|Year : 2016 | Volume
| Issue : 4 | Page : 210-213
Challenges in complete surgical resection of giant cystic hygroma
Amit K Sinha1, Bindey Kumar1, Anil Kumar2, Prem Kumar3, Zaheer Hasan1, Chandni Sinha4
1 Department of Pediatric Surgery, AIIMS, Patna, Bihar, India
2 Department of Surgery, AIIMS, Patna, Bihar, India
3 Department of Radiology, AIIMS, Patna, Bihar, India
4 Department of Anaesthesia, AIIMS, Patna, Bihar, India
|Date of Web Publication||8-Dec-2017|
Dr. Amit K Sinha
Department of Pediatric Surgery, AIIMS, Patna, Bihar
Source of Support: None, Conflict of Interest: None
Background: Giant cystic hygroma is a benign cystic lesion that is disfiguring and a life threatening lymphatic malformation. Surgical resection is the main modality of treatment. Complete surgical resection of giant cystic hygroma is difficult and challenging.
Patients and Methods: We discuss 10 cases of giant cystic hygroma wherein complete surgical resection was done. These cases were operated between 1996 and 2015. The preparation of patients, challenges, and outcome of the procedure were evaluated and recorded.
Results: The age of the 10 cases we evaluated ranged from 1 month to 10 years. Five patients less than 1 year in age presented with respiratory embarrassment and feeding problems. Older children (5 patients) presented with disfigurement of face including visible non-tender mass. All children had some degree of feeding difficulties. One case had mediastinal involvement and presented with respiratory distress. Postoperatively, 6 patients had blood transfusion, while 5 required mechanical ventilation.
Conclusion: This article focuses on difficulties encountered during surgical resection of giant cystic hygroma due to its varied clinical presentation, which is unique to the large size of the mass. These include anesthetic difficulties, operative difficulties in terms of identifying vital structures, bleeding, and cosmesis, and operative sequel and complications.
Keywords: Cervico-facial lymphatic malformation, cystic hygroma, neck mass
|How to cite this article:|
Sinha AK, Kumar B, Kumar A, Kumar P, Hasan Z, Sinha C. Challenges in complete surgical resection of giant cystic hygroma. Arch Int Surg 2016;6:210-3
| Introduction|| |
Lymphatic malformation is a benign condition consisting of masses of abnormal lymphatic channels. It is disfiguring and sometimes life threatening too. The reported incidence is 1 in 2000 to 4000 live births. It arises from embryological disturbances in the development of lymphatic system mostly due to congenital blockade or developmental arrest of primordial lymph channels. It can be seen in any part of the body having rich lymphatic areas like head and neck (45% to 52%), axilla, mediastinum, groin, or retroperitoneum., Large cystic lymphatic malformation can be diagnosed in-utero with prenatal ultrasonography as early as the 2nd trimester., The lesions are generally noted at birth and majority become evident at the age of 2 years. Postnatally, these lesions are readily diagnosed by physical examination. There are three morphologic types of cystic lymphatic lesions: macrocystic, microcystic, and combined. Macrocystic lesions are multi-locular structures consisting of cysts of variable sizes. These are often located below the level of mylohyoid muscle and involve anterior and posterior cervical triangles. The microcystic form is commonly found above the level of mylohyoid muscle and involve the oral cavity, oropharynx, tongue, parotid gland, submandibular gland, and pre-epiglottic space., The clinical course of lymphatic malformation is varied. It persists throughout life and grows proportionately with the age of the patient. The lesion may grow dramatically after upper respiratory tract infections, trauma, or inflammation. The treatment of lymphatic malformation depends upon the clinical presentation, size, site, and its complications. Complete surgical resection is the treatment of choice but poses unique challenges to the surgical team. A variety of other modalities of treatment including aspiration, drainage procedure, radiotherapy and sclerotherapy have being used to treat lymphatic malformation without much success. Sclerotherapy is frequently used as an alternative mode to surgery but is not completely curative and will lead to amelioration of symptoms in only 40% of the patients. Sclerosants are mainly effective in macrocystic variety. Commonly used sclerosants are Bleomycin and OK-432 (low-virulence Su strain group - a streptococcus pyogenes cultured with penicillin). Lesions involving the tongue poses difficult scenario due to non-feasibility of complete resection and its recurrence is common. These lesions may be treated by tongue reduction in combination with topical laser, electrocautery, or coblation therapy.,
An important variation of this lymphatic malformation worth mentioning is giant cystic hygroma. It involves the tongue, floor of mouth, neck, and mediastinum. This giant form can give rise to significant disfigurement, functional deficit, and causes problems in feeding and breathing. Due to upper respiratory tract infection, these lesions may enlarge suddenly and become life threatening to the patient. Management of these lesions is a challenge to the surgeon.
| Patients and Methods|| |
This retrospective study was conducted for cases between 1996 and 2015 and includes 10 cases of unilateral or bilateral cervico-facial lymphatic malformations of dimensions more than 10 × 10 × 10 cm [Figure 1]. All children with respiratory embarrassment were initially stabilized with respiratory support and 5 patients required mechanical ventilation preoperatively. After stabilization, all patients were investigated with chest X-ray, ultrasonography of local area, and CT scan or MRI of neck and chest. After stabilization and investigation, all patients were scheduled for elective surgery. A multidisciplinary team approach led to successful elective resection in all these patients. Early institution of enteral feeding was done in all patients through nasogastric tube and none of the patient required feeding gastrostomy.
| Results|| |
All patients were discharged successfully and kept under regular follow up. Important preoperative and postoperative aspects of patients are summed up in [Table 1]. The age of the patients age ranged from 1 month to 10 years. None of the cases were supervised antenatally and all were diagnosed in postnatal period. The mode of presentation in the patients having age less than one year (5 patients) were respiratory embarrassment and feeding problems. Older children (5 patients) presented with disfigurement of face, i.e., visible non-tender mass. All children had some degree of feeding difficulties. One case had mediastinal involvement and presented with respiratory distress. None of the cases required intra-operative blood transfusion, whereas postoperative blood transfusion was done in 6 patients. Five patients especially of younger age groups required mechanical ventilation in postoperative period. Three patients developed sepsis in postoperative period that was taken care of with intravenous antibiotics and supportive measures.
|Table 1: Important preoperative and postoperative aspects of Giant cystic hygroma|
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| Discussion|| |
The management of giant cystic hygroma is a difficult scenario for both the parents as well as the surgeon. Due to availability of different modes of treatment, it is very difficult for the parents to choose complete surgical resection as definitive treatment. But with improved techniques of excision and development of sophisticated instruments, complete surgical excision becomes easier. Also, surgical resection is important because it gives histological diagnosis and chances of recurrences become less. It also reduces morbidity like recurrent infections, airway compromise, abnormal speech, poor dental hygiene, and improper food intake. That is why opting for the surgical resection is a wise decision. This retrospective analysis aims to highlight the difficulties encountered during management of giant cystic hygroma. The challenges encountered during the surgical excision of giant cystic hygromas include:
- Positioning and airway management: For any successful surgery positioning of the patient in operation theatre is the most important measure. Due to the large size and often bilaterality of the lesion, positioning of these patients is very difficult and varies from patient to patient. We made individual plans of positioning in each of the patient. Large size and involvement of the oropharyngeal area made intubation of these patients difficult. To combat these problems cases were discussed preoperatively with the anesthesia team and help of anesthetist having experience of difficult pediatric intubation was taken. Use of fibre-optic bronchoscope was also helpful. A good venous access and an arterial line were secured. Possibility of emergency tracheostomy was discussed with the parents initially and all arrangements were kept ready. But, none of the patients required emergency tracheostomy
- Identification of vital structures: Considering that these lesions were not encapsulated and tend to interdigitate into tissue planes, enveloping neural and vascular structures, plan for identification of these important neurovascular structures was made preoperatively. This was done by proper investigation with the help of ultrasound, CT scan, and/or MRI of the local area. With these investigations important anatomical landmarks were defined. Intra-operatively, identification of these important neurovascular structures was done with the use of sophisticated equipments such as intra-operative color doppler and hand-held nerve stimulator. The other measure to prevent injury of these vital structures was to remove the tumor in piecemeal
- Bleeding: Due to the large size and proximity with vital vascular structures, bleeding was anticipated during surgical resection of these lesions. For this, we took the help of anatomical landmarks before cutting any part of tissue during surgery. We defined each structure before cutting because involvement and encasement of vital structures by giant cystic hygroma and later on superadded hemorrhage made normal anatomy distorted. Also, the important vital structures were always displaced and splayed over giant cystic hygroma and these ambiguities become more prominent as cyst with hemorrhage simulated major vascular structures. To deal with these problems, use of intra-operative color doppler was taken and it was helpful to identify major vessels
- Involvement of areas other than neck: A giant cystic hygroma may involve different areas of head and neck and for its complete surgical removal help of multidisciplinary team was taken. The lead members of this multidisciplinary team were pediatric anesthetist, plastic, ENT, Cardio- thoracic and vascular surgery (CTVS), and dental surgeons
- Post-operative feeding: Early institution of enteral feeding is one of the most important aspects for management of any surgical patient. For this, a secure naso-gastric tube was required. Also, the need of feeding gastrostomy and total parenteral nutrition was planned and discussed with the parents before surgery.
| Conclusion|| |
For a successful complete surgical resection of giant cystic hygroma, a planned surgical resection should be made with multidisciplinary teams. The extent and approach of resection must be defined preoperatively and anatomical structures must be defined methodically using a series of anatomical guide points, which may be selected by imaging and clinical examination. Special equipment like intra-operative color doppler and hand-held nerve stimulator can be used to delineate the vital structures. Finally, the need for tracheostomy and feeding gastrostomy must be planned preoperatively.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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