Surgical excision as primary treatment modality for extensive cervicofacial lymphatic malformations in children
Introduction
Lymphatic malformations are low flow vascular anomalies, the incidence being 1:5000 at birth [1]. These comprise 6% of all paediatric soft tissue tumours [2]. Morphologically these consist of cyst like dilated vascular channels filled with lymph [3]. Lymphatic malformations are classified according to cyst size into macrocystic (few cysts which are at least 2 cm3 in size), microcystic (numerous small cysts which are less than 2 cm3 in size) or mixed [4], [5]. Lymphatic malformations usually present as slow growing soft swellings in the head neck region [6]. They can rapidly increase in size as a result of bleeding or infection, or may rarely decrease in size spontaneously [7]. Lymphatic malformations can be asymptomatic or may affect breathing or swallowing, depending on their size or location.
The treatment modalities for lymphatic malformations can be divided into active observation, sclerotherapy [8], [9], [10], surgical excision [11] or a combination of these. Surgical excision has the advantage of usually a single procedure to excise the disease. It offers potential complete eradication of the disease and a lifetime cure. Sclerotherapy will always leave residual malformation.
Review of the literature on this topic revealed many articles on surgical treatment as a part of multimodality treatment or as a salvage treatment for sclerotherapy failure, but only a few articles reporting on results of surgery as the only treatment. The aim of this study was to evaluate the results of a large series of cervicofacial lymphatic malformations in children treated by surgical excision as the primary (first) treatment at a tertiary paediatric referral centre.
Section snippets
Methods
This study was conducted at the department of paediatric otolaryngology at Great Ormond Street Hospital, London. All children with lymphatic malformations under the care of the senior author (BEJH) from January 2001 to December 2010 were evaluated. The data was collected from a prospectively collected database with supplemental information from medical records. These patients were managed in a multidisciplinary setting. The diagnosis was made on clinical basis and confirmed with MRI scan in all
Results
On reviewing the records, a total of 118 children with lymphatic malformations were treated at paediatric otolaryngology department at Great Ormond Street Hospital, under the care of the senior author over a 10 year period. Of these patients 53 underwent surgical excision as the primary treatment modality for cervicofacial lymphatic malformations (Fig. 1). Forty-one patients who underwent sclerotherapy as the initial treatment were excluded. Also excluded were 6 patients who underwent thoracic
Discussion
Cervicofacial lymphatic malformations in children present in the head and neck region in 75% cases [1]. Approximately 50–65% of these are present at birth, 90% are present by 2 years of age and 95% are present by adolescence [12], [13]. These can present with breathing or swallowing difficulties depending on the anatomical site and extent of the lesion. For large lesions in children the choice of treatment is between sclerotherapy and surgical excision. The advantages of surgery are, that the
Conclusion
Cervicofacial lymphatic malformations in children should be managed in a multidisciplinary setting. Surgery remains a very important treatment modality. The majority of patients (80%) in this study had complete or near complete resolution with one surgical procedure. Isolated neck lesions have the best outcomes (100% resolution in this study). Patients with macrocystic disease, achieved complete or near complete resolution in 97% of cases, regardless of the location. Some children with
References (19)
- et al.
Lymphatic malformations: current cellular and clinical investigations
Otolaryngol. Head Neck Surg.
(2010) - et al.
Surgical excision of cervicofacial giant macrocystic lymphatic malformations in infants and children
Int. J. Pediatr. Otorhinolaryngol.
(2009) - et al.
25 years experience with lymphangiomas in children
J. Pediatr. Surg.
(1999) - et al.
Vascular tumours in children and adoloscents: a clinicopathologic study of 228 tumours in 222 patients
Pathol. Annu.
(1993) - et al.
Pathogenesis of lymphangiomas
Virchows Archiv.
(2008) - et al.
Radiofrequency ablation of microcystic lymphatic malformation in the oral cavity
Arch. Otolaryngol. Head Neck Surg.
(2006) - et al.
Treatment of lymphatic malformations of head neck with OK 432 sclerotherapy induce systemic inflammatory response
Eur. Arch. Oto-Rhino-Laryngol.
(2010) - et al.
Total, subtotal and partial surgical removal of cervicofacial lymphangiomas
Arch. Otolaryngol. Head Neck Surg.
(1999) Hemangiomas,cystic hygromas and teratomas of the head and neck
Semin. Pediatr. Surg.
(1994)
Cited by (38)
Risk factors for sequelae after surgery for lymphatic malformations in children
2024, Journal of Vascular Surgery: Venous and Lymphatic DisordersManagement of the head and neck lymphatic malformations in children: A 7-year experience of 91 surgical cases
2023, American Journal of Otolaryngology - Head and Neck Medicine and SurgeryThe VASCERN-VASCA working group diagnostic and management pathways for lymphatic malformations
2022, European Journal of Medical GeneticsCitation Excerpt :This solution causes scarring within the LM, which eventually leads to shrinking or collapse of the malformation (Fig. 1). Percutaneous sclerotherapy has replaced surgery in most cases of macrocystic malformations in the past 30 years (Acevedo et al., 2008; Poldervaart et al., 2009; Bajaj et al., 2011; Furuse et al., 2020; Burrows et al., 2008; Claesson and Kuylenstierna, 2002; Mitsukawa and Satoh, 2012). Macrocystic LMs of moderate size can be easily treated with sclerotherapy.
Effects of sirolimus in the treatment of unresectable infantile hemangioma and vascular malformations in children: A single-center experience
2021, Journal of Vascular Surgery: Venous and Lymphatic DisordersManagement of cervicofacial lymphatic malformations requires a multidisciplinary approach
2021, Journal of Pediatric SurgerySurgical resection of macrocystic lymphatic malformations of the head and neck: Short and long-term outcomes
2020, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :To date, a direct prospective comparison of these modalities has not been performed, and there are no guidelines to direct treatment [4–7]. This is particularly true in patients with purely macrocystic disease as most of the literature describes the treatment of a heterogeneous sample of patients with both macrocystic and microcystic components [4,8–10]. Surgical resection has been discussed as a mainstay of treatment for MLM at our institution due to historic success and experience.