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Cleft palate lateral synechia syndrome
Cleft palate lateral synechia syndrome Review of the literature
and case report
Nebridio M. Dinardo, DMD,” James M. Christian, DDS,b Joel A. Bennett, DDS, MD,’ and John G. Shutack, DO,d Philadelphia, Pa., and Morganstown, W. Va. HAHNEMANN
UNIVERSITY
HOSPITAL
AND
WEST
VIRGINIA
UNIVERSITY
Fig.
1. Preoperative
S
ynechiae are adhesions between anatomic structures. Synechiae of the oral cavity are fibrous adhesions that originate in the margins of a palatal cleft and attach to the mandibular alveolar ridge or the floor of the mouth. They may also be found spanning a palatal cleft. This report describes an unusual case of cleft palate associatedwith interalveolar synechiae and concomitant hydrocephalus. CASE REPORT
The mother was a 23-year-old gravida II, para I with one abortion. The parents’ medical histories were noncontributory, and they were not consanguineous. No abnormalities in growth and development of the fetus were noted during infrequent prenatal visits. The mother was hospitalized 2 weeks before delivery for treatment of asthma. She was treated with albuterol, aminophylline, and prednisone. The fetus was delivered at term by cesarean section, for cephalopelvic disproportion caused by hydrocephaly. He weighed 3500 gm and had an Apgar score of 9 at 1 and 5 minutes. At birth, the child was discovered to have multiple congenital anomalies that included micrognathia, and alveolar synechiae. At 2 days of age, the infant was transferred to Hahnemann University Hospital for the insertion of a ventriculoperitoneal shunt for hydrocephalus. The Department of Oral and Maxillofacial Surgery was consulted for evaluation of the synechiae, which prohibited him from being fed. Physical examination revealed a markedly enlarged head with a circumference of 47 cm (normal, 35 to 45 cm). The mandible appeared relatively micrognathic. Intraoral examination showed multiple fibrous bands extending
“Chief Resident, Department of Oral and Maxillofacial Surgery, Hahnemann University Hospital. ‘Assistant Professor, Department of Oral and Maxillofacial Surgery, West Virginia University. ‘Resident, Department of Anesthesiology, Hahnemann University Hospital. “Assistant Professor, Departments of Anesthesiology and Pediatrics, Hahnemann University Hospital. 7/12/11442
vertically
appearance of synechiae running between maxillary and mandibular arches.
Fig.
2. Postoperative appearance.
from the lingual surface of the maxillary alveolar ridge to the crest of the mandibular ridge. The synechiae ranged in width from 1 to 5 mm and extended from the midline posteriorly to the molar region bilaterally (Fig. 1). Mandibular motion was minimal because of the tethering of the bands and precluded examination of the tongue, the palate, or the pharynx. Because of the infant’s inability to feed orally, parenteral nutrition was initiated. Further preoper565
566
Dinardo et al.
ORAL SURG ORAL MED ORALPATHOL
November
ative evaluations revealed mild cardiac abnormalities, including signs of pulmonary hypertension and poor left ventricular function. At the age of 4 days, the patient was brought to the operating room for placement of a ventriculoperitoneal shunt and surgical division of the synechiae. Becauseof the limited oral access, a blind awake nasal intubation was planned. The infant was positioned prone with the head supported and the neck extended.‘,* This maneuver helps the anesthesiologist direct the endotracheal tube into the trachea. Atropine, 0.2 mg, was administered intravenously, followed by topical intranasal application of 4% cocaine. A 3.0 mm endotracheal tube was placed in the right nares while breath sounds were listened for. On the third attempt, a translaryngeal intubation was successful. Breath sounds were auscultated equally over both axillae and the anterior chest wall. After intubation in the prone position, the infant was placed on his back and prepared and draped for surgery. Anesthesia was maintained with nitrous oxide, oxygen, isoflurane, and pancuronium. The synechiae were clamped with curved hemostats and transected between the instruments. Hemostasis was achieved with electrocautery, limiting blood loss to less than 5 cc. Division of the synechiae produced an immediate increase in mandibular opening (Fig. 2). Both temporomandibular joints seemedto function normally. Inspection of the oral cavity revealed a midline cleft of the hard and soft palates, with an intact premaxilla. A significant ankyloglossia was also present. The neurosurgical team then completed the shunt procedure, and the patient was returned to the neonatal intensive care unit with the nasotracheal tube in place. The anesthetic course was uneventful. The child’s hospital course was complicated by progressive worsening of his cardiopulmonary function, and he died of a cardiorespiratory arrest 13 days after surgery.
however, many theories have been proposed. Mathis? postulated that the adhesions were remnants of the buccopharyngeal membrane. Hayward and Avery3 thought the adhesions developed as a result of contact between the epithelium of the palatine shelf and the floor of the mouth. The adhesions restrict facial growth and result in elevation of the floor of the mouth and tongue. This could inhibit the fusion of the palatal shelves, resulting in a cleft. The syndrome has been shown to exhibit an autosomal dominant mode of inheritence.4,5.9,lo Release of the synechiae allows the infant to feed by mouth and avoids upper respiratory tract obstruction caused by the restrictions in facial growth and development imposed by the synechiae. SUMMARY
Cleft palate lateral synechiae syndrome is a rare syndrome consisting of cord-like interalveolar adhesions and concomitant hypoplasia of the mandible. We have presented an infant with CPLS syndrome and coincident hydrocephalus. Surgical division of the adhesions is necessary to allow normal feeding, avoid upper airway obstruction, and allow normal mandibular function and growth. REFERENCES 1. 2.
3. 4
DISCUSSION
Cleft lip and palate are frequently associated with multiple congenital anomalies. Oral adhesions have often been observed originating in the margins of the cleft and spanning the cleft itself.3 A variation of these oral adhesions was recognized by Fuhrmann and coworkers,4who described cordlike adhesionsbetween the maxillary alveolar ridge and the floor of the mouth or mandibular alveolar ridge in five members of the same family. Associated with the adhesions were the consistent findings of a hypoplastic mandible and tongue and a short upper lip. He, along with others, named this group of anomalies the cleft palate lateral synechia syndrome (CPLS syndrome).4*5 Other authors have described this syndrome both before, and after, Fuhrmann reported them as congenital fusion of the gums. Both of these authors report radiographic abnormalities of the temporomandibular joints.6*’ The cause of the synechiae is still unknown;
1989
5 6. 7.
PopulaireC, Lundi JN, Pinaud M, Souron R. Elective tracheal intubation in the prone position for a neonate with Pierre Robin syndrome. Anesthesiology 1985;62:214-5. Alfery DD, Ward CF, Harwood IR, Mannino FL. Airway management for a neonate with congenital fusion of the jaws. Anesthesiology 1979;51:340-2. Hayward JR, Avery JK. A variation in cleft palate. J Oral Surg 1957;15:320-4. Fuhrmann W, Koch F, Schweckendick W. Autosomal dominante Vererbung von Gaumenspalte and Synechien zwischen Gaumen und Mundboden oder Zunge. Humangenetik 1972;14:196-203. Preus M, Fraser FC, Fuhrmann W. Cleft palate lateral synechia syndrome without the lateral synechia (CP f LS syndrome). Teratology 1974;9:135-41. Snijman PC, Prinsloo JG. Congenital fusion of the gums. Am J Dis Child 1966;112:593-5. Sternberg N, Sagher U, Golan J, Eidelman AI, Benhur N. Congenital fusion of the gums with bilateral fusion of the temporomandibular joints. Plast Reconstr Surg 1983;72:385/.
8. Mathis VH. Uber einen Fall von Ernlhrungsschwierigkeit bei connataler Syngnathie. Deutsche Zahnarizliche Ze%schrift 1962;17:1167-71. 9. Verdi GD, O’Neal B. Cleft palate and congenital alveolar synechia syndrome. Plastic Reconstructive Surgery 1984;74: 684-5. 10. Shaw WC, Simpson JP. Oral adhesions associated with cleft lip and palate and lip fistulae. Cleft Palate J 1980;7:2-6. Reprint requests to: Dr. James M. Christian Department of Oral and Maxillofacial School of Dentistry West Virginia University Morgantown, WV 26506
Surgery
and case report
Nebridio M. Dinardo, DMD,” James M. Christian, DDS,b Joel A. Bennett, DDS, MD,’ and John G. Shutack, DO,d Philadelphia, Pa., and Morganstown, W. Va. HAHNEMANN
UNIVERSITY
HOSPITAL
AND
WEST
VIRGINIA
UNIVERSITY
Fig.
1. Preoperative
S
ynechiae are adhesions between anatomic structures. Synechiae of the oral cavity are fibrous adhesions that originate in the margins of a palatal cleft and attach to the mandibular alveolar ridge or the floor of the mouth. They may also be found spanning a palatal cleft. This report describes an unusual case of cleft palate associatedwith interalveolar synechiae and concomitant hydrocephalus. CASE REPORT
The mother was a 23-year-old gravida II, para I with one abortion. The parents’ medical histories were noncontributory, and they were not consanguineous. No abnormalities in growth and development of the fetus were noted during infrequent prenatal visits. The mother was hospitalized 2 weeks before delivery for treatment of asthma. She was treated with albuterol, aminophylline, and prednisone. The fetus was delivered at term by cesarean section, for cephalopelvic disproportion caused by hydrocephaly. He weighed 3500 gm and had an Apgar score of 9 at 1 and 5 minutes. At birth, the child was discovered to have multiple congenital anomalies that included micrognathia, and alveolar synechiae. At 2 days of age, the infant was transferred to Hahnemann University Hospital for the insertion of a ventriculoperitoneal shunt for hydrocephalus. The Department of Oral and Maxillofacial Surgery was consulted for evaluation of the synechiae, which prohibited him from being fed. Physical examination revealed a markedly enlarged head with a circumference of 47 cm (normal, 35 to 45 cm). The mandible appeared relatively micrognathic. Intraoral examination showed multiple fibrous bands extending
“Chief Resident, Department of Oral and Maxillofacial Surgery, Hahnemann University Hospital. ‘Assistant Professor, Department of Oral and Maxillofacial Surgery, West Virginia University. ‘Resident, Department of Anesthesiology, Hahnemann University Hospital. “Assistant Professor, Departments of Anesthesiology and Pediatrics, Hahnemann University Hospital. 7/12/11442
vertically
appearance of synechiae running between maxillary and mandibular arches.
Fig.
2. Postoperative appearance.
from the lingual surface of the maxillary alveolar ridge to the crest of the mandibular ridge. The synechiae ranged in width from 1 to 5 mm and extended from the midline posteriorly to the molar region bilaterally (Fig. 1). Mandibular motion was minimal because of the tethering of the bands and precluded examination of the tongue, the palate, or the pharynx. Because of the infant’s inability to feed orally, parenteral nutrition was initiated. Further preoper565
566
Dinardo et al.
ORAL SURG ORAL MED ORALPATHOL
November
ative evaluations revealed mild cardiac abnormalities, including signs of pulmonary hypertension and poor left ventricular function. At the age of 4 days, the patient was brought to the operating room for placement of a ventriculoperitoneal shunt and surgical division of the synechiae. Becauseof the limited oral access, a blind awake nasal intubation was planned. The infant was positioned prone with the head supported and the neck extended.‘,* This maneuver helps the anesthesiologist direct the endotracheal tube into the trachea. Atropine, 0.2 mg, was administered intravenously, followed by topical intranasal application of 4% cocaine. A 3.0 mm endotracheal tube was placed in the right nares while breath sounds were listened for. On the third attempt, a translaryngeal intubation was successful. Breath sounds were auscultated equally over both axillae and the anterior chest wall. After intubation in the prone position, the infant was placed on his back and prepared and draped for surgery. Anesthesia was maintained with nitrous oxide, oxygen, isoflurane, and pancuronium. The synechiae were clamped with curved hemostats and transected between the instruments. Hemostasis was achieved with electrocautery, limiting blood loss to less than 5 cc. Division of the synechiae produced an immediate increase in mandibular opening (Fig. 2). Both temporomandibular joints seemedto function normally. Inspection of the oral cavity revealed a midline cleft of the hard and soft palates, with an intact premaxilla. A significant ankyloglossia was also present. The neurosurgical team then completed the shunt procedure, and the patient was returned to the neonatal intensive care unit with the nasotracheal tube in place. The anesthetic course was uneventful. The child’s hospital course was complicated by progressive worsening of his cardiopulmonary function, and he died of a cardiorespiratory arrest 13 days after surgery.
however, many theories have been proposed. Mathis? postulated that the adhesions were remnants of the buccopharyngeal membrane. Hayward and Avery3 thought the adhesions developed as a result of contact between the epithelium of the palatine shelf and the floor of the mouth. The adhesions restrict facial growth and result in elevation of the floor of the mouth and tongue. This could inhibit the fusion of the palatal shelves, resulting in a cleft. The syndrome has been shown to exhibit an autosomal dominant mode of inheritence.4,5.9,lo Release of the synechiae allows the infant to feed by mouth and avoids upper respiratory tract obstruction caused by the restrictions in facial growth and development imposed by the synechiae. SUMMARY
Cleft palate lateral synechiae syndrome is a rare syndrome consisting of cord-like interalveolar adhesions and concomitant hypoplasia of the mandible. We have presented an infant with CPLS syndrome and coincident hydrocephalus. Surgical division of the adhesions is necessary to allow normal feeding, avoid upper airway obstruction, and allow normal mandibular function and growth. REFERENCES 1. 2.
3. 4
DISCUSSION
Cleft lip and palate are frequently associated with multiple congenital anomalies. Oral adhesions have often been observed originating in the margins of the cleft and spanning the cleft itself.3 A variation of these oral adhesions was recognized by Fuhrmann and coworkers,4who described cordlike adhesionsbetween the maxillary alveolar ridge and the floor of the mouth or mandibular alveolar ridge in five members of the same family. Associated with the adhesions were the consistent findings of a hypoplastic mandible and tongue and a short upper lip. He, along with others, named this group of anomalies the cleft palate lateral synechia syndrome (CPLS syndrome).4*5 Other authors have described this syndrome both before, and after, Fuhrmann reported them as congenital fusion of the gums. Both of these authors report radiographic abnormalities of the temporomandibular joints.6*’ The cause of the synechiae is still unknown;
1989
5 6. 7.
PopulaireC, Lundi JN, Pinaud M, Souron R. Elective tracheal intubation in the prone position for a neonate with Pierre Robin syndrome. Anesthesiology 1985;62:214-5. Alfery DD, Ward CF, Harwood IR, Mannino FL. Airway management for a neonate with congenital fusion of the jaws. Anesthesiology 1979;51:340-2. Hayward JR, Avery JK. A variation in cleft palate. J Oral Surg 1957;15:320-4. Fuhrmann W, Koch F, Schweckendick W. Autosomal dominante Vererbung von Gaumenspalte and Synechien zwischen Gaumen und Mundboden oder Zunge. Humangenetik 1972;14:196-203. Preus M, Fraser FC, Fuhrmann W. Cleft palate lateral synechia syndrome without the lateral synechia (CP f LS syndrome). Teratology 1974;9:135-41. Snijman PC, Prinsloo JG. Congenital fusion of the gums. Am J Dis Child 1966;112:593-5. Sternberg N, Sagher U, Golan J, Eidelman AI, Benhur N. Congenital fusion of the gums with bilateral fusion of the temporomandibular joints. Plast Reconstr Surg 1983;72:385/.
8. Mathis VH. Uber einen Fall von Ernlhrungsschwierigkeit bei connataler Syngnathie. Deutsche Zahnarizliche Ze%schrift 1962;17:1167-71. 9. Verdi GD, O’Neal B. Cleft palate and congenital alveolar synechia syndrome. Plastic Reconstructive Surgery 1984;74: 684-5. 10. Shaw WC, Simpson JP. Oral adhesions associated with cleft lip and palate and lip fistulae. Cleft Palate J 1980;7:2-6. Reprint requests to: Dr. James M. Christian Department of Oral and Maxillofacial School of Dentistry West Virginia University Morgantown, WV 26506
Surgery