This preprint is under consideration at BMC Pediatrics. Preprints are preliminary reports that have not undergone peer review. They should not be considered conclusive, used to inform clinical practice, or referenced by the media as validated information.
Learn more about In Review
Research article
Ultrasonographic diagnosis, classification, and treatment of cervical lymphatic malformation in paediatric patients: a retrospective study
Jiaoling Li
Guangzhou University
Corresponding Author
ORCiD: https://orcid.org/0000-0002-8343-1310
License:
This work is licensed under a Creative Commons Attribution 4.0 International License. Read Full License
Background: To explore the imaging features, key diagnostic points, classification, treatment, and prognosis of cervical lymphatic malformation.
Methods: Overall, 320 patients diagnosed with cervical lymphatic malformation were retrospectively analysed in our hospital between 1 January 2014 and 31 December 2017. Imaging modalities included colour Doppler ultrasound, magnetic resonance imaging, and contrast-enhanced computed tomography. Cervical lymphatic malformations were classified by cyst diameter. Treatments included interventional therapy, surgery, and expectant treatment.
Results: Cervical lymphatic malformation was identified in 320 of 1,192 patients with lymphatic malformation, the sensitivity of ultrasonographic diagnosis was 99.38% (318/320). Cervical lymphatic malformation was classified as mixed, macrocystic, and microcystic in 184 (57.5%), 117 (36.56%), and 19 (5.94%) patients, respectively. Sixty-four (20%), ten (3.12%), seven (2.19%), and three (0.94%) patients experienced intracystic haemorrhage, infection, concurrent intracystic haemorrhage and infection, and calcification, respectively. Among 260 (81.25%) patients who underwent interventional sclerotherapy, 163 (50.94%) received it once and 96 (30%) received it two or more times. Twenty-eight (8.75%), five (1.56%), and 27 (8.44%) patients underwent surgical resection, interventional sclerotherapy plus surgery, and expectant management, respectively.
Conclusions: Ultrasonography is useful for diagnosing definitely cervical lymphatic malformation. Interventional therapy is the first choice for children with confirmed cervical lymphatic malformation.
Keywords
Cervical lymphatic malformation, classification, diagnosis, magnetic resonance imaging, treatment, ultrasound.
Figure 1
Figure 2
Cervical lymphatic malformation is the most common lymphatic malformation and the most common cervical vascular malformation in children,1-3 with an incidence of 1.2–2.8 per 1,000 individuals. It develops prenatally and can be diagnosed by prenatal ultrasonography in 50–65% of patients but by postnatal ultrasonography in 90% of patients. It is not typically diagnosed in adults. Surgical and other trauma can destroy the lymphatic drainage system, leading to acquired lymphatic malformation; other causes are also possible.4,5 Its clinical manifestations depend on the infiltration location and growth rate, and approximately 15–70% of patients have mild symptoms.6-8 Diagnosis is based on intracystic fluid echoic, a thick of cystic wall, and the cyst location; additionally, medical history and clinical manifestations are helpful for diagnosis.
Cervical lymphatic malformation can present as unilocular or multilocular cysts, and the cyst diameter varies from few millimetres to several centimetres.9 Therefore, it can be classified as macrocystic, microcystic, or a mixed type according to the cyst diameter. Although cervical lymphatic malformations are benign, they can invade adjacent critical structures, which increases the difficulty of surgical resection and the risk of postoperative recurrence. The malformations may also threaten the function of adjacent or affected tissues, organs, and as a result, appropriate treatment may be avoided.10,11 Cervical lymphatic malformations can predispose an individual to complications such as haemorrhage or infection, which further affect respiratory function and endanger life.12-14
Multiple imaging methods should be used to classify and make differential diagnoses of cervical lymphatic malformations, monitor lesion progression, and assess the relationship of the lesion with adjacent structures to guide the optimal treatment method selection,15,16 reduce complications and recurrence, and avoid injury.
The aim of this retrospective single-centre study was to explore the ultrasonographic characteristics of patients with cervical lymphatic malformations in southern China and summarise key points of their diagnosis, differential diagnosis, classification, and treatment selection as well as analyse the causes of misdiagnoses and missed diagnoses.
This study was approved by our Institutional Ethics Review Board. Parents of the children provided informed consent for ultrasonography and treatment options.
Patient selection: Records in the large data centre at our hospital were searched and data on patients diagnosed with lymphatic malformation or cervical lymphatic malformation between 1 January 2014 and 31 December 2017 were collected. We used “lymphatic malformation” and “cervical lymphatic malformation” as keywords in the search. Three hundred twenty-two patients were initially identified; four were excluded due to misdiagnosis by ultrasonography, and two previously excluded patients were included: one was due to a missed diagnosis and the other developed cervical lymphatic malformation after pyriform cyst surgery. Therefore, 320 patients with confirmed cervical lymphatic malformation were included in the analysis. The mean age of the patients was 2 years and 29 days (range, 1 day to 14 years). Thirty-five patients were prenatally identified as having cervical lymphatic malformation. Twenty-seven, 260, 5, and 28 patients underwent a follow-up ultrasonographic assessment once every two months at >6 months after diagnosis, intraoperative puncture result assessment, intraoperative puncture and postoperative pathological confirmation, and postoperative pathological confirmation of the diagnosis (regarded as the gold standard to determine the coincidence rate between image-based and puncture results or pathological diagnoses), respectively.
Imaging, diagnosis, and classification: A high-resolution ultrasound system, equipped with a 4–15 MHz transducer (Acuson S2000; Siemens, Hamberg, Germany), was used to diagnose cervical lymphatic malformation. Patients in whom the relationship between the lesion and adjacent structures was not clear by ultrasonography because of individual cysts <1 mm in diameter underwent further evaluation by magnetic resonance imaging (MRI) (Signa; GE Medical Systems, Milwaukee, WI, USA) or contrast-enhanced computed tomography (CT). All patients underwent colour Doppler and power Doppler ultrasonography. Diagnosis and differential diagnosis were based on the intracystic fluid echo, thickness of cyst wall, cyst location, and colour flow signal distribution. The macrocystic (cysts >1 cm in diameter), microcystic (individual cysts <1 cm in diameter), and mixed types were classified according to the cyst diameter. Cervical lymphatic malformation was diagnosed with haemorrhage, infection, and/or calcification according to increased cyst diameter (compared with prior size) and intracyst fine spot echo, local pain, increased temperature and abnormal blood test results, and calcified plaque in cyst before treatment, respectively.
Treatments: Patients were treated with interventional sclerotherapy, surgery, or surgery combined with interventional therapy.
The principles and methods of interventional sclerotherapy17 (using bleomycin at the recommended dose of 0.5 mg/kg in an aqueous solution of 1.5 mg/mL) were as follows: (1) to prevent the side effects of sclerosing agents in infants, patients were >6 months old unless symptoms of oppression occurred; (2) individual cysts had to be >1 cm in diameter; (3) imaging findings should suggest infiltration of the lesion into adjacent critical structures; (4) Patients with cervical lymphatic malformation patients complicated by infection first had to undergo anti-inflammatory treatment for 3–5 days before a sclerosing agent will be injected into the lesion; (5) For abscess formation complicating cervical lymphatic malformation, ultrasound-guided puncture and drainage was administered to patients followed by a simultaneous injection of a sclerosing agent; (6) cervical lymphatic malformation patients with haemorrhage first had to undergo haemostasis, after which a sclerosing agent was injected into the lesion; and (7) for patients without complications, a sclerosing agent had to be injected into the lesion or they had to undergo follow-up ultrasonography.
If the lesion did not progress, expectant treatment (namely routine observation) continued. For patients in whom the lesion increased or hardened (compared with prior size or texture), interventional sclerotherapy or surgery was performed, with routine blood tests, screening of the coagulation status, and liver function tests. For all patients who underwent interventional sclerotherapy, the puncture liquid was smeared for definite diagnosis and a contrast medium was injected to determine the size of the cyst. If the cysts were interconnected, we chose the dose of the contrast medium that excluded lymphovascular malformations.
Patients with individual cysts <1 cm in diameter underwent surgical treatment, and the determinant for surgery combined with interventional therapy was lesion size more than 4 cm in diameter. The interventional therapy targets residual lesions to prevent toxic side effects caused by excessive use of sclerosing agents.
Cervical lymphatic malformation was identified in 320 of the 1,192 patients with lymphatic malformation examined during our observation period with 189 males and 131 females (1.4:1 male-to-female ratio). the sensitivity of ultrasonographic diagnosis was 99.38% (318/320). All 320 patients had undergone further evaluation by colour Doppler ultrasonography and power Doppler ultrasonography, with 43 patients further assessed by MRI and 55 by contrast-enhanced CT. Details regarding the types of complications and medical management are summarised in Tables 1 and 2. Complications occurred in 84 patients (26.25%); of them, 64 (20%) experienced haemorrhage, 10 (3.12%) developed infections, 7 (2.19%) had concurrent haemorrhage and infection, and 3 (0.94%) exhibited calcification. Of the 260 patients (81.25%) who underwent sclerotherapy, 163 (50.94%) underwent bleomycin sclerotherapy once and 96 (30%) underwent bleomycin sclerotherapy two or more times, with one patient (0.31%) receiving seven rounds. Twenty-eight patients (8.75%) received surgical treatment and five (1.56%) received concurrent surgical treatment and bleomycin sclerotherapy. Expectant management was provided to the other 27 patients (8.44%).
The ultrasonographic features and treatments of one representative patient with cervical lymphatic malformation and those of the patient misdiagnosed with lipoblastoma are shown in Figures 1 and 2. Of the 320 patients, only 19 (5.94%) showed sparse flow signals on the cyst wall and septations.
Among the 28 patients who underwent surgical treatment, only one experienced branchial cleft fistula. Of the 260 patients who underwent bleomycin sclerotherapy, only one developed an infection. All lesions gradually resolved, with the longest taking 2 years. Two patients with pyriform cysts, one patient with a dermoid cyst, and one patient with lipoblastoma were misdiagnosed with cervical lymphatic malformation, whereas one patient with cervical lymphatic malformation was misdiagnosed as having a pyriform cyst.
Main findings
In this retrospective study of 320 patients, including one patient who developed cervical lymphatic malformation after pyriform cyst surgery and one patient who was misdiagnosed by ultrasonography, the sensitivity of which was 99.38%, the incidence of cervical lymphatic malformation complications was 26.25% before treatment. One patient developed a branchial cleft fistula and another developed an infection; the total success rate of treatment was 99.38%.
Interpretation: Cervical lymphatic malformation, a benign congenital disease, is caused by non-transportation of the lymphatic and venous systems, abnormal hyperplasia of the lymphatic epithelium, or lymphatic obstruction. Additionally, surgical trauma to the neck can destroy the lymphatic drainage system, resulting in acquired cervical lymphatic malformation.18 In this study, it was found that cervical lymphatic malformation also developed as a complication of pyriform cyst surgery.
Lymphatic malformations can occur in all areas of the body, but 75% occur in the neck.19,20 In this study, cervical lymphatic malformation was found to constitute 26.85% of all lymphatic malformation cases (320/1,192). This rate is lower than that observed previously, where foetal cervical lymphatic malformation comprised 63.29% of all lymphatic malformation cases. This may be because more pregnancies were terminated due to cervical lymphatic malformations accompanied by severe structural or chromosomal abnormalities in the foetus, or because some lesions subsided naturally.21 Two-dimensional ultrasonography showed that most cervical lymphatic malformations were multilocular cysts; large cysts had a fine separation, and small cysts had a coarse separation. Few lymphatic malformations were unilocular cysts with thin walls and good sound transmission. The boundaries of the lesions were unclear in the microcystic and mixed types but clear in the macrocystic type. Therefore, classification may help in treatment selection.
The sensitivity of ultrasonographic diagnosis in this study was 99.38%. The diagnosis and classification of cervical lymphatic malformations are established through clinical manifestations and imaging data. However, cervical lymphatic malformations can invade adjacent structures, and ultrasonography is limited in resolving the boundaries of cysts; MRI and CT scan have better tissue resolution. In our study, 43 and 55 patients underwent MRI and CT scan, respectively. When a cervical lymphatic malformation is complicated by intracystic haemorrhage or infection, fine spot echoes can be observed in the cyst.22 With complications of cystic wall fibrosis or hardening, stripe hyperechogenicity can be observed in the cyst wall.23 In this study, 2.19% patients developed concurrent intracystic haemorrhage and infection, 20% developed intracystic haemorrhage, 3.12% developed an infection, and 0.94% developed calcification. No patients in our previous analysis of foetal lymphatic malformation experienced these complications. These complications may be related to friction and trauma due to discomfort caused by the neck mass in children.
Although 1.6–16% of lymphatic malformations subside naturally and 15–70% have mild symptoms and only need outpatient follow-up,7 approximately 50% of cervical lymphatic malformations, especially those complicated by haemorrhage and infection, cause compression and distortion of important adjacent organs, affecting breathing, swallowing, and making sounds, and can be life-threatening. Therefore, surgery, radiotherapy, or interventional therapy must be carried out. However, surgery may damage adjacent blood vessels and nerves, cause hematoma, and affect appearance. Moreover, the incidence of complications from surgery is 19–33%, the postoperative recurrence rate is 53%, and the mortality rate is 6%.7,27 Therefore, interventional sclerotherapy is a better choice for recurrent and surgically unresectable lesions, reducing tumour volume before surgery, reducing injury, and improving aesthetic appearance.7,28 Sclerotherapy has thus become the primary treatment for cervical lymphatic malformations; however, it has side effects such as metabolic acidosis, hyperhaemoglobinaemia, and cellulitis.29 Therefore, patients with large-volume or multilocular cysts should undergo sclerotherapy in stages or for residual lesions after surgical resection to avoid toxic side effects. In this study, patients received treatment according to their condition; most patients underwent sclerotherapy once, and few patients underwent sclerotherapy two or more times. Several patients underwent surgical resection or concurrent surgical resection and sclerotherapy. Expectant management was provided to the patients with progressive reduction of lesions. The total success rate of treatment was 99.38%. All of the lesions eventually disappeared, with the longest regression time being 2 years.
Strengths and limitations: The strength of this study is its sample size; to the best of our knowledge, this is the largest study to date on cervical lymphatic malformations in southern China.
Our study has some limitations. First, whether the occurrence of an isolated cervical lymphatic malformation is related to gender is unclear. Among 320 patients with cervical lymphatic malformations, the male-to-female ratio was 1.4:1. A multicentre study with a large sample size would clarify this association. Second, this study did not stage cervical lymphatic malformations, and treatment methods used for the three cervical lymphatic malformation types were not compared. These aspects should be explored in a subsequent study.
In conclusion, our results demonstrated that accurate ultrasonographic diagnosis and classification of cervical lymphatic malformations may provide a basis for selecting its most appropriate treatment method. For foetuses with cervical lymphatic malformations, strengthening prenatal ultrasonographic monitoring and understanding the extent of lesion progression, tracheal compression, and distortion are vital. Multidisciplinary teams should thus be involved in comprehensively selecting delivery modes and performing timely delivery. During the neonatal period or childhood, multiple imaging methods, including ultrasonography, MRI, and CT, should be used to monitor the relationship of the lesion and adjacent structures, thus allowing surgeons to select the most appropriate treatments.
MRI: Magnetic resonance imaging; CT: Computed tomography
Ethics approval and consent to participate
This study was approved by the ethics committee of the GZ Women and Children’s Medical Centre(4AE4237E-7889-4275-95E5-8F469A5C9188). Parents of the children in the study provided informed consent for ultrasonography and treatment options. Written consent was obtained from parents of participants in this study. (Found in additional file 1).
Consent for publication
Not applicable.
Availability of data and material
All data generated or analyzed during this study are included in this article and could be found in additional file 1. (Additional file 1: Description of the data and material of 320 cases with cervical lymphatic malformation).
Competing interest
The authors declare that they have no competing interests.
Funding
The study was funded by the Guangzhou Institute of Paediatrics/Guangzhou Women and Children’s Medical Centre (No: GCP-2019-005), a total of 140,000 yuan, 90,000 yuan was used in the design of the study and collection, analysis, and interpretation of data, and 50,000 yuan was used in writing the manuscript.
Authors’ Contributions
LJL, GXP, LXF, ZhXX, and WYR were involved in the ultrasonographic diagnosis of all cases of cervical lymphatic malformation and analysed and interpreted the data regarding cervical lymphatic malformation. LHB performed interventional therapy on the patients with cervical lymphatic malformation and analysed and interpreted the data. ZhW was responsible for the conservative treatment, operation, and operation combined with interventional therapy of the remaining patients with cervical lymphatic malformation, and also analysed and interpreted the data. All authors contributed to the preparation of the manuscript, and all authors have read and approved the manuscript to be submitted. Each author can respond to any questions in regard to this study in their area of expertise.
Acknowledgments
We would like to thank our image section director, Liao Can, for supporting all aspects of the study (ethics, consent, data handling and storage, and all other aspects of Good Research Practice). We also thank Editage (www.editage.com) for English language editing.
1 Arisoy R, Erdogdu E, Kumru P, Demirci O, Yuksel MA, Pekin O, et al. Prenatal diagnosis and outcome of lymphangiomas and its relationship with fetal chromosomal abnormalities. J Matern Fetal Neonatal Med 2016;29:466-472.
2 Jiao-Ling L, Hai-Ying W, Wei Z, Jin-Rong L, Kun-Shan C, Qian F. Treatment and prognosis of fetal lymphangioma. Eur J Obstet Gynecol Reprod Biol 2018;231:274-279.
3 Li JL, Wu HY, Liu JR, He QM, Chen KS, Yang J, et al. Fetal lymphangioma: prenatal diagnosis on ultrasound, treatment, and prognosis. Eur J Obstet Gynecol Reprod Biol 2018;231:268-273.
4 Fisher R, Partington A, Dykes E. Cystic hygroma: comparison between prenatal and postnatal diagnosis. J Pediatr Surg 1996;31:473-476.
5 Khanwalkar A, Carter J, Bhushan B, Rastatter J, Maddalozzo J. Thirty-day perioperative outcomes in resection of cervical lymphatic malformations. Int J Pediatr Otorhinolaryngol 2018;106:31-34.
6 Grasso DL, Pelizzo G, Zocconi E, Schleef J. Lymphangiomas of the head and neck in children. Acta Otorhinolaryngol Ital 2008;28:17-20.
7 Manzini M, Schweiger C, Manica D, Kuhl G. Response to OK-432 sclerotherapy in the treatment of cervical lymphangioma with submucosal extension to the airway. Braz J Otorhinolaryngol 2016. https://doi.org/10.1016/j.bjorl.2016.04.016.
8 Elshaar K, AbuAleid L. Adult-onset giant cervical cystic hygroma with pressure manifestations on aerodigestive tract, managed surgically: reporting of a rare case. Ann R Coll Surg Engl 2019;101(3):e84-87.
9 Gedikbasi A, Oztarhan K, Aslan G, Demirali O, Akyol A, Sargin A, et al. Multidisciplinary approach in cystic hygroma: prenatal diagnosis, outcome, and postnatal follow up. Pediatr Int 2009;51:670-677.
10 Efe N, Altas E, Mazlumoglu MR, Aktan B, Ucuncu H, Eren S, et al. Excellent result with the use of single-dose OK-432 in cervical macrocystic lymphangioma. J Craniofac Surg 2016;27:1802-1803.
11 Bernasconi A, Yoo SJ, Golding F, Langer JC, Jaeggi ET. Etiology and outcome of prenatally detected paracardial cystic lesions: a case series and review of the literature. Ultrasound Obstet Gynecol 2007;29:388-394.
12 Shimizu J, Taga T, Kishimoto T, Ohta M, Tagawa K, Kunitsu T, et al. Airway obstruction caused by rapid enlargement of cervical lymphangioma in a five-month-old boy. Clin Case Rep 2016;4:896-898.
13 Jo MS, Jeong JY. A rare cause of dyspnea: cervicothoracic cystic lymphangioma. J Craniofac Surg 2017;28:706-707.
14 Laje P, Tharakan SJ, Hedrick HL. Immediate operative management of the fetus with airway anomalies resulting from congenital malformations. Semin Fetal Neonatal Med 2016;21:240-245.
15 Ono K, Kikuchi A, Miyashita S, Iwasawa Y, Miyachi K, Sunagawa S, et al. Fetus with prenatally diagnosed posterior mediastinal lymphangioma: characteristic ultrasound and magnetic resonance imaging findings. Congenit Anom (Kyoto) 2007;47:158-160.
16 Cozzi DA, Olivieri C, Manganaro F, Savelli S, Giancotti A, Manganaro L. Fetal abdominal lymphangioma enhanced by ultrafast MRI. Fetal Diagn Ther 2010;27:46-50.
17 Porwal PK, Dubey KP, Morey A, Singh H, Pooja S, Bose A. Bleomycin sclerotherapy in lymphangiomas of head and neck: prospective study of 8 cases. Indian J Otolaryngol Head Neck Surg 2017;70:145-148.
18 Inde Y, Yamagishi E, Kawabata I, Sekiguchi A, Nakai A, Takeshita T. Morphological changes observed via fetal ultrasound in prenatally diagnosed and isolated congenital lymphangiomas: three case reports. J Med Ultrason 2013;40:265-269.
19Chang TS, Ricketts R, Abramowsky CR, Cotter BD, Steelman CK, Husain A, et al. Mesenteric cystic masses: a series of 21 pediatric cases and review of the literature. Fetal Pediatr Pathol 2011;30:40-44.
20 Lu D, Wang Y, Zeng W, Peng B. Giant fetal lymphangioma at chest wall and prognosis: case report and literature review. Taiwan J Obstet Gynecol 2015;54:62-65.
21 Noia G, Pellegrino M, Masini L, Visconti D, Manzoni C, Chiaradia G, et al. Fetal cystic hygroma: the importance of natural history. Eur J Obstet Gynecol Reprod Biol 2013;170:407-413.
22 Furue A, Mochizuki J, Onishi Y, Kawano S, Kanai Y, Kemmochi M, et al. Ultrasonic findings of fetal axillary lymphangioma with intralesional hemorrhage. J Med Ultrason (2001) 2016;43:285-289.
23 Buccoliero AM, Castiglione F, Maio V, Morelli C, Martin A, Messineo A, et al. Calcified cystic lymphangioma of the mesentery: case report. Fetal Pediatr Pathol 2009;28:209-215.
24 Scofield-Kaplan SM, Sobti D, Evers BM, Hogan RN, Mancini R. Unusual location for a periorbital dermoid cyst. Ophthal Plast Reconstr Surg 2018;34:e208-e209.
25 Quintanilla-Dieck L, Penn EB Jr. Congenital neck masses. Clin Perinatol 2018;45:769-785.
26 Premkumar K, Basle MA, Jassim K, Waseem Ahamed TP. An unusual case of cervical lipoblastoma with review of literature. J Cancer Res Ther 2015;11:1025.
27 Warnock WT, Khoshnam N, Bird KM, Schaffer LR, Lou LH, Hawkins CM, et al. Congenital cyst of the pancreas: a case report and review of literature. Fetal Pediatr Pathol 2016;35:265-271.
28 Chiappinelli A, Forgues D, Galifer RB. Congenital abdominal cystic lymphangiomas: what is the correct management? J Matern Fetal Neonatal Med 2012;25:915-919.
29 Coughlin K, Flibotte J, Cahil AM, Osterhoudt K, Hedrick H, Vrecenak J. Methemoglobinemia in an infant after sclerotherapy with high-dose doxycycline. Pediatrics 2019;143:pii:e20181642.
Table 1
Summary of ultrasonographic typing and complications of 320 patients of cervical lymphatic malformation
|
Typing |
Patient number |
Simple cervical lymphatic malformation |
Haemorrhage |
Infection |
Haemorrhage and infection |
Calcification |
|
Mixed type |
184 |
143 |
32 |
3 |
3 |
3 |
|
Macrocystic type |
117 |
78 |
31 |
5 |
3 |
0 |
|
Microcystic type |
19 |
15 |
1 |
2 |
1 |
0 |
|
Total |
320 |
236 (73.75) |
64 (20) |
10 (3.12) |
7 (2.19) |
3 (0.94) |
Table 2
Summary of ultrasonographic typing and treatments of 320 patients of cervical lymphatic malformation
|
Typing |
Patient number |
Interventional therapy (once) |
Interventional therapy (twice or more) |
Operation |
Operation combined with interventional therapy |
Expectant treatment |
|
Mixed type |
184 |
85 |
62 |
16 |
4 |
17 |
|
Macrocystic type |
117 |
69 |
32 |
11 |
0 |
5 |
|
Microcystic type |
19 |
9 |
3 |
1 |
1 |
5 |
|
Total |
320 |
163 (50.94) |
97 (30.31) |
28 (8.75) |
5 (1.56) |
27 (8.44) |
This is a list of supplementary files associated with this preprint. Click to download.
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Under Review
Version 1
Posted 22 May, 2020
No community comments so far
Editor assigned
On 18 May, 2020
Submission checks complete
On 18 May, 2020
Editor invited
On 18 May, 2020
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Pediatrics
Learn more about our company.
This preprint is under consideration at BMC Pediatrics. Preprints are preliminary reports that have not undergone peer review. They should not be considered conclusive, used to inform clinical practice, or referenced by the media as validated information.
Learn more about In Review
Research article
Ultrasonographic diagnosis, classification, and treatment of cervical lymphatic malformation in paediatric patients: a retrospective study
Jiaoling Li
Guangzhou University
Corresponding Author
ORCiD: https://orcid.org/0000-0002-8343-1310
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Under Review
Version 1
Posted 22 May, 2020
No community comments so far
Editor assigned
On 18 May, 2020
Submission checks complete
On 18 May, 2020
Editor invited
On 18 May, 2020
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Pediatrics
License:
This work is licensed under a Creative Commons Attribution 4.0 International License. Read Full License
Background: To explore the imaging features, key diagnostic points, classification, treatment, and prognosis of cervical lymphatic malformation.
Methods: Overall, 320 patients diagnosed with cervical lymphatic malformation were retrospectively analysed in our hospital between 1 January 2014 and 31 December 2017. Imaging modalities included colour Doppler ultrasound, magnetic resonance imaging, and contrast-enhanced computed tomography. Cervical lymphatic malformations were classified by cyst diameter. Treatments included interventional therapy, surgery, and expectant treatment.
Results: Cervical lymphatic malformation was identified in 320 of 1,192 patients with lymphatic malformation, the sensitivity of ultrasonographic diagnosis was 99.38% (318/320). Cervical lymphatic malformation was classified as mixed, macrocystic, and microcystic in 184 (57.5%), 117 (36.56%), and 19 (5.94%) patients, respectively. Sixty-four (20%), ten (3.12%), seven (2.19%), and three (0.94%) patients experienced intracystic haemorrhage, infection, concurrent intracystic haemorrhage and infection, and calcification, respectively. Among 260 (81.25%) patients who underwent interventional sclerotherapy, 163 (50.94%) received it once and 96 (30%) received it two or more times. Twenty-eight (8.75%), five (1.56%), and 27 (8.44%) patients underwent surgical resection, interventional sclerotherapy plus surgery, and expectant management, respectively.
Conclusions: Ultrasonography is useful for diagnosing definitely cervical lymphatic malformation. Interventional therapy is the first choice for children with confirmed cervical lymphatic malformation.
Figure 1
Figure 2
Cervical lymphatic malformation is the most common lymphatic malformation and the most common cervical vascular malformation in children,1-3 with an incidence of 1.2–2.8 per 1,000 individuals. It develops prenatally and can be diagnosed by prenatal ultrasonography in 50–65% of patients but by postnatal ultrasonography in 90% of patients. It is not typically diagnosed in adults. Surgical and other trauma can destroy the lymphatic drainage system, leading to acquired lymphatic malformation; other causes are also possible.4,5 Its clinical manifestations depend on the infiltration location and growth rate, and approximately 15–70% of patients have mild symptoms.6-8 Diagnosis is based on intracystic fluid echoic, a thick of cystic wall, and the cyst location; additionally, medical history and clinical manifestations are helpful for diagnosis.
Cervical lymphatic malformation can present as unilocular or multilocular cysts, and the cyst diameter varies from few millimetres to several centimetres.9 Therefore, it can be classified as macrocystic, microcystic, or a mixed type according to the cyst diameter. Although cervical lymphatic malformations are benign, they can invade adjacent critical structures, which increases the difficulty of surgical resection and the risk of postoperative recurrence. The malformations may also threaten the function of adjacent or affected tissues, organs, and as a result, appropriate treatment may be avoided.10,11 Cervical lymphatic malformations can predispose an individual to complications such as haemorrhage or infection, which further affect respiratory function and endanger life.12-14
Multiple imaging methods should be used to classify and make differential diagnoses of cervical lymphatic malformations, monitor lesion progression, and assess the relationship of the lesion with adjacent structures to guide the optimal treatment method selection,15,16 reduce complications and recurrence, and avoid injury.
The aim of this retrospective single-centre study was to explore the ultrasonographic characteristics of patients with cervical lymphatic malformations in southern China and summarise key points of their diagnosis, differential diagnosis, classification, and treatment selection as well as analyse the causes of misdiagnoses and missed diagnoses.
This study was approved by our Institutional Ethics Review Board. Parents of the children provided informed consent for ultrasonography and treatment options.
Patient selection: Records in the large data centre at our hospital were searched and data on patients diagnosed with lymphatic malformation or cervical lymphatic malformation between 1 January 2014 and 31 December 2017 were collected. We used “lymphatic malformation” and “cervical lymphatic malformation” as keywords in the search. Three hundred twenty-two patients were initially identified; four were excluded due to misdiagnosis by ultrasonography, and two previously excluded patients were included: one was due to a missed diagnosis and the other developed cervical lymphatic malformation after pyriform cyst surgery. Therefore, 320 patients with confirmed cervical lymphatic malformation were included in the analysis. The mean age of the patients was 2 years and 29 days (range, 1 day to 14 years). Thirty-five patients were prenatally identified as having cervical lymphatic malformation. Twenty-seven, 260, 5, and 28 patients underwent a follow-up ultrasonographic assessment once every two months at >6 months after diagnosis, intraoperative puncture result assessment, intraoperative puncture and postoperative pathological confirmation, and postoperative pathological confirmation of the diagnosis (regarded as the gold standard to determine the coincidence rate between image-based and puncture results or pathological diagnoses), respectively.
Imaging, diagnosis, and classification: A high-resolution ultrasound system, equipped with a 4–15 MHz transducer (Acuson S2000; Siemens, Hamberg, Germany), was used to diagnose cervical lymphatic malformation. Patients in whom the relationship between the lesion and adjacent structures was not clear by ultrasonography because of individual cysts <1 mm in diameter underwent further evaluation by magnetic resonance imaging (MRI) (Signa; GE Medical Systems, Milwaukee, WI, USA) or contrast-enhanced computed tomography (CT). All patients underwent colour Doppler and power Doppler ultrasonography. Diagnosis and differential diagnosis were based on the intracystic fluid echo, thickness of cyst wall, cyst location, and colour flow signal distribution. The macrocystic (cysts >1 cm in diameter), microcystic (individual cysts <1 cm in diameter), and mixed types were classified according to the cyst diameter. Cervical lymphatic malformation was diagnosed with haemorrhage, infection, and/or calcification according to increased cyst diameter (compared with prior size) and intracyst fine spot echo, local pain, increased temperature and abnormal blood test results, and calcified plaque in cyst before treatment, respectively.
Treatments: Patients were treated with interventional sclerotherapy, surgery, or surgery combined with interventional therapy.
The principles and methods of interventional sclerotherapy17 (using bleomycin at the recommended dose of 0.5 mg/kg in an aqueous solution of 1.5 mg/mL) were as follows: (1) to prevent the side effects of sclerosing agents in infants, patients were >6 months old unless symptoms of oppression occurred; (2) individual cysts had to be >1 cm in diameter; (3) imaging findings should suggest infiltration of the lesion into adjacent critical structures; (4) Patients with cervical lymphatic malformation patients complicated by infection first had to undergo anti-inflammatory treatment for 3–5 days before a sclerosing agent will be injected into the lesion; (5) For abscess formation complicating cervical lymphatic malformation, ultrasound-guided puncture and drainage was administered to patients followed by a simultaneous injection of a sclerosing agent; (6) cervical lymphatic malformation patients with haemorrhage first had to undergo haemostasis, after which a sclerosing agent was injected into the lesion; and (7) for patients without complications, a sclerosing agent had to be injected into the lesion or they had to undergo follow-up ultrasonography.
If the lesion did not progress, expectant treatment (namely routine observation) continued. For patients in whom the lesion increased or hardened (compared with prior size or texture), interventional sclerotherapy or surgery was performed, with routine blood tests, screening of the coagulation status, and liver function tests. For all patients who underwent interventional sclerotherapy, the puncture liquid was smeared for definite diagnosis and a contrast medium was injected to determine the size of the cyst. If the cysts were interconnected, we chose the dose of the contrast medium that excluded lymphovascular malformations.
Patients with individual cysts <1 cm in diameter underwent surgical treatment, and the determinant for surgery combined with interventional therapy was lesion size more than 4 cm in diameter. The interventional therapy targets residual lesions to prevent toxic side effects caused by excessive use of sclerosing agents.
Cervical lymphatic malformation was identified in 320 of the 1,192 patients with lymphatic malformation examined during our observation period with 189 males and 131 females (1.4:1 male-to-female ratio). the sensitivity of ultrasonographic diagnosis was 99.38% (318/320). All 320 patients had undergone further evaluation by colour Doppler ultrasonography and power Doppler ultrasonography, with 43 patients further assessed by MRI and 55 by contrast-enhanced CT. Details regarding the types of complications and medical management are summarised in Tables 1 and 2. Complications occurred in 84 patients (26.25%); of them, 64 (20%) experienced haemorrhage, 10 (3.12%) developed infections, 7 (2.19%) had concurrent haemorrhage and infection, and 3 (0.94%) exhibited calcification. Of the 260 patients (81.25%) who underwent sclerotherapy, 163 (50.94%) underwent bleomycin sclerotherapy once and 96 (30%) underwent bleomycin sclerotherapy two or more times, with one patient (0.31%) receiving seven rounds. Twenty-eight patients (8.75%) received surgical treatment and five (1.56%) received concurrent surgical treatment and bleomycin sclerotherapy. Expectant management was provided to the other 27 patients (8.44%).
The ultrasonographic features and treatments of one representative patient with cervical lymphatic malformation and those of the patient misdiagnosed with lipoblastoma are shown in Figures 1 and 2. Of the 320 patients, only 19 (5.94%) showed sparse flow signals on the cyst wall and septations.
Among the 28 patients who underwent surgical treatment, only one experienced branchial cleft fistula. Of the 260 patients who underwent bleomycin sclerotherapy, only one developed an infection. All lesions gradually resolved, with the longest taking 2 years. Two patients with pyriform cysts, one patient with a dermoid cyst, and one patient with lipoblastoma were misdiagnosed with cervical lymphatic malformation, whereas one patient with cervical lymphatic malformation was misdiagnosed as having a pyriform cyst.
Main findings
In this retrospective study of 320 patients, including one patient who developed cervical lymphatic malformation after pyriform cyst surgery and one patient who was misdiagnosed by ultrasonography, the sensitivity of which was 99.38%, the incidence of cervical lymphatic malformation complications was 26.25% before treatment. One patient developed a branchial cleft fistula and another developed an infection; the total success rate of treatment was 99.38%.
Interpretation: Cervical lymphatic malformation, a benign congenital disease, is caused by non-transportation of the lymphatic and venous systems, abnormal hyperplasia of the lymphatic epithelium, or lymphatic obstruction. Additionally, surgical trauma to the neck can destroy the lymphatic drainage system, resulting in acquired cervical lymphatic malformation.18 In this study, it was found that cervical lymphatic malformation also developed as a complication of pyriform cyst surgery.
Lymphatic malformations can occur in all areas of the body, but 75% occur in the neck.19,20 In this study, cervical lymphatic malformation was found to constitute 26.85% of all lymphatic malformation cases (320/1,192). This rate is lower than that observed previously, where foetal cervical lymphatic malformation comprised 63.29% of all lymphatic malformation cases. This may be because more pregnancies were terminated due to cervical lymphatic malformations accompanied by severe structural or chromosomal abnormalities in the foetus, or because some lesions subsided naturally.21 Two-dimensional ultrasonography showed that most cervical lymphatic malformations were multilocular cysts; large cysts had a fine separation, and small cysts had a coarse separation. Few lymphatic malformations were unilocular cysts with thin walls and good sound transmission. The boundaries of the lesions were unclear in the microcystic and mixed types but clear in the macrocystic type. Therefore, classification may help in treatment selection.
The sensitivity of ultrasonographic diagnosis in this study was 99.38%. The diagnosis and classification of cervical lymphatic malformations are established through clinical manifestations and imaging data. However, cervical lymphatic malformations can invade adjacent structures, and ultrasonography is limited in resolving the boundaries of cysts; MRI and CT scan have better tissue resolution. In our study, 43 and 55 patients underwent MRI and CT scan, respectively. When a cervical lymphatic malformation is complicated by intracystic haemorrhage or infection, fine spot echoes can be observed in the cyst.22 With complications of cystic wall fibrosis or hardening, stripe hyperechogenicity can be observed in the cyst wall.23 In this study, 2.19% patients developed concurrent intracystic haemorrhage and infection, 20% developed intracystic haemorrhage, 3.12% developed an infection, and 0.94% developed calcification. No patients in our previous analysis of foetal lymphatic malformation experienced these complications. These complications may be related to friction and trauma due to discomfort caused by the neck mass in children.
Although 1.6–16% of lymphatic malformations subside naturally and 15–70% have mild symptoms and only need outpatient follow-up,7 approximately 50% of cervical lymphatic malformations, especially those complicated by haemorrhage and infection, cause compression and distortion of important adjacent organs, affecting breathing, swallowing, and making sounds, and can be life-threatening. Therefore, surgery, radiotherapy, or interventional therapy must be carried out. However, surgery may damage adjacent blood vessels and nerves, cause hematoma, and affect appearance. Moreover, the incidence of complications from surgery is 19–33%, the postoperative recurrence rate is 53%, and the mortality rate is 6%.7,27 Therefore, interventional sclerotherapy is a better choice for recurrent and surgically unresectable lesions, reducing tumour volume before surgery, reducing injury, and improving aesthetic appearance.7,28 Sclerotherapy has thus become the primary treatment for cervical lymphatic malformations; however, it has side effects such as metabolic acidosis, hyperhaemoglobinaemia, and cellulitis.29 Therefore, patients with large-volume or multilocular cysts should undergo sclerotherapy in stages or for residual lesions after surgical resection to avoid toxic side effects. In this study, patients received treatment according to their condition; most patients underwent sclerotherapy once, and few patients underwent sclerotherapy two or more times. Several patients underwent surgical resection or concurrent surgical resection and sclerotherapy. Expectant management was provided to the patients with progressive reduction of lesions. The total success rate of treatment was 99.38%. All of the lesions eventually disappeared, with the longest regression time being 2 years.
Strengths and limitations: The strength of this study is its sample size; to the best of our knowledge, this is the largest study to date on cervical lymphatic malformations in southern China.
Our study has some limitations. First, whether the occurrence of an isolated cervical lymphatic malformation is related to gender is unclear. Among 320 patients with cervical lymphatic malformations, the male-to-female ratio was 1.4:1. A multicentre study with a large sample size would clarify this association. Second, this study did not stage cervical lymphatic malformations, and treatment methods used for the three cervical lymphatic malformation types were not compared. These aspects should be explored in a subsequent study.
In conclusion, our results demonstrated that accurate ultrasonographic diagnosis and classification of cervical lymphatic malformations may provide a basis for selecting its most appropriate treatment method. For foetuses with cervical lymphatic malformations, strengthening prenatal ultrasonographic monitoring and understanding the extent of lesion progression, tracheal compression, and distortion are vital. Multidisciplinary teams should thus be involved in comprehensively selecting delivery modes and performing timely delivery. During the neonatal period or childhood, multiple imaging methods, including ultrasonography, MRI, and CT, should be used to monitor the relationship of the lesion and adjacent structures, thus allowing surgeons to select the most appropriate treatments.
MRI: Magnetic resonance imaging; CT: Computed tomography
Ethics approval and consent to participate
This study was approved by the ethics committee of the GZ Women and Children’s Medical Centre(4AE4237E-7889-4275-95E5-8F469A5C9188). Parents of the children in the study provided informed consent for ultrasonography and treatment options. Written consent was obtained from parents of participants in this study. (Found in additional file 1).
Consent for publication
Not applicable.
Availability of data and material
All data generated or analyzed during this study are included in this article and could be found in additional file 1. (Additional file 1: Description of the data and material of 320 cases with cervical lymphatic malformation).
Competing interest
The authors declare that they have no competing interests.
Funding
The study was funded by the Guangzhou Institute of Paediatrics/Guangzhou Women and Children’s Medical Centre (No: GCP-2019-005), a total of 140,000 yuan, 90,000 yuan was used in the design of the study and collection, analysis, and interpretation of data, and 50,000 yuan was used in writing the manuscript.
Authors’ Contributions
LJL, GXP, LXF, ZhXX, and WYR were involved in the ultrasonographic diagnosis of all cases of cervical lymphatic malformation and analysed and interpreted the data regarding cervical lymphatic malformation. LHB performed interventional therapy on the patients with cervical lymphatic malformation and analysed and interpreted the data. ZhW was responsible for the conservative treatment, operation, and operation combined with interventional therapy of the remaining patients with cervical lymphatic malformation, and also analysed and interpreted the data. All authors contributed to the preparation of the manuscript, and all authors have read and approved the manuscript to be submitted. Each author can respond to any questions in regard to this study in their area of expertise.
Acknowledgments
We would like to thank our image section director, Liao Can, for supporting all aspects of the study (ethics, consent, data handling and storage, and all other aspects of Good Research Practice). We also thank Editage (www.editage.com) for English language editing.
1 Arisoy R, Erdogdu E, Kumru P, Demirci O, Yuksel MA, Pekin O, et al. Prenatal diagnosis and outcome of lymphangiomas and its relationship with fetal chromosomal abnormalities. J Matern Fetal Neonatal Med 2016;29:466-472.
2 Jiao-Ling L, Hai-Ying W, Wei Z, Jin-Rong L, Kun-Shan C, Qian F. Treatment and prognosis of fetal lymphangioma. Eur J Obstet Gynecol Reprod Biol 2018;231:274-279.
3 Li JL, Wu HY, Liu JR, He QM, Chen KS, Yang J, et al. Fetal lymphangioma: prenatal diagnosis on ultrasound, treatment, and prognosis. Eur J Obstet Gynecol Reprod Biol 2018;231:268-273.
4 Fisher R, Partington A, Dykes E. Cystic hygroma: comparison between prenatal and postnatal diagnosis. J Pediatr Surg 1996;31:473-476.
5 Khanwalkar A, Carter J, Bhushan B, Rastatter J, Maddalozzo J. Thirty-day perioperative outcomes in resection of cervical lymphatic malformations. Int J Pediatr Otorhinolaryngol 2018;106:31-34.
6 Grasso DL, Pelizzo G, Zocconi E, Schleef J. Lymphangiomas of the head and neck in children. Acta Otorhinolaryngol Ital 2008;28:17-20.
7 Manzini M, Schweiger C, Manica D, Kuhl G. Response to OK-432 sclerotherapy in the treatment of cervical lymphangioma with submucosal extension to the airway. Braz J Otorhinolaryngol 2016. https://doi.org/10.1016/j.bjorl.2016.04.016.
8 Elshaar K, AbuAleid L. Adult-onset giant cervical cystic hygroma with pressure manifestations on aerodigestive tract, managed surgically: reporting of a rare case. Ann R Coll Surg Engl 2019;101(3):e84-87.
9 Gedikbasi A, Oztarhan K, Aslan G, Demirali O, Akyol A, Sargin A, et al. Multidisciplinary approach in cystic hygroma: prenatal diagnosis, outcome, and postnatal follow up. Pediatr Int 2009;51:670-677.
10 Efe N, Altas E, Mazlumoglu MR, Aktan B, Ucuncu H, Eren S, et al. Excellent result with the use of single-dose OK-432 in cervical macrocystic lymphangioma. J Craniofac Surg 2016;27:1802-1803.
11 Bernasconi A, Yoo SJ, Golding F, Langer JC, Jaeggi ET. Etiology and outcome of prenatally detected paracardial cystic lesions: a case series and review of the literature. Ultrasound Obstet Gynecol 2007;29:388-394.
12 Shimizu J, Taga T, Kishimoto T, Ohta M, Tagawa K, Kunitsu T, et al. Airway obstruction caused by rapid enlargement of cervical lymphangioma in a five-month-old boy. Clin Case Rep 2016;4:896-898.
13 Jo MS, Jeong JY. A rare cause of dyspnea: cervicothoracic cystic lymphangioma. J Craniofac Surg 2017;28:706-707.
14 Laje P, Tharakan SJ, Hedrick HL. Immediate operative management of the fetus with airway anomalies resulting from congenital malformations. Semin Fetal Neonatal Med 2016;21:240-245.
15 Ono K, Kikuchi A, Miyashita S, Iwasawa Y, Miyachi K, Sunagawa S, et al. Fetus with prenatally diagnosed posterior mediastinal lymphangioma: characteristic ultrasound and magnetic resonance imaging findings. Congenit Anom (Kyoto) 2007;47:158-160.
16 Cozzi DA, Olivieri C, Manganaro F, Savelli S, Giancotti A, Manganaro L. Fetal abdominal lymphangioma enhanced by ultrafast MRI. Fetal Diagn Ther 2010;27:46-50.
17 Porwal PK, Dubey KP, Morey A, Singh H, Pooja S, Bose A. Bleomycin sclerotherapy in lymphangiomas of head and neck: prospective study of 8 cases. Indian J Otolaryngol Head Neck Surg 2017;70:145-148.
18 Inde Y, Yamagishi E, Kawabata I, Sekiguchi A, Nakai A, Takeshita T. Morphological changes observed via fetal ultrasound in prenatally diagnosed and isolated congenital lymphangiomas: three case reports. J Med Ultrason 2013;40:265-269.
19Chang TS, Ricketts R, Abramowsky CR, Cotter BD, Steelman CK, Husain A, et al. Mesenteric cystic masses: a series of 21 pediatric cases and review of the literature. Fetal Pediatr Pathol 2011;30:40-44.
20 Lu D, Wang Y, Zeng W, Peng B. Giant fetal lymphangioma at chest wall and prognosis: case report and literature review. Taiwan J Obstet Gynecol 2015;54:62-65.
21 Noia G, Pellegrino M, Masini L, Visconti D, Manzoni C, Chiaradia G, et al. Fetal cystic hygroma: the importance of natural history. Eur J Obstet Gynecol Reprod Biol 2013;170:407-413.
22 Furue A, Mochizuki J, Onishi Y, Kawano S, Kanai Y, Kemmochi M, et al. Ultrasonic findings of fetal axillary lymphangioma with intralesional hemorrhage. J Med Ultrason (2001) 2016;43:285-289.
23 Buccoliero AM, Castiglione F, Maio V, Morelli C, Martin A, Messineo A, et al. Calcified cystic lymphangioma of the mesentery: case report. Fetal Pediatr Pathol 2009;28:209-215.
24 Scofield-Kaplan SM, Sobti D, Evers BM, Hogan RN, Mancini R. Unusual location for a periorbital dermoid cyst. Ophthal Plast Reconstr Surg 2018;34:e208-e209.
25 Quintanilla-Dieck L, Penn EB Jr. Congenital neck masses. Clin Perinatol 2018;45:769-785.
26 Premkumar K, Basle MA, Jassim K, Waseem Ahamed TP. An unusual case of cervical lipoblastoma with review of literature. J Cancer Res Ther 2015;11:1025.
27 Warnock WT, Khoshnam N, Bird KM, Schaffer LR, Lou LH, Hawkins CM, et al. Congenital cyst of the pancreas: a case report and review of literature. Fetal Pediatr Pathol 2016;35:265-271.
28 Chiappinelli A, Forgues D, Galifer RB. Congenital abdominal cystic lymphangiomas: what is the correct management? J Matern Fetal Neonatal Med 2012;25:915-919.
29 Coughlin K, Flibotte J, Cahil AM, Osterhoudt K, Hedrick H, Vrecenak J. Methemoglobinemia in an infant after sclerotherapy with high-dose doxycycline. Pediatrics 2019;143:pii:e20181642.
Table 1
Summary of ultrasonographic typing and complications of 320 patients of cervical lymphatic malformation
|
Typing |
Patient number |
Simple cervical lymphatic malformation |
Haemorrhage |
Infection |
Haemorrhage and infection |
Calcification |
|
Mixed type |
184 |
143 |
32 |
3 |
3 |
3 |
|
Macrocystic type |
117 |
78 |
31 |
5 |
3 |
0 |
|
Microcystic type |
19 |
15 |
1 |
2 |
1 |
0 |
|
Total |
320 |
236 (73.75) |
64 (20) |
10 (3.12) |
7 (2.19) |
3 (0.94) |
Table 2
Summary of ultrasonographic typing and treatments of 320 patients of cervical lymphatic malformation
|
Typing |
Patient number |
Interventional therapy (once) |
Interventional therapy (twice or more) |
Operation |
Operation combined with interventional therapy |
Expectant treatment |
|
Mixed type |
184 |
85 |
62 |
16 |
4 |
17 |
|
Macrocystic type |
117 |
69 |
32 |
11 |
0 |
5 |
|
Microcystic type |
19 |
9 |
3 |
1 |
1 |
5 |
|
Total |
320 |
163 (50.94) |
97 (30.31) |
28 (8.75) |
5 (1.56) |
27 (8.44) |