6 3.9 16 19 13 175 116 4501 40.1 12.1 g/dl mg/dl mg/dl U/l U/l U/l U/l U/l U/l sec sec 6.58.2 1.32.2 0.21.0 00.4 1040 545 1673 104338 120245 35008000 26.038.0 8.010.0 20 2005 11 Le Fort 4mm 4mm 2mm 5mm 11 mm 4 mm Fig 6 X Figs 7 10 5.6 mg/dl 3.9 mg/dl APTT40.1 26.0 38.0 PT12.1 8.012.0 53.8 GOT, GPT Table 3 60 40 PT APTT K15mg! 1 PT APTT 2006 2

212 36, 2007 Fig 11 Facial views at after post-surgical orthodontics. Fig 12 Intraoral views at after post-surgical orthodontics.!! 4 25 300 ml 10 mg 10 6.3 mg/dl 4.7 mg/dl 2 3.6 mg/dl, 2.9 mg/dl Table 4 3 APTT 34.5 PT 11.0 8 2 L 7 2

1 213 4 2 MFT 11 1 SSRO Fig 13 Panoramic tomography after post-surgical orthodontics. Fig 14 Lateral and frontal cephalograms after post-surgical orthodontics. Fig 15 Cephalometric superimposition.

214 36, 2007 12 13, 14 15 mm SSRO 15 16 concave type straight type 17 overjet 8.5 mm 3.5 mm, overbite 1.5 mm 2.0 mm Angle Figs 11, 12 X Fig 13 IMF X 4mm 8mm ANB 6.03.3Table 1N-Me ANS-Me Table 2 X dental decompensation dental decompensation Figs 14, 15 2 Crigler-Najjor Gilbert Rotor Dubin-Johnson 4 Gilbert Rotor Dubin- Johnson Dubin-Johnson 71.1 1020 3 6 25 mg/dl 68.4 7 ICG Mandema BSP black liver 6, 18 19 20 23 25 24 Dubin- Johnson C

1 215 23 25 8 Dubin-Johnson 10 26 8 K PT APTT PT APTT 27 Selingsohn 27 K PT APTT APTT PT APTT 1 2 2 26 2 9 28 10 6.3 mg/dl 10 mg/dl 8, 18, 24, 26, 29 20 Dubin-Johnson 4.92.9 mg/dl 14.310.7 mg/dl 8120 22, 30, 31 Dubin-Johnson 20 mg/dl 10, 32 2 32 Dubin- Johnson Dubin-Johnson 1 Dubin-Johnson 2 3 4 K 16, 190 195, 2006 1

216 36, 2007 33, 726 727, 2005 1 17, 42 50, 2007 Dubin IN and Johnson FB : Chronic idiopathic jaundice with unidentified pigmentin liver cells. Medicine 33, 155 197, 1954 Sprinz H and Nelson RS : Persistent non-hemolytic hyperbilirubinemia associated with lipochrome-like pigment in liver cells. Ann Intern Med 41, 952 962, 1954 Dubin-Johnson 21, 298 303, 1989 41, 566 573, 1983 Dubin-Johnson 1 63, 214 218, 2002 Dubin-Johnson 3, 33 36, 1995 Dubin-Johnson Gilbert 40, 119 123, 1991 X 4, 87 95, 1994 14, 26 34, 2004 4, 115 122, 1994 12, 77 83, 2002 Obwegeser H : Der offene Biss in chirurgischer Sicht. SSO Schweiz Monatsschr Zahnheilkd 74, 668 687, 1964 Malakouti B : Combined procedures in corrective surgery of prognathism and associated deformities. J Oral Surg 28, 506 515, 1970 Le Fort 1 12, 85 93, 2002 Dubin-Johnson 1 66, 1105 1110, 2005 Dubin-Johnson 1 8, 256 261, 2003 Dubin-Johnson 1 30, 729 733, 1995 Dubin-Johnson 1 17, 168 170, 2000 Dubin-Johnson 48, 27 31, 1988 C Dubin-Johnson 2 34, 161 165, 1993 Dubin-Johnson 1 61, 2453 2457, 2000 Dubin-Johnson C 1 23, 776 780, 1995 Dubin-Johnson 38, 496 502, 1976 Seligsohn U, Shani M, Ramot B, Adam A and Sheba C : Dubin-Johnson syndrome in Israel. Q J Med 39, 569 584, 1970 Taylor S : Gilbert s syndrome as a cause of postoperative jaundice. Anaesthesia 39, 1222 1224, 1984 Dubin-Johnson 1 59, 116 118, 1997 Riely CA : The use of plasmapheresis in patients with liver disease. Am J Gastroenterol 82, 882, 1987 Ueno S, Tanabe G, Hanazono K, Ogawa H, Yoshidome S, Aikou T and Yoshida A : Postoperative management following massive hepatectomy in a patient with Dubin-Johnson syndrome : report of a case. Surg Today 28, 1274 1278, 1998 15, 39 42, 1986 2007 5 21 2007 5 30