Respiratory Medicine CME
Volume 1, Issue 4 , Pages 270-273, 2008

Isolated mediastinal cystic lymphangioma in children: About two cases

  • L. Ghedira

      Affiliations

    • Pediatric Department, Fattouma Bourguiba Hospital, Monastir 5000, Tunisia
    • ,
  • S. Haddad

      Affiliations

    • Pediatric Department, Fattouma Bourguiba Hospital, Monastir 5000, Tunisia
    • Corresponding Author InformationCorresponding author. Tel.: +216 97774714.
    • ,
  • K. Lajmi

      Affiliations

    • Pediatric Department, Fattouma Bourguiba Hospital, Monastir 5000, Tunisia
    • ,
  • C. Ben Meriem

      Affiliations

    • Pediatric Department, Fattouma Bourguiba Hospital, Monastir 5000, Tunisia
    • ,
  • S. Hammami

      Affiliations

    • Pediatric Department, Fattouma Bourguiba Hospital, Monastir 5000, Tunisia
    • ,
  • A. Zakhama

      Affiliations

    • Cytopathology Department, Fattouma Bourguiba Hospital, Monastir 5000, Tunisia
    • ,
  • M. Mekki

      Affiliations

    • Pediatric Surgery Department, Fattouma Bourguiba Hospital, Monastir 5000, Tunisia
    • ,
  • MN. Guediche

      Affiliations

    • Pediatric Department, Fattouma Bourguiba Hospital, Monastir 5000, Tunisia

Received 28 June 2008; accepted 1 July 2008.

Article Outline

Summary 

Lymphangiomas are a heterogeneous group of benign vascular malformations of the lymphatic system composed of cystically dilated lymphatics. Most cystic lymphangiomas are found in the cervical region. Isolated mediastinal cystic lymphangiomas are very uncommon. Herein we present two pediatric cases of isolated mediastinal cystic lymphangioma. In the first case, a seven-year-old girl presented with chest pain and dyspnea revealing a giant anterior mediastinal cystic lymphangioma extending inferiorly into the upper part of the abdomen. In the second case, a four-week-old boy presented with an acute respiratory distress revealing a voluminous posterior mediastinal cyst lymphangioma. In the view of literature, these cystic lymphangiomas are accepted to be atypical because of their locations and large size.

Keywords: Respiratory distress, Cystic lymphangioma, Mediastinal tumor

 

Back to Article Outline

Introduction 

Cystic lymphangioma, also known as cystic hygroma is a congenital malformation of the lymphatics resulting in blockage of lymphatic flow.1 As they develop in the body surface, cervical region (75%) and axillary region (20%), the majority of them are detected before the age of 2 years. Of cervical lymphangiomas only 2–3% may be associated with an intrathoracic extension.2 An isolated mediastinal lymphangioma without a cervical component is an uncommon occurrence which account for less than 1%.3

Herein we present two pediatric cases of isolated mediastinal cystic lymphangioma. In the first case, a seven-year-old girl presented with chest pain and dyspnea revealing a giant anterior mediastinal cystic lymphangioma extending inferiorly into the upper part of the abdomen. In the second case, a four-week-old boy presented with an acute respiratory distress revealing a voluminous posterior mediastinal cyst lymphangioma.

Back to Article Outline

Case reports 

Case 1 

A 7-year-old girl, with no significant previous medical history, presented to the pediatric department with dyspnea and left chest pain. Physically, she was febrile, mildly tachypneic. Pulmonary examination revealed a matity at the left side on percussion and a diminished breath sounds at auscultation. A chest radiogram showed a large homogeneous opacity in the left lower lung field (Fig. 1).

Investigations revealed the following: haemoglobin: 11, 3g/dl, white cells: 13 500, C Reactive protein: 105mg/l. Serology of hydatid cyst was negative. Ultra sound showed a multicystic lesion in the lower third of the left hemithorax associated with a pleural effusion.

Computed tomography (CT) of the chest showed a voluminous 10×15×24cm multilocular cystic lesion in the anterior mediastinum. This lesion extended inferiorly into the upper part of the abdomen through a diaphragmatic defect until the interhepatodiaphragmatic space (Fig. 2).

The radiologic appearance suggested a diagnosis of lymphangioma of the mediastinum. Laparoscopy was performed. A very large cystic lesion was found. It compresses the segment II of the liver. There was discharge of hemorrhagic fluid at fine-needle aspiration of the cyst. The dissection of the mediastinal mass over the diaphragmatic defect was performed without thoracoscopic procedure. Than the diaphragmatic defect was sutured. Complete excision of the cystic lymphangioma was performed.

Histopathology confirmed it to be a lymphangioma. The post-operative course was uneventful, and she was discharged on the seventh post-operative day. At discharge, chest radiographic findings were normal. The patient is asymptomatic three years after surgical excision with no recurrence.

Case 2 

A 4-week-old boy weighting 3.85kg with no significant previous medical history, was admitted to the Pediatric Department for dyspnea, fever, cough, in context of epidemia of viral bronchiolitis.

Physical finding were: tachypnea (a respiratory rate of 70breaths/mn), tachycardia, intercostal and suprasternal retraction. Ronchi and crackles rales were revealed at auscultation.

A chest radiogram showed a bilateral pulmonary hyperinflation associated at a homogeneous opacity in the upper part of the right lung considered as an atelectasis.

Respiratory symptoms persist, and 48h later, the neonate developed an acute respiratory distress. Blood gas results were the following: pH: 7.15; PCO2: 56mmHg and PO2: 96mmHg. The baby was immediately managed by tracheal intubation and controlled mechanical ventilation. After 17 days of ventilation, he was extubated, but the acute respiratory distress had recidived and the neonate was reventilated.

Chest radiogram showed a homogeneous opacity of the upper two third of the right lung. Computed tomographic scan of the chest revealed a large 10×9cm multilocular cystic lesion in the posterior mediastinum. Heart, oesophagus and diaphragm were displaced but not compressed by the mass (Fig. 3). The diagnosis of cystic lymphangioma was suspected.

Under general anesthesia, a left postérolateral thoracotomy was performed. Intraoperatively, there was a large multicystic mass of the posterior mediastinum, this mass displace and compresses the left lung, encompasses the aorta and the oesophagus and exterted to the diaphragm, the mass extend to the right side of mediastinum. To facilitate dissection, the mass was decompressed with a trocar with removal of a clear fluid. Complete excision of a macrocystic lymphangioma were performed. A thoracostomy tube drain was left in situ.

A post-operative chest radiograph finding were normal. Histopathology confirmed the diagnosis of cystic lymphangioma. The post-operative course was uneventful. Seven-years follow-up showed no evidence of recurrence.

Back to Article Outline

Discussion 

Lymphangioma are a heterogeneous group of benign vascular malformations of the lymphatic system, composed of cystically dilated lymphatics.4 Most cystic lymphangioma are found in the cervical region and only 1–2% extend into the mediastinum.1

Isolated mediastinal cystic lymphangiomas are very uncommon, they represent less than 1% of all cystic lymphangioma.2 Cystic lymphangioma make up 5–6% of mediastinal masses in children.5

Most of mediastinal cystic lymphangiomas are not diagnosed during childhood until growing to be very large, and three quarters of these lesions are diagnosed in adults.2, 6

The majority of mediastinal cystic hygromas are located in the superior and anterior compartments. Posterior location is exceptional.2, 5, 7 In our first case, a giant cystic lymphangioma was located in the anterior mediastinum which extended into the abdomen. And in the second case, a giant cystic lymphangioma of the posterior mediastinum is reported.

These mediastinal cystic lesions are mostly asymptomatic, and the diagnosis usually is entertained only after mediastinal widening is noted in chest radiogram.3

However, in children under two years of age with isolated mediastinal cystic lymphangioma, the most common presenting symptoms are respiratory: dyspnea, cough, chest pain, acute respiratory distress, and respiratory failure. Rarely symptoms like recurrent respiratory symptoms, dysphagia, stridor, vocal cord paralysis, venous compression, and hemoptysis are found.6, 7, 8, 9, 10, 11, 12, 13

In the first case, chest pain and dyspnea were the main symptoms. In the second case an acute respiratory distress was revealing a giant mediastinal lymphangioma in a neonate.

Conventional radiography remains the primary radiologic examination in the evaluation of mediastinal masses. The chest radiograph is used to identify and localize the mass to one of the mediastinal compartments, but provides limited information about the morphology and extent of the lesion.5

The radiographic diagnosis of mediastinal cyst lymphangioma is based on the result of ultrasonographic evaluation and computed tomography.5 The sonographic appearance of lymphangioma is well documented. Lymphangioma appears as multilocular predominantly cystic masses containing septa of variable thickness and solid components.13

CT remains the procedure of choice to further evaluate a mediastinal mass shown on chest radiography. It evaluates the size of the mass, determine its location within the mediastinum, define its morphology and delineate its extent.5, 14 As described by Shaffer et al.,15 the most common characteristics of cystic lymphangioma were a well-capsulated, smoothly marginated and cystic. Her density is close to the signal of water or protein.14

Other cystic masses that must be considered in the differential diagnosis include bronchogenic cyst, cystic thymoma, cystic teratoma, and malignant lymphoma.3, 5

Recently magnetic resonance imaging has the potential avantage of conclusive demonstration of cystic components, it revealed the multilocular septum within the mass which is an important finding and demonstrate better the relationship to adjacent structures.13, 14 Siegel et al. reported a typical MR appearance of cystic lymphangioma with low signal intensity on T1W1 and high signal intensity on T2W1 and focal inhomogeneities within the lesions.16

Pathologically, lymphangiomas are classified into three types, i.e unilocular, cavernous and intermediate types.14 In order to correlate pathological and imaging findings, Charruau et al.,17 have studied CT an MR imaging features of mediastinal lymphangioma in adults. They conclude that the most common unilocular type is a non-enhancing-walled mass on CT. A less frequent cavernous type can be suggested based on a multiseptated and loculated mass on CT and/or MR examination.17

Spontaneous regression of mediastinal cystic lymphangioma has not been reported. Usually a progressive increase in size occurs and, in most cases, it would not be prudent to recommend observation of a cystic lymphangioma of the mediastinum.3

Surgical excision is the treatment of choice.6, 7, 18 A surgical approach via a thoracotomy is easy and provides excellent exposure for a meticulous dissection of all the cysts which is essential to prevent recurrence.11 Since lymphangioma is a benign tumor, but infiltrating the adjacent vital structures must be delineated and safeguarded.11

A good prognosis could be achieved if the tumor is completely resected.6, 11, 19 Thoracoscopic treatment of mediastinal cystic lymphangioma has been reported.20 But compressive cysts with lung distension and mediastinal shift remain a contraindication for these procedure.20 Unlike cervical lymphangiomas, treatment of mediastinal lymphangioma with intralesional OKT-432 is a limited option since a reactionary increase in the size of the cysts can compromise respiration.21

In conclusion, an isolated lymphangioma of the mediastinum in the pediatric population is exceedingly uncommon.

Here we reported two atypical cases because of age at diagnosis in the second case (a neonate with acute respiratory distress), their location (a posterior mediastinal cyst lymphangioma) and their large size exceeding 10cm of diameter in the second case, and a giant thoracoabdominal cyst lymphangioma in the first case. In both cases, complete resections were performed without any complications and without any recurrences.

Back to Article Outline

Conflict of interest statement 

None of the authors have a conflict of interest to declare in relation to this work.

Back to Article Outline

References 

  1. Glasson MJ, Taylor SF. Cervical, cervicomediastinal and intrathoracic lymphangioma. Prog Pediatr Surg. 1991;27:62–83
  2. Brown LR, Reiman HM, Rosenow ED, Gloviczki PN, Diverti MB. Intrathoracic lymphangioma. Mayo Clin Proc. 1986;61:882–892
  3. Wright CC, Cohen DM, Vegunta RK, Davis TJ, King DR. Intrathoracic cystic hygroma: a report of three cases. J Pediatr Surg. 1996;31:1430–1432
  4. Alqahtan A, Nguyen LT, Flageole H, Show K, Laberge JM. 25 years' experience with lymphangiomas in children. J Pediatr Surg. 1999;34:1164–1168
  5. Merten DF. Diagnostic imaging of mediastinal masses in children. AJR. 1992;158:825–832
  6. Kavunkal AM, Ramkumar J, Gangahumaiah S, Parimelazhagan KN, Cherian VK, Nadu T. Isolated mediastinal cystic lymphangioma in a child. J Thorac Cardiovasc Surg. 2007;134:1596–1597
  7. Curley SA, Ablin DS, Kosloske AM. Giant cystic hygroma of the posterior mediastinum. J Pediatr Surg. 1989;24:398–400
  8. Takanatsu H, Noguchi H, Tahara H, Nagushi Y. Giant mediastinal lymphangioma in a neonate: report of a case. Surg Today. 1996;26:527–531
  9. Ch Yu, Lu WT, Wu FF, Liao YJ, Liang WW. Mediastinal lymphangioma in an infant. J Formos Med Assoc. 1996;95(3):255–257
  10. Agartan C, Olguner M, Mirac F, Aktug T. A case of mediastinal cystic hygroma whose only symptom was hoarseness. J Pediatr Surg. 1998;33:642–643
  11. Handa R, Kale R, Upadhyay KK. Isolated mediastinal lymphangioma herniating through the intercostal space. Asian J Surg. 2004;27(3):241–242
  12. Balkaran BN, Maharaj P, Pitt Miller P. Intrathoracic cystic hygroma in an infant with respiratory failure. West Indian Med J. 1997;46:128–129
  13. Borecky N, Gudinchet F, Laurini R, Duvoisin B, Hoklfeld J, Shunyder P. Imaging of cervico-thoracic lymphangiomas in children. Pediatr Radiol. 1995;25:127–130
  14. Oshikiri T, Morikawa T, Jinushi E, Kawakami Y, Katoh H. Five cases of lymphangioma of the mediastinum in adult. Ann Thorac Surg. 2001;7:103–105
  15. Shaffer K, Christenson MLR, Patzef JR, Young S, Farver CF. Thoracic lymphangioma in adults: CT and MR imaging futures. AJR. 1994;162:283–289
  16. Siegel MJ, Glazer HS, St Amour TE, Rosenthal DD. Lymphangiomas in children: MR imaging. Radiology. 1989;170:467–470
  17. Charruau L, Parrens M, Jougon J, Montodon M, Blachere H, Latrabe V, et al. Mediastinal lymphangioma in adults: CT and MR imaging features. Eur Radiol. 2000;10(8):1310–1314
  18. Yildirim E, Dural K, Kaplan T, Skinci U. Cystic lymphangioma: report of two atypical cases. Interact Cardiovasc Thorac Surg. 2004;3:63–65
  19. Hancock BJ, St-Vil Dickens, Luks Francois F, Di Lorenzo M, Blanchard H. Complications of lymphangiomas in children. J Pediatr Surg. 1992;27:220–226
  20. Michel JL, Revillon Y, Montupet P, Sarvat F, Sarnachi S, Sayegh N, et al. Thoracoscopic treatment of mediastinal cysts in children. J Pediatr Surg. 1998;33:1745–1748
  21. Okazaki T, Iwatani S, Yanai T, Kobayashi H, Kato Y, Mausaka T, et al. Treatment of lymphangioma in children: our experience of 128 cases. J Pediatr Surg. 2007;42:386–389

PII: S1755-0017(08)00059-6

doi:10.1016/j.rmedc.2008.07.005

Respiratory Medicine CME
Volume 1, Issue 4 , Pages 270-273, 2008

Article Tools

* Image Usage & Resolution