Craniotomy in Klippel-Trenaunay syndrome: Concerns and challenges.

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterised by cutaneous capillary malformations, soft tissue and bone hypertrophy and venous varicosities. The coexistence of Chiari 1 malformation and an intracranial tumour has been rarely reported in the literature. Multisystem involvement of this syndrome mandates adequate preparation and planning, with meticulous conduct of anaesthesia to achieve favourable outcomes. We report a case of KTS syndrome with Chiari 1 malformation who had presented for craniotomy, and thereby discuss the challenges faced during anaesthetic management of these patients for major surgeries. Copyright:

INTRODUCTION

Klippel-Trenaunay syndrome (KTS), originally described as naevus vasculosus osteohypertrophicus, is a rare congenital syndrome of combined capillary-venous-lymphatic malformation associated with soft tissue hypertrophy and skeletal hyperostosis, with an incidence of 3–5/100,000. As an isolated disease, these patients present for excision of spinal haemangiomas, correction of kyphoscoliosis and hip dislocation[1] and fixation of pathological fractures.[2] Its coexistence with Chiari 1[3] and an intracranial tumour[4] has been reported earlier. Moreover, previous reports have discussed the concerns regarding airway management and the possibility of intraoperative massive haemorrhage.[5] In this report, we will discuss the additional challenges faced during anaesthetic management of these patients for major surgeries such as craniotomy.

CASE REPORT

A 17-year-old girl, weighing 50 kg with a history of delayed developmental milestones, had presented with a right frontal swelling [Figure 1a] associated with headache of 3-year duration. She underwent only partial excision of the same swelling 2 years ago in view of excessive bleeding during surgery. Moreover, complete excision of a similar swelling diagnosed as fibrous dysplasia over the contralateral parietal eminence was done 5 years back. On examination, her neurocutaneous markers suggested classical KTS with presence of distinct, linear bordered port-wine stains [Figure 1b] over her trunk, lower back and extensor aspects of bilateral upper and lower limbs and multiple small subcutaneous firm swellings (neurofibromas) over the right thenar eminence, right olecranon and dorsal spine at D4-D10 level. She had lymphoedema of both the lower limbs and disproportionately enlarged feet with clubfoot deformity [Figure 1c]. All these features were reported to be present since birth, with exacerbation of these manifestations following the attainment of menarche at 12 years of age. Magnetic resonance imaging (MRI) demonstrated right frontal calvarial haemangioma measuring 8 cm × 9 cm × 5 cm with intracranial extension compressing the ipsilateral frontal lobe [Figure 1d] and associated Chiari 1 malformation [Figure 1e]. The cerebral angiogram revealed increased vascularity of the lesion with multiple feeders predominantly from the right middle meningeal artery. Preoperative embolisation was considered but not performed in view of concerned risk of cortical infarcts in the presence of multiple feeders. Ultrasound examination of the abdomen excluded hepatic haemangiomas and other solid organ involvement. In addition, cardiac workup ruled out associated congenital heart disease. Her chest X-ray revealed left hemidiaphragm elevation with a possible eventration of the diaphragm. Blood investigations including coagulation profile were normal. She was scheduled for craniotomy and excision of the haemangioma. Preoperative preparation included continuation of levetiracetam 500 mg thrice daily, arrangement of adequate blood products in view of anticipated blood loss and written informed consent. As mask ventilation was easy, standard anaesthetic induction was performed. In view of Chiari malformation and anticipated soft tissue changes in the airway, intubation was performed using C-Mac videolaryngoscope at the first attempt. Soft tissue overgrowth in the region of palate was observed during intubation. The patient was positioned carefully and craniotomy was performed [Figure 1f]. Balanced anaesthesia was used with sevoflurane and intermittent boluses of opioids and muscle relaxants. Intraoperatively, 1.5 L of crystalloids (plasmalyte) were administered with adequate urine output obtained. The intraoperative blood loss was 2500 mL over 3-hour duration of surgery and this was replaced with 5 units of packed red cells, 3 units of fresh frozen plasma and 2 units of platelets. Extubation and recovery from anaesthesia were uneventful.

Figure 1

(a) Preoperative image showing right frontal scalp swelling with right facial hyperostosis causing facial asymmetry; (b) Bilateral acral hypertrophy with port-wine stain; (c) Bilateral lymphoedema with hypertrophy of toes; (d) Preoperative MRI brain image showing right calvarial lesion with intracranial extension; (e) Preoperative MRI image showing type 1 Chiari malformation; (f) Intraoperative image showing calvarial hyperostosis and tumour

DISCUSSION

Patients with KTS present unique challenges during the perioperative period for both the anaesthesiologist and the surgeon. The anaesthetic concerns include the challenges faced during vascular access necessitating the use of ultrasonography to diagnose and avoid inadvertent injury to superficial arteriovenous fistulous malformations. Similar concerns exist during scalp block regarding inadvertent local anaesthetic injection into occult subcutaneous scalp haemangiomas. Presence of associated Chiari 1 malformation requires care (avoidance of excessive head and neck movement) during intubation and positioning. Oropharyngeal neurofibromatosis lesions may pose difficulty in securing the airway. A preoperative examination of the airway using indirect laryngoscopy may be required to understand the potential airway difficulty and formulate intubation plan. As these patients are prone to pathological fractures, careful positioning of limbs should be performed. Calvarial hyperostosis can result in difficulty during craniotomy. The proximity of the haemangioma to the superior sagittal sinus places the patient at risk of venous air embolism especially when head end of the operating table is raised to minimise blood loss and thereby reduce intracranial pressure. Significant blood loss is common and preparation for implementation of massive blood transfusion protocol may be required. Preoperative embolisation may reduce blood loss and transfusion requirement. However, in tumours with multiple feeding vessels, embolisation may result in cortical infarcts precluding use of this option, as was the case in our patient. Prolonged duration of surgery in these patients may predispose to deep venous thrombosis, necessitating the use of perioperative intermittent pneumatic compression device. These patients also have elevated D-dimer and iron-deficiency anaemia which can predispose to disseminated intravascular coagulopathy.

Adequate preoperative evaluation with cerebral angiography is necessary to identify and delineate the arterial feeders to the tumour and exclude intracranial arteriovenous malformations and aneurysms, the presence of which may necessitate special considerations during anaesthetic management unique to these conditions.

CONCLUSION

Multisystem involvement, the rarity of presentation, risk of massive blood loss and potential airway difficulty contributes to challenges in perioperative management in patients with KTS. This necessitates adequate preparation and planning, and meticulous conduct of anaesthesia and surgery in these patients to achieve favourable outcomes.

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.

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Conflicts of interest

There are no conflicts of interest.