Submandibular cystic hygroma resembling a plunging ranula in a neonate Review and report
of a case
Thomas E. Osborne, DDS,a J. Alex Hailer, MD,b L. Stefan Levin, DDS, MSD.“~ Barbara J. Little, MD,d and Karen E. King, MD,e Atlanta, Ga., and Baltimore, Md. THE JOHNS HOPKINS SCHOOL OF MEDICINE Cystic hygromas are large lymphangiomas that are most often found in the posterior triangle of the neck and the axilla in children. They are most frequently found before age 2 and may be massive. After upper respiratory infection, they may become infected and enlarged, causing dysphagia and toxemia. The diagnosis can usually be made by history and physical examination and confirmed by biopsy. Treatment is by surgical excision of small lesions and staged debulking excisions in more severe cases. A patient with a cystic hygroma having many clinical characteristics of a plunging ranula is presented. The cyst fluid was aspirated and analyzed for its amylase, sodium, potassium, chloride, urea nitrogen, glucose, and total protein content. The characteristics of the fluid were atso compared with those of lymph and saliva. This report demonstrates the difficulty in determining the diagnosis of a tumor that has the clinical features of a cystic hygroma, as well as a plunging ranuta. The necessity of a proper presurgical diagnosis is essential since the form of therapy for each is different and conflicting. A method that distinguishes between the cervical cystic hygroma and a plunging ranula by means of aspirated fluid is discussed. (ORAL SURC ORAL MED ORAL PATHOL 1991;71:16-20)
C
ystic hygromas are large lymphangiomas that are most often found in the posterior triangle of the neck and axilla in children. Less frequently, they are located in the mediastinum, mesentery, colon, and retroperitoneum.’ These lesions are composed of multiple endothelium-lined, lymph-filled spaces and may be as large as 18 cm in the neck and submandibular areas in some infants.* Cystic hygromas are usually infiltrative, often separating fascial planes and incorporating nerves, muscle, and blood vessels.3 Fifty percent of cystic hygromas are present at Vlinical Assistant Professor of Oral and Maxillofacial Surgery, Emory University School of Postgraduate Dentistry, Atlanta; in private practice, Atlanta. bRobert Garrett Professor of Pediatric Surgery; Director, Division of Pediatric Surgery, The Johns Hopkins University School of Medicine, Baltimore. cAssociate Professor, Department of Otolaryngology-Head and Neck Surgery, Division of Dentistry and Oral and Maxillofacial Surgery; Assistant Professor, Department of Medicine (Genetics); Joint Appointment, Department of Dermatology; The Johns Hopkins University School of Medicine, Baltimore. dResident, Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore. eResident, Department of Laboratory Medicine, The Johns Hop kins University School of Medicine, Baltimore. 7/12/15716
16
birth, and 90% are evident by age 2. ‘3 4 They are usually easily compressible, painless enlargements that are multilocular and transilluminate well.5 At birth, some are very large whereas others are not noticeable and grow proportionately with the child. Many cystic hygromas grow steadily and attain their greatest size by the second or third year of age; others grow slowly, have periods of dormancy, or may even diminish in size.6 Growth is usually self-limited, but there is no way to predict ultimate size or when growth will cease. Spontaneous remissions are rare.‘, 3*7 There have been few case reports of cystic hygromas developing in adulthood.*-” Cystic hygromas often enlarge rapidly secondary to an upper respiratory infection. Under these circumstances, the lesion may become tense, indurated, painful, erythematous, and develop purulent content. These changes may lead to dysphagia, respiratory embarrassment, and life-threatening airway obstruction. The enlarging lymphangioma separates muscle bundles, vessels, and nerves, leading to necrosis and increasing inflammation. 11-14Because cystic hygromas are highly vascular, acute hemorrhage into the cystic areas and toxemia may develop.i2,t5 In the preantibiotic era, these sequelae caused a high mortality rate; they remain a serious comp1ication.i6 Complete surgical excision is the treatment of
Volume Number
Submandibular
71 1
Fig.
cystic hygroma
17
2. Postoperativeview of patient 2 monthsafter sur-
gery.
agnosis. By physical examination alone, it was im-
possible to distinguish between the two most likely lesions-a cervical cystic hygroma and a congenital plunging ranula-in the differential diagnosis. A method to facilitate obtaining the correct diagnosis is presented. CASE REPORT Fig.
1. A, Preoperative facial view of patient. Note
cleft lip and submandibular swelling of cystic hygroma in the right submandibular area. B, Preoperative intraoral view of patient showing elevation of floor of mouth and displacementof tongue.
choice for cystic hygromas and offers the best chance for cure. Smaller lesions should be completely removed as early as possible to minimize future growth and recurrence. Large and complex lesions are often intimately associated with vital structures such as cranial nerves and major vessels of the head and neck; multiple-staged excisions and debulking procedures are often needed. Other treatment modalities have included aspiration and incision and drainage, radiation, and sclerosis. None of these forms of therapy has proven effective in eradicating these lesions.Incision
and drainage may be used in emergency situations to decompress the tumor; surgical excision is performed later. Repeated incision and drainage may lead to life-threatening infection.*’ ‘, I71I8 In this report an infant is described with a submandibular lesion that presented difficulties in di-
An 1l-day-old white maleinfant wasreferred for evaluation of an enlargingmassin the left submandibulararea. At birth, it wasnotedthat an “excessiveamount of skin” waspresentunderthe left body of the mandible.In addition, he wasborn with a right cleft lip and associatednasaldeformity. He wasdischarged,andtheparentswereinstructed to seekfurther consultationwith respectto surgicalreconstruction of the cleft lip and not to be concernedabout the excessskin under the mandible.Shortly thereafter, an upper respiratory tract infection developed,and the areaunder the left mandibleenlarged.On evaluation,a soft, moderately well-circumscribed,5 X 4.5 cm, mobile masswas observedin the left midsubmandibular spaceanterior to the angleof the mandible(Fig. 1, A). The lesionwasnot fixed to skin or boneand transilluminatedwell. The tonguewas raisedlaterally andslightly anteriorly by a blue cystlike lesionin the sublingualspace(Fig. 1, B). The lesiondid not impairthe patient’sability to breatheandnurseandwasnot painful to palpation. Consultationwasobtained from severalspecialties,including oral and maxillofacial surgery, pediatric surgery, andoral pathology.The differential diagnosisincludedcystic hygroma and a congenitalplunging ranula. Since the patient wasnot in distress,it wasdecidedthat surgicalexcisionshouldnot be done until age 6 months.This delay would allow the immunologicsystemto mature and the
18 Osborne et al.
Fig. 3. Excised tumor. Note multilocular character of large cyst and presenceof smallercysts.
child to produce his own antibodies. The patient was discharged,and closefollow-up wasarranged. Two dayslater the patient wasadmitted to the hospital becausethe tumor had enlargedand wascausingdifficulty in feeding.There wasno airway embarrassment. Anteriorposteriorand lateral neck soft tissueradiographswereobtained. A round submandibularsoft tissuemasswasnoted on the left side.The tongueon the lateral radiographabutted the soft palate,but no other airway abnormalitieswere identified. Sincethe lesionwasenlarging,it waselectedto excisethe cystic massand repair the cleft lip. With the patient undergeneralanesthesia,10ml of clear straw-coloredfluid wasaspiratedfrom the lesion.The fluid did not froth onagitation but did coagulateonstanding.The samplewasanalyzed for electrolytes,amylase,urea nitrogen,glucose,and total protein(Table 1).The resultsof these studiesconfirmedthe diagnosisof cystic hygroma,andthus the lesionwassurgicallyremoved.On excisionwith the use of an extraoral approach,the masswasfound to be located underthe platysmamuscle,and it descended to the lateral aspectof the hyoid bone.It alsoextendedsuperiorlyinto the sublingualspace(Fig. 2). In addition to the main cystic mass,severalsmallercysts along the lateral aspectof the larger cyst were removed(Fig. 3). After excision of the cystic mass,the cleft lip was repaired.The patient toleratedthe surgerywell, and there werenopostoperativecomplications.At 6-monthfollow-up, there wasno recurrenceof the mass;healingof the cleft lip hasbeenexcellent (Fig. 2). The surgicalspecimenwascomposedprimarily of many irregularly shaped,thin-walled vascularspacesof various sizes(Fig. 4). Thesespaceswerelined with spindle-shaped endothelialcells,and in someinstancescontainedeosinophilic material, interpretedaslymph. Looseconnectivetissue with moderatenumbersof spindle-shaped fibroblasts separatedthe lymphatic channels.A few of thesechannels werealsofoundbetweenthe adjacentsalivary glandlobules. No significantinflammatory componentwasseen.The diagnosisof lymphangioma(cystic hygroma) wasmade.
Fig. 4. Photomicrographof cystic hygroma. Numerous thin-walled vascular spacesare noted. (Original magnihcation, X55.) DISCUSSION
The diagnosis of cystic hygroma can usually be made on physical examination. Any mass in the neck that is large, lobulated, and blue-gray; transilluminates well; and is covered with thin skin that is not adherent to the lesion should be suspected to be a cystic hygroma, especially if the patient is under 2 years of age. ‘14 The thyroglossal tract cyst, benign cystic lymph node, epidermal inclusion cyst, lipoma, hemangioma, and ranula should be considered in the differential diagnosis.“, ** The thyroglossal tract cyst is smaller than the cystic hygroma, has more well-defined borders, does not transilluminate well, and elevates when the tongue is protruded.9* I4 Although usually in the midline, almost 20% of the thyroglossal tract cysts are lateral but nonetheless close to the midline.20 However, the cystic hygroma, if in the midline, will always have extensions well into one side of the neck.14 The thyroglossal tract cyst occasionally becomes acutely inflamed and enlarged; however, it does so less frequently and less severely than the cystic hygroma.14 The benign cystic lymph node is usually found along the anterior edge of the sternocleidomastoid muscle, more commonly the lower third. It occurs most commonly in young adults and occasionally becomes enlarged secondary to upper respiratory infection. In constrast to the cystic hygroma, it is usually smaller, firmer in consistency, well circumscribed, and often has attachments to deep cervical structures9, I4 The liquid content of the benign cystic lymph node is usually thick.14 The epidermal inclusion cyst is superficial, adheres to the skin, and has a doughy consistency.’ Lipomas have margins that are less well defined than cystic hygromas, are not fluctuant, grow more slowly, and are not subject to inflammatory exacerbations.14 They may, however, like cystic hygromas, transmit
Submandibular
Volume 7 1 Number 1
cystic hygroma
19
Analysis of cystic hygroma fluid compared with serum and saliva Consistent
Amylase (U/L) Sodium (mEq/L) Potassium (mEq/L) Chloride (mEq/L) Urea nitrogen (mg/ 100 ml) Glucose (mg/ 100 ml) Total protein (mg/ 100 ml)
Fluid from cystic hygroma
4 140 4.1 111 3.0 93 3.7
*From Geigy -. Scientific Tables, West Caldwell, N.J., Ciba-Geigy, tReference
Normal serum values*
60-180 135-145 3.5-5.0 95-105 IO-15 75-105 5.5-8.0
Normal salivary (submandibular
values gland?)
1IO-330 21 17 20 7
of a case
Thomas E. Osborne, DDS,a J. Alex Hailer, MD,b L. Stefan Levin, DDS, MSD.“~ Barbara J. Little, MD,d and Karen E. King, MD,e Atlanta, Ga., and Baltimore, Md. THE JOHNS HOPKINS SCHOOL OF MEDICINE Cystic hygromas are large lymphangiomas that are most often found in the posterior triangle of the neck and the axilla in children. They are most frequently found before age 2 and may be massive. After upper respiratory infection, they may become infected and enlarged, causing dysphagia and toxemia. The diagnosis can usually be made by history and physical examination and confirmed by biopsy. Treatment is by surgical excision of small lesions and staged debulking excisions in more severe cases. A patient with a cystic hygroma having many clinical characteristics of a plunging ranula is presented. The cyst fluid was aspirated and analyzed for its amylase, sodium, potassium, chloride, urea nitrogen, glucose, and total protein content. The characteristics of the fluid were atso compared with those of lymph and saliva. This report demonstrates the difficulty in determining the diagnosis of a tumor that has the clinical features of a cystic hygroma, as well as a plunging ranuta. The necessity of a proper presurgical diagnosis is essential since the form of therapy for each is different and conflicting. A method that distinguishes between the cervical cystic hygroma and a plunging ranula by means of aspirated fluid is discussed. (ORAL SURC ORAL MED ORAL PATHOL 1991;71:16-20)
C
ystic hygromas are large lymphangiomas that are most often found in the posterior triangle of the neck and axilla in children. Less frequently, they are located in the mediastinum, mesentery, colon, and retroperitoneum.’ These lesions are composed of multiple endothelium-lined, lymph-filled spaces and may be as large as 18 cm in the neck and submandibular areas in some infants.* Cystic hygromas are usually infiltrative, often separating fascial planes and incorporating nerves, muscle, and blood vessels.3 Fifty percent of cystic hygromas are present at Vlinical Assistant Professor of Oral and Maxillofacial Surgery, Emory University School of Postgraduate Dentistry, Atlanta; in private practice, Atlanta. bRobert Garrett Professor of Pediatric Surgery; Director, Division of Pediatric Surgery, The Johns Hopkins University School of Medicine, Baltimore. cAssociate Professor, Department of Otolaryngology-Head and Neck Surgery, Division of Dentistry and Oral and Maxillofacial Surgery; Assistant Professor, Department of Medicine (Genetics); Joint Appointment, Department of Dermatology; The Johns Hopkins University School of Medicine, Baltimore. dResident, Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore. eResident, Department of Laboratory Medicine, The Johns Hop kins University School of Medicine, Baltimore. 7/12/15716
16
birth, and 90% are evident by age 2. ‘3 4 They are usually easily compressible, painless enlargements that are multilocular and transilluminate well.5 At birth, some are very large whereas others are not noticeable and grow proportionately with the child. Many cystic hygromas grow steadily and attain their greatest size by the second or third year of age; others grow slowly, have periods of dormancy, or may even diminish in size.6 Growth is usually self-limited, but there is no way to predict ultimate size or when growth will cease. Spontaneous remissions are rare.‘, 3*7 There have been few case reports of cystic hygromas developing in adulthood.*-” Cystic hygromas often enlarge rapidly secondary to an upper respiratory infection. Under these circumstances, the lesion may become tense, indurated, painful, erythematous, and develop purulent content. These changes may lead to dysphagia, respiratory embarrassment, and life-threatening airway obstruction. The enlarging lymphangioma separates muscle bundles, vessels, and nerves, leading to necrosis and increasing inflammation. 11-14Because cystic hygromas are highly vascular, acute hemorrhage into the cystic areas and toxemia may develop.i2,t5 In the preantibiotic era, these sequelae caused a high mortality rate; they remain a serious comp1ication.i6 Complete surgical excision is the treatment of
Volume Number
Submandibular
71 1
Fig.
cystic hygroma
17
2. Postoperativeview of patient 2 monthsafter sur-
gery.
agnosis. By physical examination alone, it was im-
possible to distinguish between the two most likely lesions-a cervical cystic hygroma and a congenital plunging ranula-in the differential diagnosis. A method to facilitate obtaining the correct diagnosis is presented. CASE REPORT Fig.
1. A, Preoperative facial view of patient. Note
cleft lip and submandibular swelling of cystic hygroma in the right submandibular area. B, Preoperative intraoral view of patient showing elevation of floor of mouth and displacementof tongue.
choice for cystic hygromas and offers the best chance for cure. Smaller lesions should be completely removed as early as possible to minimize future growth and recurrence. Large and complex lesions are often intimately associated with vital structures such as cranial nerves and major vessels of the head and neck; multiple-staged excisions and debulking procedures are often needed. Other treatment modalities have included aspiration and incision and drainage, radiation, and sclerosis. None of these forms of therapy has proven effective in eradicating these lesions.Incision
and drainage may be used in emergency situations to decompress the tumor; surgical excision is performed later. Repeated incision and drainage may lead to life-threatening infection.*’ ‘, I71I8 In this report an infant is described with a submandibular lesion that presented difficulties in di-
An 1l-day-old white maleinfant wasreferred for evaluation of an enlargingmassin the left submandibulararea. At birth, it wasnotedthat an “excessiveamount of skin” waspresentunderthe left body of the mandible.In addition, he wasborn with a right cleft lip and associatednasaldeformity. He wasdischarged,andtheparentswereinstructed to seekfurther consultationwith respectto surgicalreconstruction of the cleft lip and not to be concernedabout the excessskin under the mandible.Shortly thereafter, an upper respiratory tract infection developed,and the areaunder the left mandibleenlarged.On evaluation,a soft, moderately well-circumscribed,5 X 4.5 cm, mobile masswas observedin the left midsubmandibular spaceanterior to the angleof the mandible(Fig. 1, A). The lesionwasnot fixed to skin or boneand transilluminatedwell. The tonguewas raisedlaterally andslightly anteriorly by a blue cystlike lesionin the sublingualspace(Fig. 1, B). The lesiondid not impairthe patient’sability to breatheandnurseandwasnot painful to palpation. Consultationwasobtained from severalspecialties,including oral and maxillofacial surgery, pediatric surgery, andoral pathology.The differential diagnosisincludedcystic hygroma and a congenitalplunging ranula. Since the patient wasnot in distress,it wasdecidedthat surgicalexcisionshouldnot be done until age 6 months.This delay would allow the immunologicsystemto mature and the
18 Osborne et al.
Fig. 3. Excised tumor. Note multilocular character of large cyst and presenceof smallercysts.
child to produce his own antibodies. The patient was discharged,and closefollow-up wasarranged. Two dayslater the patient wasadmitted to the hospital becausethe tumor had enlargedand wascausingdifficulty in feeding.There wasno airway embarrassment. Anteriorposteriorand lateral neck soft tissueradiographswereobtained. A round submandibularsoft tissuemasswasnoted on the left side.The tongueon the lateral radiographabutted the soft palate,but no other airway abnormalitieswere identified. Sincethe lesionwasenlarging,it waselectedto excisethe cystic massand repair the cleft lip. With the patient undergeneralanesthesia,10ml of clear straw-coloredfluid wasaspiratedfrom the lesion.The fluid did not froth onagitation but did coagulateonstanding.The samplewasanalyzed for electrolytes,amylase,urea nitrogen,glucose,and total protein(Table 1).The resultsof these studiesconfirmedthe diagnosisof cystic hygroma,andthus the lesionwassurgicallyremoved.On excisionwith the use of an extraoral approach,the masswasfound to be located underthe platysmamuscle,and it descended to the lateral aspectof the hyoid bone.It alsoextendedsuperiorlyinto the sublingualspace(Fig. 2). In addition to the main cystic mass,severalsmallercysts along the lateral aspectof the larger cyst were removed(Fig. 3). After excision of the cystic mass,the cleft lip was repaired.The patient toleratedthe surgerywell, and there werenopostoperativecomplications.At 6-monthfollow-up, there wasno recurrenceof the mass;healingof the cleft lip hasbeenexcellent (Fig. 2). The surgicalspecimenwascomposedprimarily of many irregularly shaped,thin-walled vascularspacesof various sizes(Fig. 4). Thesespaceswerelined with spindle-shaped endothelialcells,and in someinstancescontainedeosinophilic material, interpretedaslymph. Looseconnectivetissue with moderatenumbersof spindle-shaped fibroblasts separatedthe lymphatic channels.A few of thesechannels werealsofoundbetweenthe adjacentsalivary glandlobules. No significantinflammatory componentwasseen.The diagnosisof lymphangioma(cystic hygroma) wasmade.
Fig. 4. Photomicrographof cystic hygroma. Numerous thin-walled vascular spacesare noted. (Original magnihcation, X55.) DISCUSSION
The diagnosis of cystic hygroma can usually be made on physical examination. Any mass in the neck that is large, lobulated, and blue-gray; transilluminates well; and is covered with thin skin that is not adherent to the lesion should be suspected to be a cystic hygroma, especially if the patient is under 2 years of age. ‘14 The thyroglossal tract cyst, benign cystic lymph node, epidermal inclusion cyst, lipoma, hemangioma, and ranula should be considered in the differential diagnosis.“, ** The thyroglossal tract cyst is smaller than the cystic hygroma, has more well-defined borders, does not transilluminate well, and elevates when the tongue is protruded.9* I4 Although usually in the midline, almost 20% of the thyroglossal tract cysts are lateral but nonetheless close to the midline.20 However, the cystic hygroma, if in the midline, will always have extensions well into one side of the neck.14 The thyroglossal tract cyst occasionally becomes acutely inflamed and enlarged; however, it does so less frequently and less severely than the cystic hygroma.14 The benign cystic lymph node is usually found along the anterior edge of the sternocleidomastoid muscle, more commonly the lower third. It occurs most commonly in young adults and occasionally becomes enlarged secondary to upper respiratory infection. In constrast to the cystic hygroma, it is usually smaller, firmer in consistency, well circumscribed, and often has attachments to deep cervical structures9, I4 The liquid content of the benign cystic lymph node is usually thick.14 The epidermal inclusion cyst is superficial, adheres to the skin, and has a doughy consistency.’ Lipomas have margins that are less well defined than cystic hygromas, are not fluctuant, grow more slowly, and are not subject to inflammatory exacerbations.14 They may, however, like cystic hygromas, transmit
Submandibular
Volume 7 1 Number 1
cystic hygroma
19
Analysis of cystic hygroma fluid compared with serum and saliva Consistent
Amylase (U/L) Sodium (mEq/L) Potassium (mEq/L) Chloride (mEq/L) Urea nitrogen (mg/ 100 ml) Glucose (mg/ 100 ml) Total protein (mg/ 100 ml)
Fluid from cystic hygroma
4 140 4.1 111 3.0 93 3.7
*From Geigy -. Scientific Tables, West Caldwell, N.J., Ciba-Geigy, tReference
Normal serum values*
60-180 135-145 3.5-5.0 95-105 IO-15 75-105 5.5-8.0
Normal salivary (submandibular
values gland?)
1IO-330 21 17 20 7
