KNEEZEL
J Oral Maxillofac 48.499-502,
499
AND TILGHMAN
Surg
1990
Orthognathic Surgical Correction of a Dentofacial Deformity in a Patient With Cystic Fibrosis RANDY C. KNEEZEL, DMD,* AND DONALD M. TILGHMAN,
Cystic fibrosis (CF), an autosomal recessive disease, is characterized by abnormally thick secretions from exocrine glands. The main clinical feature is a chronic, diffuse, obstructive pulmonary process that leads to recurrent infection, chronic bronchitis, and bronchiectasis.‘-3 Staphylococci and pseudomonads are often found infecting the upper airways.4 In the pancreas, increased viscosity results in obstruction of the secretory ducts, with subsequent exocrine insufficiency and malabsorption.5-7 A three- to fivefold increase in concentration of sodium and chloride is found in eccrine sweat gland secretions,8-9 and parotid flow rates and electrolyte levels are elevated.““’ An association has been made between upper respiratory problems and development of dentofacial deformities in individuals with adenoid hyperplasia. l2 However, although patients with CF also have upper respiratory problems because of pronounced constriction of the nasal cavity and chronic maxillary sinusitis,‘3-‘4 Reitman and Faber” could not demonstrate cephalometric abnormalities in 59 of their patients with this disorder. When confronted with a significant dentofacial deformity in a patient with CF, the clinician not only needs to correct the deformity surgically but must also carefully manage the systemic disease during all phases of treatment. The purpose of this article is to demonstrate comprehensive treatment of a patient with CF who had a significant dentofacial deformity. Since these patients are living longer
Received from the Division of Dentistry and Oral and Maxillofacial Surgery, The Johns Hopkins Hospital, Baltimore, MD. * Chief Resident. t Associate Professor, Oral and Maxiliofacial Surgery, Department of Surgery; Professor, Oral and Maxillofacial Surgery, and Director of Postgraduate Training in Oral and Maxillofacial Surgery. University of Maryland Medical Systems, Baltimore. Address correspondence and reprint requests to Dr Kneezel: 529 Sheffield Dr, Decatur, IL 62526. 0 1990 American geons
Association
0278-2391/90/4805-0012$3.00/O
of Oral and Maxillofacial
Sur-
DDSt
than previously because of improved medical care, l6 treatment of their dentofacial deformities is necessary to improve quality of life. Report of a Case An l&year-old white woman with CF was admitted to the Oral and Maxillofacial Surgery Service at The Johns
Hopkins Hospital with chief complaints that she had difficulty chewing and that her face was too long. For the previous 2 years, she had undergone preoperative orthodontic therapy to level and align her dental arches. Since birth, the patient had been followed in The Johns Hopkins Cystic Fibrosis Clinic. She contracted pneumonia at 5 months of age. Between the ages of 4 and 9 years, polyethylene tubes were placed three separate times to relieve middle ear effusions. In spite of these tubes, the patient developed upper respiratory tract infections three to four times a year; these infections were characterized by increased coughing and wheezing, for which she required oral antibiotics. Before admission, the patient’s most recent throat culture in April 1988 was positive for Staphylococcus aureus and Pseudomonas ueruginosa. Presently she receives percussion and postural drainage (P and PD) from her parents two to three times each day. Her medications include pancrease with meals and multivitamin supplements. On examination, the patient’s upper, middle, and lower facial thirds measured 40 mm, 54 mm, and 70 mm, respectively. Her mandibular plane angle (SN-MGo) was 57 degrees (normal = 32 degrees) (Fig 1). The ANWNS-MGo angle was 43 degrees (normal = 25 degrees). No other significant skeletal cephalometric abnormalities were present. Seven millimeters of incisor showed at rest, with 3 mm of gingiva evident on smiling. Her bite from mandibular incisal tip to maxillary incisal tip was open 8 mm (Fig 2), with the only contact occurring on the second molars. The palatal vault was very deep and constricted, and a posterior crossbite was evident on the right side. Oral hygiene was poor. The patient had a severe constriction of the nasal airway, with narrowing of the nasal base. On chest auscultation, she had slightly decreased air entry at the bases, with good middle and upper lung fields. No rales, rhonchi, or wheezes were present. The chest radiograph showed increased perihilar upper lobe markings, but no change had occurred since October 1987. Pulmonary function tests were normal. The patient’s preoperative weight was 41.5 kilograms. On advice from The Division of Pulmonary Medicine,
CORRECTIVE
SURGERY IN CYSTIC FIBROSIS PATIENT
FIGURE 1. A, Preoperative cephalometric radiograph showing high mandibular angle, deficient midface, severe open bite, and lips in unstrained rest position. Note the soft tissue bulge of hyperplastic mentalis muscle. B, Preoperative photograph showing excessive lower third, strained position of lips attempting to mask open bite, and unesthetic !%degree submental-anterior neck angle (normal is 1lo-130 degrees).
P and PD was performed three times the day before surgery. In addition tobramycin 50 mg every 8 hours, oxacillin 1 g every 4 hours, and ceftazidine 1 g every 12 hours, were administered intravenously to eliminate the patient’s abnormal upper respiratory flora. In the operating room, after use of cocaine and slow, deliberate nasopharyngeal tube dilatation, the patient’s
FIGURE 2. Severe aperthognathia in a CF patient resulting in both inability to masticate food properly and poor overall facial esthetics.
constricted left nasal passage was intubated with relative ease. A standard Le Fort I osteotomy was then performed. After the maxilla was downfractured, the maxillary sinuses were noted to be only 15 mm in greatest dimension and completely obliterated by mucosal polyps. After removal of these polyps by curettage, a channel was created in the thick medial walls of the sinuses to allow for drainage. To enlarge the nasal passages, the constricted piriform rims were significantly widened and the nasal floor was anatomically lowered using a large round bur. The maxilla was differentially impacted, 5 mm at the first molars and 3 mm at the incisors. The maxilla was then fixed using four titanium mesh strips secured with sixteen 5-mm titanium screws (TiMesh, Inc, Calabasas, CA). After a bilateral sagittal split ramus osteotomy, the mandible was repositioned superiorly 9 mm (in large part due to autorotation) and posteriorly 5 mm, closing the open bite and bringing the facial thirds into balance (Figs 3 A and B, and 4A). The patient was then placed in maxillomandibular fixation using 26-gauge stainless steel wires and an interocclusal acrylic splint. Two inferior border titanium mesh strips (TiMesh, Inc) and two superior border wires achieved stability in the mandible. As a result of the surgery, the mandibular plane angle decreased from 57 degrees to 47 degrees, and the ANSIPNS-MGo angle increased from 15 degrees to 26 degrees, which is within normal limits. Postoperatively, the patient remained intubated and was taken to the surgical intensive care unit where she received chest physiotherapy and pulmonary toilet every
KNEEZEL
AND TILGHMAN
FIGURE 3. A, Postoperative radiograph (at 6 months) illustrating a more normocephalic mandibular angle and closure of the open bite. The mentalis soft tissue bulge remains. B, Postoperative photograph (at 6 months) shows improved vertical facial balance, improved contour of deficient maxilla, and more esthetic throat form.
FIGURE 4. A, Comparison of cephalometric tracings made preoperatively (solid line) and 5 days after surgery (broken line). An occlusal splint was present when the postsurgical cephalogram was made. B, Comparison of cephalometric tracings made 5 days after surgery (solid line) and 6 months after surgery (broken line). The change seen between tracings represents removal of the acrylic occlusal splint.
502
CORRECTIVE
1 to 2 hours. She was extubated the next morning without complication, and the chest radiograph showed no significant infiltrates. Postoperative care was aggressive. Every 4 hours, 0.3 mL of Alupent (Boehringer Ingelheim Pharmaceuticals, Inc, Ridgefield, CT) as nebulized in 2.5 mL of normal saline via face tent, with subsequent P and PD. The patient was encouraged to cough and deepbreathe to clear secretions. During the night, a pulse oximeter was attached to her finger, and the nurses were instructed to administer 40% oxygen if the patient’s oxygen saturation dropped to 94%. Frequent throat cultures were taken, and the patient was kept on triple antibiotics until just before discharge when growth of pseudomonas had ceased. Because the patient did not follow instructions to cough and ambulate appropriately, secretions accumulated in her lungs. By the third postoperative day, coarse rhonchi were heard in all lung fields, and the chest radiograph showed infiltrates in the right and left lower lobes along with the right middle lobe. Toward the end of the seventh postoperative day, her compliance greatly improved, along with her respiratory signs. It was also difficult to encourage the patient to eat. For the first 4 postoperative days the patient consumed a maximum 150 mL/d of clear liquids, although she had no problems swallowing. On the fifth postoperative day the patient was released from her maxillomandibular fixation and her intake increased to 600 mL. Because she could
countants, 1 received his MD degree, and 1 is an associate professor of psychology. Increased life span as well as the intellectual and socioeconomic achievements of patients with CF underscore the necessity for surgically correcting dentofacial deformities in order to improve masticatory function, psychosocial interactions, and to raise self-esteem. In addition, because CF patients have nasal airway constriction, with resultant difficulty in nasal breathing, 13*14the postsurgical improvement in nasal air exchange seen in our case suggests that enlargement of the nasal airway should be incorporated into the overall surgical treatment plan. Another significant observation can be made from this case. Early release from maxillomandibular fixation allowed our patient both to better manage her secretions and to dramatically improve her ability to eat. Lack of relapse after 6 months, even after a significant counterclockwise surgical movement of the mandible, suggests that with rigid fixation the surgeon can, without apprehension, release maxillomandibular fixation early in CF patients to facilitate medical rehabilitation.
not digest fat, pancrease was added to her diet when it was advanced to full liquids. Ensure (Ross Laboratories, Columbus, OH) and Magnacal (Sherwood Medical, Mahwah, NJ) were then added to increase her calorie intake. Six months after discharge the patient was interviewed. Her self-image had greatly improved. Oral hygiene was
References
good. The patient could successfully masticate sandwiches and pizza, and could breathe through her nose. A cephalometric radiograph taken at 6 months after surgery showed no relapse when compared with the initial postoperative cephalometric radiograph taken 5 days after surgery (Fig 4B).
Discussion Cystic fibrosis is not an uncommon disease, occurring in approximately 1 of every 2,000births in whites. Death results from severe pulmonary infections and respiratory failure. Cor pulmonale is a common late complication. In the past, patients with CF would normally die as teenagers.16 However, in the last two decades, survival until the third and fourth decades has become common.‘6 Advances in antimicrobial therapy, especially in treating pseudomonas infection, have been the key to extended survival.4 Increased physician awareness has led to early diagnosis and early institution of therapy.6 Establishment of CF clinics has increased access of patients to medical care.16 Education of family members both in therapy and early recognition of potential problems has contributed to extension of life. Forty-two of 70 patients in Shwachman and Kowalski’s16 study were married, 41 were college graduates, 9 pursued graduate education, 2 patients graduated from law school, 17 became teachers, two became certified public ac-
SURGERY IN CYSTIC FIBROSIS PATIENT
1. Addington WW, Cugell DW, Zelhowitz PS, et al: Cystic fibrosis of the pancreas-A comparison of the pulmonary manifestations in children and young adults. Chest 59:306, 1971 2. Shwachman H, Khaw KT, Kowalski M: Gentamycin and other antibiotics in pulmonary infections associated with cystic fibrosis. Clin Pediatr 14: 1115, 1975 3. Braunwald E, lsselbacher KJ: Harrison’s Principles of lntemal Medicine. New York, NY, McGraw-Hill, 1988, pp 1542-1544 4. Stem RC, Boat TF, Doershuk CF: Course of cystic fibrosis in 95 patients. J Pediatr 89:406, 1976 5. Tomoshefski JF, Chrisstoforidis AJ, Abdullah AK: Cystic fibrosis in young adults. Chest 57:28, 1970 6. Shwachman H. Doolev RR. Guilmette F. et al: Cvstic fibrosis of the pancreas wit6 varying degrees of -pancreatic insufficiency. Am J Dis Child 92:347, 1956 7. Cece JD, Henry JP, Toigo A: Pancreatic cystic fibrosis in an adult. JAMA 181:31, 1972 8. Peterson EM: Consideration of cystic fibrosis in adults, with a study of sweat electrolytic values. JAMA 171:87, 1959 9. Scwachman H, Antonowicz I: Problems in cystic fibrosisThe sweat test in cystic fibrosis. Ann NY Acad Sci 93:600, 1962 10. Kahizaki G: A new diagnostic test for pancreatic disorders by examination of parotid saliva. Am J Gastroenterol 65:437, 1976 11. Mormer J, Borbero GJ, Sibings MS: The pattern of parotid gland secretion in cystic fibrosis of the pancreas. Gastroenterology 50:551, 1%6 12. Ricketts RM: Respiratory obstruction syndrome. Am J Orthod 54:495, 1968 13. Rose LF, Kaye T: Internal Medicine for Dentistry. St Louis, MO. Mosbv. 1983, up 1101-1102 14. Shwachman G, Kulczydhi LL, Mueller HL: Nasal polyposis in natients with cvstic fibrosis. Pediatrics 30:389, 1962 15. Reit&n AA, Faber-RD: A cephalometric study of patients with cystic fibrosis. J Am Dent Assoc 96:83, 1978 16. Shwachman H, Kowalski M, Khaw K-T: Cystic fibrosis, a new outlook-70 patients above 25 years of age. Medicine 56: 129, 1977
J Oral Maxillofac 48.499-502,
499
AND TILGHMAN
Surg
1990
Orthognathic Surgical Correction of a Dentofacial Deformity in a Patient With Cystic Fibrosis RANDY C. KNEEZEL, DMD,* AND DONALD M. TILGHMAN,
Cystic fibrosis (CF), an autosomal recessive disease, is characterized by abnormally thick secretions from exocrine glands. The main clinical feature is a chronic, diffuse, obstructive pulmonary process that leads to recurrent infection, chronic bronchitis, and bronchiectasis.‘-3 Staphylococci and pseudomonads are often found infecting the upper airways.4 In the pancreas, increased viscosity results in obstruction of the secretory ducts, with subsequent exocrine insufficiency and malabsorption.5-7 A three- to fivefold increase in concentration of sodium and chloride is found in eccrine sweat gland secretions,8-9 and parotid flow rates and electrolyte levels are elevated.““’ An association has been made between upper respiratory problems and development of dentofacial deformities in individuals with adenoid hyperplasia. l2 However, although patients with CF also have upper respiratory problems because of pronounced constriction of the nasal cavity and chronic maxillary sinusitis,‘3-‘4 Reitman and Faber” could not demonstrate cephalometric abnormalities in 59 of their patients with this disorder. When confronted with a significant dentofacial deformity in a patient with CF, the clinician not only needs to correct the deformity surgically but must also carefully manage the systemic disease during all phases of treatment. The purpose of this article is to demonstrate comprehensive treatment of a patient with CF who had a significant dentofacial deformity. Since these patients are living longer
Received from the Division of Dentistry and Oral and Maxillofacial Surgery, The Johns Hopkins Hospital, Baltimore, MD. * Chief Resident. t Associate Professor, Oral and Maxiliofacial Surgery, Department of Surgery; Professor, Oral and Maxillofacial Surgery, and Director of Postgraduate Training in Oral and Maxillofacial Surgery. University of Maryland Medical Systems, Baltimore. Address correspondence and reprint requests to Dr Kneezel: 529 Sheffield Dr, Decatur, IL 62526. 0 1990 American geons
Association
0278-2391/90/4805-0012$3.00/O
of Oral and Maxillofacial
Sur-
DDSt
than previously because of improved medical care, l6 treatment of their dentofacial deformities is necessary to improve quality of life. Report of a Case An l&year-old white woman with CF was admitted to the Oral and Maxillofacial Surgery Service at The Johns
Hopkins Hospital with chief complaints that she had difficulty chewing and that her face was too long. For the previous 2 years, she had undergone preoperative orthodontic therapy to level and align her dental arches. Since birth, the patient had been followed in The Johns Hopkins Cystic Fibrosis Clinic. She contracted pneumonia at 5 months of age. Between the ages of 4 and 9 years, polyethylene tubes were placed three separate times to relieve middle ear effusions. In spite of these tubes, the patient developed upper respiratory tract infections three to four times a year; these infections were characterized by increased coughing and wheezing, for which she required oral antibiotics. Before admission, the patient’s most recent throat culture in April 1988 was positive for Staphylococcus aureus and Pseudomonas ueruginosa. Presently she receives percussion and postural drainage (P and PD) from her parents two to three times each day. Her medications include pancrease with meals and multivitamin supplements. On examination, the patient’s upper, middle, and lower facial thirds measured 40 mm, 54 mm, and 70 mm, respectively. Her mandibular plane angle (SN-MGo) was 57 degrees (normal = 32 degrees) (Fig 1). The ANWNS-MGo angle was 43 degrees (normal = 25 degrees). No other significant skeletal cephalometric abnormalities were present. Seven millimeters of incisor showed at rest, with 3 mm of gingiva evident on smiling. Her bite from mandibular incisal tip to maxillary incisal tip was open 8 mm (Fig 2), with the only contact occurring on the second molars. The palatal vault was very deep and constricted, and a posterior crossbite was evident on the right side. Oral hygiene was poor. The patient had a severe constriction of the nasal airway, with narrowing of the nasal base. On chest auscultation, she had slightly decreased air entry at the bases, with good middle and upper lung fields. No rales, rhonchi, or wheezes were present. The chest radiograph showed increased perihilar upper lobe markings, but no change had occurred since October 1987. Pulmonary function tests were normal. The patient’s preoperative weight was 41.5 kilograms. On advice from The Division of Pulmonary Medicine,
CORRECTIVE
SURGERY IN CYSTIC FIBROSIS PATIENT
FIGURE 1. A, Preoperative cephalometric radiograph showing high mandibular angle, deficient midface, severe open bite, and lips in unstrained rest position. Note the soft tissue bulge of hyperplastic mentalis muscle. B, Preoperative photograph showing excessive lower third, strained position of lips attempting to mask open bite, and unesthetic !%degree submental-anterior neck angle (normal is 1lo-130 degrees).
P and PD was performed three times the day before surgery. In addition tobramycin 50 mg every 8 hours, oxacillin 1 g every 4 hours, and ceftazidine 1 g every 12 hours, were administered intravenously to eliminate the patient’s abnormal upper respiratory flora. In the operating room, after use of cocaine and slow, deliberate nasopharyngeal tube dilatation, the patient’s
FIGURE 2. Severe aperthognathia in a CF patient resulting in both inability to masticate food properly and poor overall facial esthetics.
constricted left nasal passage was intubated with relative ease. A standard Le Fort I osteotomy was then performed. After the maxilla was downfractured, the maxillary sinuses were noted to be only 15 mm in greatest dimension and completely obliterated by mucosal polyps. After removal of these polyps by curettage, a channel was created in the thick medial walls of the sinuses to allow for drainage. To enlarge the nasal passages, the constricted piriform rims were significantly widened and the nasal floor was anatomically lowered using a large round bur. The maxilla was differentially impacted, 5 mm at the first molars and 3 mm at the incisors. The maxilla was then fixed using four titanium mesh strips secured with sixteen 5-mm titanium screws (TiMesh, Inc, Calabasas, CA). After a bilateral sagittal split ramus osteotomy, the mandible was repositioned superiorly 9 mm (in large part due to autorotation) and posteriorly 5 mm, closing the open bite and bringing the facial thirds into balance (Figs 3 A and B, and 4A). The patient was then placed in maxillomandibular fixation using 26-gauge stainless steel wires and an interocclusal acrylic splint. Two inferior border titanium mesh strips (TiMesh, Inc) and two superior border wires achieved stability in the mandible. As a result of the surgery, the mandibular plane angle decreased from 57 degrees to 47 degrees, and the ANSIPNS-MGo angle increased from 15 degrees to 26 degrees, which is within normal limits. Postoperatively, the patient remained intubated and was taken to the surgical intensive care unit where she received chest physiotherapy and pulmonary toilet every
KNEEZEL
AND TILGHMAN
FIGURE 3. A, Postoperative radiograph (at 6 months) illustrating a more normocephalic mandibular angle and closure of the open bite. The mentalis soft tissue bulge remains. B, Postoperative photograph (at 6 months) shows improved vertical facial balance, improved contour of deficient maxilla, and more esthetic throat form.
FIGURE 4. A, Comparison of cephalometric tracings made preoperatively (solid line) and 5 days after surgery (broken line). An occlusal splint was present when the postsurgical cephalogram was made. B, Comparison of cephalometric tracings made 5 days after surgery (solid line) and 6 months after surgery (broken line). The change seen between tracings represents removal of the acrylic occlusal splint.
502
CORRECTIVE
1 to 2 hours. She was extubated the next morning without complication, and the chest radiograph showed no significant infiltrates. Postoperative care was aggressive. Every 4 hours, 0.3 mL of Alupent (Boehringer Ingelheim Pharmaceuticals, Inc, Ridgefield, CT) as nebulized in 2.5 mL of normal saline via face tent, with subsequent P and PD. The patient was encouraged to cough and deepbreathe to clear secretions. During the night, a pulse oximeter was attached to her finger, and the nurses were instructed to administer 40% oxygen if the patient’s oxygen saturation dropped to 94%. Frequent throat cultures were taken, and the patient was kept on triple antibiotics until just before discharge when growth of pseudomonas had ceased. Because the patient did not follow instructions to cough and ambulate appropriately, secretions accumulated in her lungs. By the third postoperative day, coarse rhonchi were heard in all lung fields, and the chest radiograph showed infiltrates in the right and left lower lobes along with the right middle lobe. Toward the end of the seventh postoperative day, her compliance greatly improved, along with her respiratory signs. It was also difficult to encourage the patient to eat. For the first 4 postoperative days the patient consumed a maximum 150 mL/d of clear liquids, although she had no problems swallowing. On the fifth postoperative day the patient was released from her maxillomandibular fixation and her intake increased to 600 mL. Because she could
countants, 1 received his MD degree, and 1 is an associate professor of psychology. Increased life span as well as the intellectual and socioeconomic achievements of patients with CF underscore the necessity for surgically correcting dentofacial deformities in order to improve masticatory function, psychosocial interactions, and to raise self-esteem. In addition, because CF patients have nasal airway constriction, with resultant difficulty in nasal breathing, 13*14the postsurgical improvement in nasal air exchange seen in our case suggests that enlargement of the nasal airway should be incorporated into the overall surgical treatment plan. Another significant observation can be made from this case. Early release from maxillomandibular fixation allowed our patient both to better manage her secretions and to dramatically improve her ability to eat. Lack of relapse after 6 months, even after a significant counterclockwise surgical movement of the mandible, suggests that with rigid fixation the surgeon can, without apprehension, release maxillomandibular fixation early in CF patients to facilitate medical rehabilitation.
not digest fat, pancrease was added to her diet when it was advanced to full liquids. Ensure (Ross Laboratories, Columbus, OH) and Magnacal (Sherwood Medical, Mahwah, NJ) were then added to increase her calorie intake. Six months after discharge the patient was interviewed. Her self-image had greatly improved. Oral hygiene was
References
good. The patient could successfully masticate sandwiches and pizza, and could breathe through her nose. A cephalometric radiograph taken at 6 months after surgery showed no relapse when compared with the initial postoperative cephalometric radiograph taken 5 days after surgery (Fig 4B).
Discussion Cystic fibrosis is not an uncommon disease, occurring in approximately 1 of every 2,000births in whites. Death results from severe pulmonary infections and respiratory failure. Cor pulmonale is a common late complication. In the past, patients with CF would normally die as teenagers.16 However, in the last two decades, survival until the third and fourth decades has become common.‘6 Advances in antimicrobial therapy, especially in treating pseudomonas infection, have been the key to extended survival.4 Increased physician awareness has led to early diagnosis and early institution of therapy.6 Establishment of CF clinics has increased access of patients to medical care.16 Education of family members both in therapy and early recognition of potential problems has contributed to extension of life. Forty-two of 70 patients in Shwachman and Kowalski’s16 study were married, 41 were college graduates, 9 pursued graduate education, 2 patients graduated from law school, 17 became teachers, two became certified public ac-
SURGERY IN CYSTIC FIBROSIS PATIENT
1. Addington WW, Cugell DW, Zelhowitz PS, et al: Cystic fibrosis of the pancreas-A comparison of the pulmonary manifestations in children and young adults. Chest 59:306, 1971 2. Shwachman H, Khaw KT, Kowalski M: Gentamycin and other antibiotics in pulmonary infections associated with cystic fibrosis. Clin Pediatr 14: 1115, 1975 3. Braunwald E, lsselbacher KJ: Harrison’s Principles of lntemal Medicine. New York, NY, McGraw-Hill, 1988, pp 1542-1544 4. Stem RC, Boat TF, Doershuk CF: Course of cystic fibrosis in 95 patients. J Pediatr 89:406, 1976 5. Tomoshefski JF, Chrisstoforidis AJ, Abdullah AK: Cystic fibrosis in young adults. Chest 57:28, 1970 6. Shwachman H. Doolev RR. Guilmette F. et al: Cvstic fibrosis of the pancreas wit6 varying degrees of -pancreatic insufficiency. Am J Dis Child 92:347, 1956 7. Cece JD, Henry JP, Toigo A: Pancreatic cystic fibrosis in an adult. JAMA 181:31, 1972 8. Peterson EM: Consideration of cystic fibrosis in adults, with a study of sweat electrolytic values. JAMA 171:87, 1959 9. Scwachman H, Antonowicz I: Problems in cystic fibrosisThe sweat test in cystic fibrosis. Ann NY Acad Sci 93:600, 1962 10. Kahizaki G: A new diagnostic test for pancreatic disorders by examination of parotid saliva. Am J Gastroenterol 65:437, 1976 11. Mormer J, Borbero GJ, Sibings MS: The pattern of parotid gland secretion in cystic fibrosis of the pancreas. Gastroenterology 50:551, 1%6 12. Ricketts RM: Respiratory obstruction syndrome. Am J Orthod 54:495, 1968 13. Rose LF, Kaye T: Internal Medicine for Dentistry. St Louis, MO. Mosbv. 1983, up 1101-1102 14. Shwachman G, Kulczydhi LL, Mueller HL: Nasal polyposis in natients with cvstic fibrosis. Pediatrics 30:389, 1962 15. Reit&n AA, Faber-RD: A cephalometric study of patients with cystic fibrosis. J Am Dent Assoc 96:83, 1978 16. Shwachman H, Kowalski M, Khaw K-T: Cystic fibrosis, a new outlook-70 patients above 25 years of age. Medicine 56: 129, 1977
