J Korean Assoc Pediatr Surg JKAPS Journal of the Korean Association of Pediatric Surgeons 2383-5036 Korean Association of Pediatric Surgeons 10.13029/jkaps.2015.21.2.17 Original Article Surgical Treatment of Difficult Cervicofacial Lymphangioma in Children Hwang Deokbi 1 Lee Sanghoon 1 Lim So Young 2 Lee Suk-Koo 1 Seo Jeong-Meen 1 Division of Pediatric Surgery, Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. Department of Plastic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. Correspondence: Jeong-Meen Seo. Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 06351, Korea. Tel: +82-2-3410-0282, Fax: +82-2-3410-0040, jm0815.seo@samsung.com 12 2015 22 12 2015 21 2 17 23 28 07 2015 21 09 2015 12 10 2015 Copyright © 2015 by the Korean Association of Pediatric Surgeons 2015 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Purpose

Cervical lymphangiomas are rare lymphovascular malformations arising in the neck, which form huge fluid-containing cysts. Treatment of the malformation consists of surgery and sclerotherapy. However, the optimal approach is still controversial. Here, we describe a series of cervical lymphangiomas which have been treated with surgical approaches.

 Methods

We retrospectively investigated the medical records of 82 patients who had been diagnosed with cervicofacial lymphangioma from 2001 to 2012 in our center. A closed suction drainage with negative pressure was placed on the operative lesion following excision to prevent reaccumulation of lymphatic fluid and the drainage tube was removed after injecting OK-432 through the tube.

 Results

Twelve patients underwent surgical excision of cervical lymphangioma. The median patient age was 3 months at the time of the operation. The patients have been followed-up over a period of 34 months. When lesions were located near vital organs such as the trachea or carotid artery or did not respond to repetitive OK-432 injections, surgical treatment might bring good outcomes. However, swallowing difficulty, lip palsy, or dyslalia due to adjacent nerve damage temporarily appeared as postoperative complications. Five children had tracheostomy due to tracheal or subglottic stenosis and 2 patients had gastrostomy due to aspiration while they eat after surgery.

 Conclusion

Surgery for cervicofacial lymphangioma should be conducted carefully in selective cases. A well thought-out surgical plan with a multidisciplinary surgical team approach and placement of closed suction drainage tube after surgery and adjuvant OK-432 sclerotherapy through drainage tube seem to be helpful for good outcome.

 Cervical lymphangioma Lymphatic malformation OK-432 sclerotherapy Excision Suction drainage

No potential conflict of interest relevant to this article was reported.

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(A) Patient No. 2 was diagnosed with cervical lymphangioma prenatally by fetal MRI. (B) The patient was about to have a surgery 18 days after birth through EXIT procedure. (C) This picture was taken 1 year and 8 months after surgery. EXIT, ex utero intrapartum treatment.

(A) Patient No. 4 was transferred to our hospital after OK-432 sclerotherapy. (B) This patient had a surgery about lymphangioma involving from right temporal area to neck. (C) This photograph was taken 9 months after surgery and a subsequent sclerotherapy.

(A) Patient No. 6 visited our hospital after having gotten several times of sclerotherapies using OK-432 and ethanol. (B) This photograph was taken 3 years after surgery.

(A) MRI shows macrocystic lymphatic malformations which are involving from right neck to axilla before OK-432 sclerotherapy (patient No. 9). (B) Intralesional hemorrhage happened one month after sclerotherapy.

(A) Sagittal T2-weighted MRI shows cervical lymphangioma which are infiltrating tongue base (patient No. 11). (B) Decreased lymphatic malformation after surgery.

Preoperative Characteristics of 12 Patients with Cervical Lymphangioma and perioperative Details

POD, postoperative day; EXIT, ex utero intrapartum treatment; RFA, radiofrequency ablation.

a)Median 300 min, range 59-559 min. b)Mean 19 days, range 7-46 days.

Distribution of Our Patients according to the Anatomic Location of Lymphangiomas