Cystic lymphangioma with enlarged tongue: A case report David Syracuse,
Marshall, N.
D.D.S.,
and
Daniel
D.
Rabuzzi,
M.D.*
Y.
T
he cystic lymphangioma., “cystic hygroma,” is a congenital abnormality of the lymphatic system. Thought to be due to sequestration of the primitive lymph sacs, these benign tumors are most commonly posterior cervical masses that are present at birth. When laterally placed and well loculated, complete surgical excision is the treatment of choice along with marsupialization of unresectable cysts. Unfortunately, many lymphangiomas send fingerlike processes into surrounding tissues, so that extension into the axilla and mediastinum is not infrequent. When this lesion is sublingual and/or submental in position, bilateral, and especially with invasion of tongue and suprahyroid structures, complete resection is virtually impossible. Over the past 4 years we have had under our care a boy, now 8 years old, with such a remarkably extensive lesion. Because of the massive tongue involvement at birth, the child had undergone an immediate tracheotomy and subsequently had undergone two partial neck resections of the lesion. Surgical trauma to the facial nerve at the second resection had caused a complete peripheral facial paralysis on the left side. When first seen, the boy had massive tongue enlargement, constant drooling, and protrusion with a very obtuse maxillary-mandibular angle. He was unable to breathe with his tracheotomy tube obstructed. On full evaluation, it was noted that the grapelike lymphangioma masses were studded throughout the floor of the mouth and over the soft palate as well as being deeply infiltrated into the tongue. Furthermore, cystic masses were also noted throughout the entire supraglottic larynx. Remarkably, the child could swallow well and there was minimal aspiration. Prior to orthodontic consultation, osteotomy to reduce the mandible was recommended. After examination of the patient with diagnostic aids, it appeared desirable to attempt orthodontic treatment without surgical intervention. A head *Associate Professor of Otorhinolaryngology.
of
Otorhinolaryngology,
Upstate
Medical
Center,
Department
685
Fig. and
1. Pretreatment an enlarged
and tongue.
posttreatment
photographs
of
patient
with
cystic
lymphangioma
cap with a leather chin strap was constructed; this was designed so that pressure was exerted upward and backward. This appliance was worn day and night and adjusted at intervals of 3 weeks. St the time of these adjustments, the elastic pull was changed to control its direction ant1 movement. Toward the end of treatment, the mandible was rctstorcd to its normal position, but a small open-bite remained. Bands were placed on the four permanent incisors in the maxilla and mandible, and by means of interelastics, upper and lower anterior tcctb established a normal relationship. Elastics wcrc diseontinued. At present, the boy is under observation awaiting eruption of the rcmainder of the permanent dentition. Supersaturated saline solution was injected into the tongue on three separate occasions in an effort to cause tissue destruction, with subsequent scarring and decrease in tongue size. These attempts \vcre unsucecssful, possibly because of the marked lvrnI)hangionlatt,us infiltration within the muscle fibers of the tonguc itself. A decision was then ma& to perform ;I ~.edgc rcseetion of the tongue in order to adequately decrease its size. This was pcrformctl 3 years ago and CHtailed V-wetlge resection of’ tbc anterior two thirds of the tongue in the midline with sparing of the lingual artorics. Following uncomplicated healing, it was obvious that the tongue could bc quite readily ac,c~ornmodatct1lat~~l in the arch of the dcformetl mandible.
Chest
(l&16-70)
vozume
71
2. larged following
A, Pretreatment cephalogram of patient with tongue. B, Cephalogram taken after surgical 6 months of orthodontic treatment. C, After
Number
Fig.
6
Cystic lywphazgioma
with enlarged
tofzgue
687
cystic lymphangioma and an enremoval of section of the tongue one year of orthodontic treatment.
Chest Posteroauterior and lateral view of the chest with oblique views of the chest show no evidence of inflammatory disease in the right lowr lobe. The remaining lung fields are clear as well, and the heart is of normal size. Conclusion: Negative chest without evidence for pneumonia. Temporomandibulal joints (lZ-17-70) Coned-down views of both temporomandibular joints, as well as tomographic cuts in anteroposterior and lateral projections, show that there .is marked deformity of the mandible with marked obtuse angulation of the jaw angle. The glenoid fossa of the mandibular condyle is very shallow bilaterally, and there is poor development of the anterior tubercle so that, on all the views, the mandibular condyle is anteriorly placed and the mouth is in an open position. There is no evidence of abnormal erosion or other changes in the temporomandibular joints. Conohsion: Shallow temporomandibular fossae Inlaterally with poor development of the anterior tubercle with the mandibular head in anterior subluxed position. There appears to be some form of hypoplasia of the mandibular fossae l~ilxterally. Discussion
In this case the mandibular body and condylar growth each pursued its own devclopmcntal course. It is apparent that the mandibular body responded to the growth of the associated facial viscera, mainly the tongue; therefore, the growth
of the body of the mandible is essentially independent of the growth of the conclyh.?. Kazanjian’ reported a case of congenital bilateral absence of the mandibular rami. The woman described had a mandibular body of good size and spatial orientation supporting an essentially normal dentition. This proves that the mandibular body not only can grow but can alter its spatial orientation without the existence of a condylar cartilage. Data in papers by Clearer” and LindegardG substant,iatc the theory that growth of the mandibular body and growth of the condyle arc relatively independent events. These findings indicate no significant correlation between the length of the condylar process and the anteroposterior length of t,he mandibular boil>7 (corpus). Further, the work of Baume and Beck@ establishes the same conclusion, stressing the separate courses of growth of the mandibu1a.r body and condyle. Harnat and Rngle,R in 1953, reported on the growth of the mandible following surgical removal of the condyles in Macncrr rhesus monkeys. This procedure affected ramal height alone, and the body length was essentially normal. Our patient now has a relatively stable maxillary-mandibular relationship SO that, with a decrease in tongue size and with the mandible repositioned by orthodontic means, he is able to articulate satisfactorily and to breathe without dcpendence upon his tracheotomy tube. The residual cystic lymphagiomatous tissue on the entire oral larynx, hypopharynx, and cervical areas remains and, indeed, fluctuates in size from time to time. At present, the patient is undergoing cryosurgical therapy to the supraglottic larynx in an attempt to eradicate some of the diseased tissue in this area. REFERENCES
1. Touloukian, R. .J., Iiickert, R. R., Lange, R. C., et al.: The microvascular circulation of lymphangioma: A study of Xe 133 clearance and pathology, Pediatrics 48: 36-40, 1971. 2. Singh, S., Raboo, M. I,., and Pathak, T. C.: Cystic lymphangioma in rhildrcn: Report of 32 cases including lesions at rare sites, Surgery 69: 947-951, 1971. :3. W’ard, I’. H., Harris, I’. F‘., and Downey, W.: Arch. Otolaryngol, 91: 508-514, 1970. 4. Knowles, C. C.: Malocclusion associated with an extensivo lymphangioma of the face, Dent. Pratt. Dent. Rec. 22: 143.146, 1971. 5. Cleaver, F. H.: A contrilmtion to the l)iometric study of thp human mandible, Biometrika 29: 80-112, 1973. 6. Lindegard, R. : Processus alveolaris mandibulae hominis, Odontol. Revy 2: 260.274, 195 1. 7. Kazanjian, V. H.: Bilateral allsence of the ascending rami of the mandible, nr. ;r. Plast. Surg. 9: 77-82, 1956. 8. Samat, H. C’r., and Engle, M. H.: Effect of surgical removal of rondylr on the growing mandible; an experimental study in the Mncncu rhesus monkey, AM. J. ORTHOD. 39: 147, lg5.7. 9. Baume, L. J., and Iseeks, H. : The development of the dentition of ikfacnca mulattn: 1 ts difference from the human pattern, ALI. J. ORTHOD. 36: 723-748, 1950. 1164 I?. Genesee
St.
(lSSl0)
Marshall, N.
D.D.S.,
and
Daniel
D.
Rabuzzi,
M.D.*
Y.
T
he cystic lymphangioma., “cystic hygroma,” is a congenital abnormality of the lymphatic system. Thought to be due to sequestration of the primitive lymph sacs, these benign tumors are most commonly posterior cervical masses that are present at birth. When laterally placed and well loculated, complete surgical excision is the treatment of choice along with marsupialization of unresectable cysts. Unfortunately, many lymphangiomas send fingerlike processes into surrounding tissues, so that extension into the axilla and mediastinum is not infrequent. When this lesion is sublingual and/or submental in position, bilateral, and especially with invasion of tongue and suprahyroid structures, complete resection is virtually impossible. Over the past 4 years we have had under our care a boy, now 8 years old, with such a remarkably extensive lesion. Because of the massive tongue involvement at birth, the child had undergone an immediate tracheotomy and subsequently had undergone two partial neck resections of the lesion. Surgical trauma to the facial nerve at the second resection had caused a complete peripheral facial paralysis on the left side. When first seen, the boy had massive tongue enlargement, constant drooling, and protrusion with a very obtuse maxillary-mandibular angle. He was unable to breathe with his tracheotomy tube obstructed. On full evaluation, it was noted that the grapelike lymphangioma masses were studded throughout the floor of the mouth and over the soft palate as well as being deeply infiltrated into the tongue. Furthermore, cystic masses were also noted throughout the entire supraglottic larynx. Remarkably, the child could swallow well and there was minimal aspiration. Prior to orthodontic consultation, osteotomy to reduce the mandible was recommended. After examination of the patient with diagnostic aids, it appeared desirable to attempt orthodontic treatment without surgical intervention. A head *Associate Professor of Otorhinolaryngology.
of
Otorhinolaryngology,
Upstate
Medical
Center,
Department
685
Fig. and
1. Pretreatment an enlarged
and tongue.
posttreatment
photographs
of
patient
with
cystic
lymphangioma
cap with a leather chin strap was constructed; this was designed so that pressure was exerted upward and backward. This appliance was worn day and night and adjusted at intervals of 3 weeks. St the time of these adjustments, the elastic pull was changed to control its direction ant1 movement. Toward the end of treatment, the mandible was rctstorcd to its normal position, but a small open-bite remained. Bands were placed on the four permanent incisors in the maxilla and mandible, and by means of interelastics, upper and lower anterior tcctb established a normal relationship. Elastics wcrc diseontinued. At present, the boy is under observation awaiting eruption of the rcmainder of the permanent dentition. Supersaturated saline solution was injected into the tongue on three separate occasions in an effort to cause tissue destruction, with subsequent scarring and decrease in tongue size. These attempts \vcre unsucecssful, possibly because of the marked lvrnI)hangionlatt,us infiltration within the muscle fibers of the tonguc itself. A decision was then ma& to perform ;I ~.edgc rcseetion of the tongue in order to adequately decrease its size. This was pcrformctl 3 years ago and CHtailed V-wetlge resection of’ tbc anterior two thirds of the tongue in the midline with sparing of the lingual artorics. Following uncomplicated healing, it was obvious that the tongue could bc quite readily ac,c~ornmodatct1lat~~l in the arch of the dcformetl mandible.
Chest
(l&16-70)
vozume
71
2. larged following
A, Pretreatment cephalogram of patient with tongue. B, Cephalogram taken after surgical 6 months of orthodontic treatment. C, After
Number
Fig.
6
Cystic lywphazgioma
with enlarged
tofzgue
687
cystic lymphangioma and an enremoval of section of the tongue one year of orthodontic treatment.
Chest Posteroauterior and lateral view of the chest with oblique views of the chest show no evidence of inflammatory disease in the right lowr lobe. The remaining lung fields are clear as well, and the heart is of normal size. Conclusion: Negative chest without evidence for pneumonia. Temporomandibulal joints (lZ-17-70) Coned-down views of both temporomandibular joints, as well as tomographic cuts in anteroposterior and lateral projections, show that there .is marked deformity of the mandible with marked obtuse angulation of the jaw angle. The glenoid fossa of the mandibular condyle is very shallow bilaterally, and there is poor development of the anterior tubercle so that, on all the views, the mandibular condyle is anteriorly placed and the mouth is in an open position. There is no evidence of abnormal erosion or other changes in the temporomandibular joints. Conohsion: Shallow temporomandibular fossae Inlaterally with poor development of the anterior tubercle with the mandibular head in anterior subluxed position. There appears to be some form of hypoplasia of the mandibular fossae l~ilxterally. Discussion
In this case the mandibular body and condylar growth each pursued its own devclopmcntal course. It is apparent that the mandibular body responded to the growth of the associated facial viscera, mainly the tongue; therefore, the growth
of the body of the mandible is essentially independent of the growth of the conclyh.?. Kazanjian’ reported a case of congenital bilateral absence of the mandibular rami. The woman described had a mandibular body of good size and spatial orientation supporting an essentially normal dentition. This proves that the mandibular body not only can grow but can alter its spatial orientation without the existence of a condylar cartilage. Data in papers by Clearer” and LindegardG substant,iatc the theory that growth of the mandibular body and growth of the condyle arc relatively independent events. These findings indicate no significant correlation between the length of the condylar process and the anteroposterior length of t,he mandibular boil>7 (corpus). Further, the work of Baume and Beck@ establishes the same conclusion, stressing the separate courses of growth of the mandibu1a.r body and condyle. Harnat and Rngle,R in 1953, reported on the growth of the mandible following surgical removal of the condyles in Macncrr rhesus monkeys. This procedure affected ramal height alone, and the body length was essentially normal. Our patient now has a relatively stable maxillary-mandibular relationship SO that, with a decrease in tongue size and with the mandible repositioned by orthodontic means, he is able to articulate satisfactorily and to breathe without dcpendence upon his tracheotomy tube. The residual cystic lymphagiomatous tissue on the entire oral larynx, hypopharynx, and cervical areas remains and, indeed, fluctuates in size from time to time. At present, the patient is undergoing cryosurgical therapy to the supraglottic larynx in an attempt to eradicate some of the diseased tissue in this area. REFERENCES
1. Touloukian, R. .J., Iiickert, R. R., Lange, R. C., et al.: The microvascular circulation of lymphangioma: A study of Xe 133 clearance and pathology, Pediatrics 48: 36-40, 1971. 2. Singh, S., Raboo, M. I,., and Pathak, T. C.: Cystic lymphangioma in rhildrcn: Report of 32 cases including lesions at rare sites, Surgery 69: 947-951, 1971. :3. W’ard, I’. H., Harris, I’. F‘., and Downey, W.: Arch. Otolaryngol, 91: 508-514, 1970. 4. Knowles, C. C.: Malocclusion associated with an extensivo lymphangioma of the face, Dent. Pratt. Dent. Rec. 22: 143.146, 1971. 5. Cleaver, F. H.: A contrilmtion to the l)iometric study of thp human mandible, Biometrika 29: 80-112, 1973. 6. Lindegard, R. : Processus alveolaris mandibulae hominis, Odontol. Revy 2: 260.274, 195 1. 7. Kazanjian, V. H.: Bilateral allsence of the ascending rami of the mandible, nr. ;r. Plast. Surg. 9: 77-82, 1956. 8. Samat, H. C’r., and Engle, M. H.: Effect of surgical removal of rondylr on the growing mandible; an experimental study in the Mncncu rhesus monkey, AM. J. ORTHOD. 39: 147, lg5.7. 9. Baume, L. J., and Iseeks, H. : The development of the dentition of ikfacnca mulattn: 1 ts difference from the human pattern, ALI. J. ORTHOD. 36: 723-748, 1950. 1164 I?. Genesee
St.
(lSSl0)
