- Email: [email protected]
Lymphatic malformation of the parapharyngeal space
British Journal of Oral and Maxillofacial Surgery (2004) 42, 33—35
Lymphatic malformation of the parapharyngeal space Erdinc Aygenc*, Fatih Fidan, Cafer Ozdem Department of Head and Neck Surgery, Numune Training and Research Hospital, Second Otolaryngology, Ergin sok 27/11 Mebusevleri, 06580 Ankara, Turkey Accepted 8 September 2003
KEYWORDS Congenital tumours of head and neck; Lymphatic malformation; Cystic hygroma; Parapharyngeal space
Summary Lymphatic malformations are rare benign congenital lesions. A 28-monthold girl presented with a parapharyngeal lymphatic malformation. A cystic lesion had been diagnosed at 18 weeks’ gestation by ultrasonography and she had been followed up conservatively until her referral to our department. We used a transcervical approach to excise the parapharyngeal lesion and 1 year later, there was no sign of recurrence. © 2003 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Introduction The variety of anatomic structures within or bordering the parapharyngeal space (PPS) accounts for the many types of tumours that involve this area. Tumours of neural origin and benign salivary gland tumours are the most common.1,2 The cysts that make up a lymphatic malformation vary in size from a few millimetres to more than several centimetres in diameter.3 They occur mainly in the head and neck, but may be found anywhere. About half are present at birth, and nearly all detected by the age of 2 years.
Case report A 28-month-old girl was admitted to our department with a painless soft mass on the right side
*Corresponding author. Tel.: +90-312-215-6343; fax: +90-312-481-6992. E-mail address: [email protected] (E. Aygenc).
of the neck. She had moderate dysphagia and recurrent rhinopharyngeal infections. A cystic lesion had been diagnosed at 18 weeks’ gestation by ultrasonography. Fetal karyotype and family history were unremarkable. She had been observed conservatively until referral to our hospital. Physical examination showed medial displacement of the right palatine tonsil. A soft mass became enlarged while she was crying. The cranial nerves were intact. Ultrasonography showed a hypoechogenic multilocular cystic mass with septa of variable thickness and size. Magnetic resonance imaging showed a heterogeneous complex cystic lesion of the right parapharyngeal space medial to the great vessels. A lymphatic malformation (cystic hygroma) was diagnosed, according to De Serres et al.4 At operation a submandibular skin incision was made and a subplastysmal flap raised. The posterior facial vein was identified, ligated, and suspended to preserve the mandibular branch of the facial nerve. The facial artery was ligated and the submandibular gland retracted anteriorly. The lesion was detected in the parapharyngeal space and was adherent to the internal jugular vein. The carotid
0266-4356/$ — see front matter © 2003 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/S0266-4356(03)00191-8
34
Figure 1 isolated.
E. Aygenc et al.
The resected specimen that consisted of multiple cysts of various sizes, some interconnected and some
sheath was identified and dissected upwards. The lesion extended from the parapharyngeal space to the carotid bifurcation. We used blunt dissection to separate the mass from the neurovascular structures in the parapharyngeal space (Fig. 1). She was discharged home well on the third postoperative day. Pathological examination showed a macrocystic lymphangioma (cystic hygroma). A year later, there was no sign of recurrence.
Discussion Tumours in the parapharyngeal space include primary neoplasms, direct extension from adjacent regions, and metastatic disease. Lymphangiomas are very rare. They arise from a congenital blockage or arrest of normal development of the primordial lymph channels.5 The cysts enlarge from the accumulation of lymph and the formation of additional endothelial sprouts. These sprouts force the lesion into areas of least resistance, between muscle and vessels, invading tissue planes and causing atrophy, fibrosis, and hyalinisation of engulfed tissue. Lymphatic malformations do not involute; they expand or contract depending on the ebb and flow of lymphatic fluid and the occurrence of inflammation and intralesional bleeding. Most of these lesions are evident at birth or detected before 2 years of age; but they can suddenly appear in an older child and occasionally in adolescents or adults. Treatment of lymphatic malformations can be challenging, and various methods have been tried, including operation, radiation therapy, aspiration, and sclerotherapy.6 Although emergency decompression by needle aspiration may be necessary if there is obstruction to the airway, this alleviates symptoms only temporarily and cannot be curative. Large cysts can be treated by aspiration of lymphatic fluid and instil-
lation of a sclerosant such as tetracycline, sodium diatrizoate tetrahydrate (Ethibloc), pure ethanol, sodium tetradecyl sulphate, sodium morrhuate, 50% dextrose, or bleomycin. Recent publications reporting the use of picibanil (OK-432–—a killed strain of group A streptococcus) have shown promising results in the treatment of unresectable lesions.6 Although macrocystic lesions are more likely than microcystic lesions to respond, recurrence is common after sclerotherapy. Scarring produced by diffusion of the sclerosing agent through the cyst walls makes subsequent operation, if needed, far more difficult and places vital structures adjacent to the cyst at risk of injury. Local infection and paralysis of the marginal mandibular branch of the facial nerve has been reported after aspiration.3 Resection is the only way to ensure a cure. Although early operation is often advised, some authors advise that treatment should be primarily conservative until the end of the first year of life. Operation may be delayed until at least 6 months of age when the patient will better tolerate a general anaesthetic. Complete excision may not be feasible in some cases because of extension around vital structures. Recurrence is common if removal is incomplete. Transsected lymphatic channels regenerate after subtotal excision. Surgeons who operate in the parapharyngeal space must be thoroughly familiar with the complex anatomy of blood vessels, nerves, and muscles. Children with cervicofacial lymphatic malformation pose special challenges because of risks of airway obstruction, functional disability, surgical complications, and recurrent or persistent lesions.
References 1. Maran AGD, Mackenzie IJ, Murray JAM. The parapharyngeal space. J Laryngol Otol 1984;98:371—80.
Lymphatic malformation of the parapharyngeal space 2. Batsakis JG, Sneige N. Parapharyngeal and retropharyngeal space disease. Ann Otol Rhinol Laryngol 1989;98:320—1. 3. Hamoir M, Plouin-Gaudon I, Rombaux P, Francois G, Cornu AS, Desuter G, et al. Lymphatic malformations of the head and neck: a retrospective review and a support for staging. Head Neck 2001;23:326—37. 4. De Serres L, Sie K, Richardson M. Lymphatic malformation of the head and neck: a proposal for staging. Arch Otolaryngol Head Neck Surg 1995;121:577—82.
35 5. Ricciardelli EJ, Richardson MA. Cervicofacial cystic hygroma. Patterns of recurrence and management of the difficult case. Arch Otolaryngol Head Neck Surg 1991;117:546— 53. 6. Sung MW, Lee DW, Kim DY, Lee SJ, Hwang CH, Park SW, et al. Sclerotherapy with picibanil (OK-432) for congenital lymphatic malformation in the head and neck. Laryngoscope 2001;111:1430—3.
HISTORICAL CASE WILHELM FRIEDRICH VON LUDWIG (1790—1865) German surgeon and obstetrician. Most famous eponym: Ludwig angina–—a bilateral life threatening diffuse infection and inflammation of the sublingual, submental and submandibular spaces. Complications include asphyxia and cavernous sinus thrombosis with subsequent meningitis. Staphylococci are present in nearly all cases and fusiform bacilli and streptococci are also common. von Ludwig began his medical career as an apprentice to a surgeon, before formally studying medicine at the University of Tübingen, where he qualified in 1811. During the 1812 Napoleonic campaign against Russia, Ludwig fell ill and was imprisoned by the Russians. He was finally released after he had provided his
medical services to the Russians for 2 years. Ludwig was subsequently appointed Professor of Surgery and Midwifery at Tübingen in 1815, and became an authority on obstetrics and surgery, both with the royal family and among his colleagues. The description of Ludwig’s angina was his only notable clinical observation. He suffered with cataracts and renal stones, and when he died left the majority of his fortune to found a hospital for the poor in Württemberg, which was opened in 1874. 1. von Ludwig WF. Uber eine in neurer Zeit weiderholt hier viogekommene Foim von HalsentZung. Med Conesp Wurtt Aitzl Verein 1836;6:21—5. P. A. Brennan
Lymphatic malformation of the parapharyngeal space Erdinc Aygenc*, Fatih Fidan, Cafer Ozdem Department of Head and Neck Surgery, Numune Training and Research Hospital, Second Otolaryngology, Ergin sok 27/11 Mebusevleri, 06580 Ankara, Turkey Accepted 8 September 2003
KEYWORDS Congenital tumours of head and neck; Lymphatic malformation; Cystic hygroma; Parapharyngeal space
Summary Lymphatic malformations are rare benign congenital lesions. A 28-monthold girl presented with a parapharyngeal lymphatic malformation. A cystic lesion had been diagnosed at 18 weeks’ gestation by ultrasonography and she had been followed up conservatively until her referral to our department. We used a transcervical approach to excise the parapharyngeal lesion and 1 year later, there was no sign of recurrence. © 2003 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Introduction The variety of anatomic structures within or bordering the parapharyngeal space (PPS) accounts for the many types of tumours that involve this area. Tumours of neural origin and benign salivary gland tumours are the most common.1,2 The cysts that make up a lymphatic malformation vary in size from a few millimetres to more than several centimetres in diameter.3 They occur mainly in the head and neck, but may be found anywhere. About half are present at birth, and nearly all detected by the age of 2 years.
Case report A 28-month-old girl was admitted to our department with a painless soft mass on the right side
*Corresponding author. Tel.: +90-312-215-6343; fax: +90-312-481-6992. E-mail address: [email protected] (E. Aygenc).
of the neck. She had moderate dysphagia and recurrent rhinopharyngeal infections. A cystic lesion had been diagnosed at 18 weeks’ gestation by ultrasonography. Fetal karyotype and family history were unremarkable. She had been observed conservatively until referral to our hospital. Physical examination showed medial displacement of the right palatine tonsil. A soft mass became enlarged while she was crying. The cranial nerves were intact. Ultrasonography showed a hypoechogenic multilocular cystic mass with septa of variable thickness and size. Magnetic resonance imaging showed a heterogeneous complex cystic lesion of the right parapharyngeal space medial to the great vessels. A lymphatic malformation (cystic hygroma) was diagnosed, according to De Serres et al.4 At operation a submandibular skin incision was made and a subplastysmal flap raised. The posterior facial vein was identified, ligated, and suspended to preserve the mandibular branch of the facial nerve. The facial artery was ligated and the submandibular gland retracted anteriorly. The lesion was detected in the parapharyngeal space and was adherent to the internal jugular vein. The carotid
0266-4356/$ — see front matter © 2003 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/S0266-4356(03)00191-8
34
Figure 1 isolated.
E. Aygenc et al.
The resected specimen that consisted of multiple cysts of various sizes, some interconnected and some
sheath was identified and dissected upwards. The lesion extended from the parapharyngeal space to the carotid bifurcation. We used blunt dissection to separate the mass from the neurovascular structures in the parapharyngeal space (Fig. 1). She was discharged home well on the third postoperative day. Pathological examination showed a macrocystic lymphangioma (cystic hygroma). A year later, there was no sign of recurrence.
Discussion Tumours in the parapharyngeal space include primary neoplasms, direct extension from adjacent regions, and metastatic disease. Lymphangiomas are very rare. They arise from a congenital blockage or arrest of normal development of the primordial lymph channels.5 The cysts enlarge from the accumulation of lymph and the formation of additional endothelial sprouts. These sprouts force the lesion into areas of least resistance, between muscle and vessels, invading tissue planes and causing atrophy, fibrosis, and hyalinisation of engulfed tissue. Lymphatic malformations do not involute; they expand or contract depending on the ebb and flow of lymphatic fluid and the occurrence of inflammation and intralesional bleeding. Most of these lesions are evident at birth or detected before 2 years of age; but they can suddenly appear in an older child and occasionally in adolescents or adults. Treatment of lymphatic malformations can be challenging, and various methods have been tried, including operation, radiation therapy, aspiration, and sclerotherapy.6 Although emergency decompression by needle aspiration may be necessary if there is obstruction to the airway, this alleviates symptoms only temporarily and cannot be curative. Large cysts can be treated by aspiration of lymphatic fluid and instil-
lation of a sclerosant such as tetracycline, sodium diatrizoate tetrahydrate (Ethibloc), pure ethanol, sodium tetradecyl sulphate, sodium morrhuate, 50% dextrose, or bleomycin. Recent publications reporting the use of picibanil (OK-432–—a killed strain of group A streptococcus) have shown promising results in the treatment of unresectable lesions.6 Although macrocystic lesions are more likely than microcystic lesions to respond, recurrence is common after sclerotherapy. Scarring produced by diffusion of the sclerosing agent through the cyst walls makes subsequent operation, if needed, far more difficult and places vital structures adjacent to the cyst at risk of injury. Local infection and paralysis of the marginal mandibular branch of the facial nerve has been reported after aspiration.3 Resection is the only way to ensure a cure. Although early operation is often advised, some authors advise that treatment should be primarily conservative until the end of the first year of life. Operation may be delayed until at least 6 months of age when the patient will better tolerate a general anaesthetic. Complete excision may not be feasible in some cases because of extension around vital structures. Recurrence is common if removal is incomplete. Transsected lymphatic channels regenerate after subtotal excision. Surgeons who operate in the parapharyngeal space must be thoroughly familiar with the complex anatomy of blood vessels, nerves, and muscles. Children with cervicofacial lymphatic malformation pose special challenges because of risks of airway obstruction, functional disability, surgical complications, and recurrent or persistent lesions.
References 1. Maran AGD, Mackenzie IJ, Murray JAM. The parapharyngeal space. J Laryngol Otol 1984;98:371—80.
Lymphatic malformation of the parapharyngeal space 2. Batsakis JG, Sneige N. Parapharyngeal and retropharyngeal space disease. Ann Otol Rhinol Laryngol 1989;98:320—1. 3. Hamoir M, Plouin-Gaudon I, Rombaux P, Francois G, Cornu AS, Desuter G, et al. Lymphatic malformations of the head and neck: a retrospective review and a support for staging. Head Neck 2001;23:326—37. 4. De Serres L, Sie K, Richardson M. Lymphatic malformation of the head and neck: a proposal for staging. Arch Otolaryngol Head Neck Surg 1995;121:577—82.
35 5. Ricciardelli EJ, Richardson MA. Cervicofacial cystic hygroma. Patterns of recurrence and management of the difficult case. Arch Otolaryngol Head Neck Surg 1991;117:546— 53. 6. Sung MW, Lee DW, Kim DY, Lee SJ, Hwang CH, Park SW, et al. Sclerotherapy with picibanil (OK-432) for congenital lymphatic malformation in the head and neck. Laryngoscope 2001;111:1430—3.
HISTORICAL CASE WILHELM FRIEDRICH VON LUDWIG (1790—1865) German surgeon and obstetrician. Most famous eponym: Ludwig angina–—a bilateral life threatening diffuse infection and inflammation of the sublingual, submental and submandibular spaces. Complications include asphyxia and cavernous sinus thrombosis with subsequent meningitis. Staphylococci are present in nearly all cases and fusiform bacilli and streptococci are also common. von Ludwig began his medical career as an apprentice to a surgeon, before formally studying medicine at the University of Tübingen, where he qualified in 1811. During the 1812 Napoleonic campaign against Russia, Ludwig fell ill and was imprisoned by the Russians. He was finally released after he had provided his
medical services to the Russians for 2 years. Ludwig was subsequently appointed Professor of Surgery and Midwifery at Tübingen in 1815, and became an authority on obstetrics and surgery, both with the royal family and among his colleagues. The description of Ludwig’s angina was his only notable clinical observation. He suffered with cataracts and renal stones, and when he died left the majority of his fortune to found a hospital for the poor in Württemberg, which was opened in 1874. 1. von Ludwig WF. Uber eine in neurer Zeit weiderholt hier viogekommene Foim von HalsentZung. Med Conesp Wurtt Aitzl Verein 1836;6:21—5. P. A. Brennan