Sympathetic Blocks for Raynaud’s Phenomena in Pediatric Rheumatological Disorders

Abstract

Objective

Sympathetic blocks are invaluable to prevent morbidity from Raynaud’s phenomenon (RP). RP may occur in children with rheumatological disorders and causes severe pain, discoloration of digits, gangrene, and auto-amputation. We describe the planning and execution of sympathectomy blocks in children with rheumatological disorders presenting with RP.

Methods

With upper-limb involvement, ultrasound-guided stellate ganglion block (USGB) was given with ropivacaine and clonidine. When all four limbs were involved, intrathecal block with bupivacaine and clonidine was also given.

Results

A total of 68 sympathectomy blocks were performed: 28 bilateral USGBs, two unilateral USGBs, and 10 intrathecal injections. Multiple interventions in a single day were frequently required. For safety, all USGBs were performed with an ultrasound with strict adherence to local anaesthetic volume was maintained, with periprocedure monitoring of 2–3 hours. All blocks were performed by an experienced specialist. All children reported immediate pain relief with prevention of major amputation.

Conclusion

With meticulous planning, monitoring, and precautions, sympathectomy of limbs in pediatric rheumatological disorders with RP can be safely undertaken. Bilateral stellate ganglion block with ultrasound is safe in children, and clonidine is a useful adjunct for vasodilation and prolongation of the effect of sympathectomies in children.

Raynaud’s Phenomenon, Children, Rheumatology Disorders, Sympathectomy, Intrathecal, Stellate Ganglion Block, Pediatric

Introduction

Raynaud’s phenomenon (RP) is the result of vasospasm secondary to various etiologies, with rheumatic disorders being one of them [1]. It causes immense pain and discoloration of peripheral toes and fingers and can lead to gangrene and the auto-amputation of digits.

RP has a prevalence of 18% in girls and 12% in boys. RP in children with rheumatological disorders can occur with irregular medical treatment, relapse or exacerbation of the disease condition, or late reporting of symptoms, as well as in the winter season. This is commonly treated with alteration in type of drugs or dosages of prescribed drugs. However, the maximal effect can take a few days, by which time RP can progress and cause various adverse sequelae [2]. Sympathetic blocks at this stage will be invaluable to halt the progress of RP until the action of medical drugs sets in and also will aid in medical management by increasing drug delivery to the vasodilated affected peripheral limbs [3].

However, sympathetic blocks for RP secondary to rheumatological disorders in children have not been previously described. The present article describes the planning and execution of sympathectomy blocks in children.

Methods

The retrospective data described here are from May 2018 through October 2021.

Six children on medical management for rheumatological illnesses (cutaneous polyarteritis nodosa, mixed connective tissue disorder, systemic sclerosis) who presented with RP were referred from the Pediatric Rheumatology Outpatient Department to the Pain Outpatient Department for further management (Table 1).

Table 1.

Referred children with rheumatological illness on medical management with RP

Case No.	Age/Sex	Diagnosis	Duration of Illness	Medical Management
1	13 years / M	Cutaneous polyarteritis arteritis nodosa	5 months	Steroids Antiplatelets Antihypertensives Pentoxyphylline
2	13 years / M	Cutaneous poly nodosa	2 months	Analgesics Antihypertensives DMARDs Antiplatelets Steroids Pentoxyphyline
3	13 years / F	Mixed connective tissue disease	7 years	Analgesics Antihypertensives DMARDs Antiplatelets Steroids Pentoxyphyline
4	10 years / M	Systemic sclerosis	2 years	DMARD Vasodilator Antihypertensive
5	14 years / F	Polyarteritis nodosa	5 years	Steroids Vasodilators Antihypertensives DMARDs
6	9 years / M	Systemic polyarteritis nodosa	5 years	Steroids Antiplatelets Antihypertensives Pentoxyphylline

M= male; F= female; DMARD= disease-modifying antirheumatic drug.

The ages of the children ranged from 9 to 14 years, and they had a duration of illness of 5 months to 7 years (Table 1). Bilateral upper limbs were involved in all six children, and lower limbs were also affected in three children. Baseline pain scores were assessed on a numerical rating scale (NRS) for pain or on the Wong-Baker FACES Pain Rating Scale. Discoloration and oxygen saturation (SpO₂) of all digits were noted. Sympathectomy blocks were given when the NRS pain score was >3. Written informed consent from the parents of all patients was obtained, and all children were admitted for the procedure. Basic biochemical investigations, including coagulation parameters, were assessed (already performed by the rheumatology department).

All blocks were undertaken in a monitored pain block area with standard aseptic precautions. Sedation with intravenous ketamine and/or midazolam and application of local anesthetic skin cream were done, if required.

Technique

For the affected upper extremity, ultrasound-guided stellate ganglion block (USGB), as previously described, was given through the use of a linear probe (5–12 MHz), with a 26G 2½-inch needle attached to a 10-cm extension line [4]. A drug solution of 2–4 mL of 0.25% ropivacaine with clonidine 0.2–0.3 µg/kg as an adjuvant was used. When all four extremities were involved, USGB was first given to the more affected upper extremity, along with intrathecal injection of 0.5% bupivacaine 0.5 mL with clonidine 0.3–0.5 µg/kg for the affected bilateral lower limbs. The child was monitored for 2–3 hours for any side effects, after which USGB was given on the other upper extremity, followed by monitoring of 2–3 hours.

Perioperative monitoring included vitals (heart rate and noninvasive blood pressure); complications like dysphagia, hoarseness, difficulty in breathing, sedation, bradycardia, and hypotension; pre- and postprocedure limb temperature (obtained via a surface temperature electrode or by manually comparing the temperatures of both limbs by physician); and SpO₂ of each affected digit before and after the procedure.

The NRS pain score was noted at 30 minutes after the procedure and then every 12 hours. Discoloration and SpO₂ of digits were noted every day. When the NRS pain score was >3, the appropriate block was repeated until the pain score decreased to 0–3, at which point no more blocks were given.

A total of 68 sympathectomy blocks were performed in six children, of which 28 were bilateral USGBs, two were unilateral USGBs, and 10 were intrathecal injections.

Results

All children reported a significantly reduced NRS pain score, from a baseline of 5–10 to 0–3 after the procedure. An increase in limb temperature and an improvement in SpO₂ in almost all digits were observed in all children as compared with preprocedure readings (Table 2). All children reported a halt in gangrene progression (Tables 2 and 3). In Cases 2, 3, and 6, at presentation, there was blackish discoloration of peripheral digits with an NRS pain score of >5. In these children, after the sympathectomies, there was immediate relief of pain; however, there was auto-amputation of a few digits at follow-up, with complete reversal of the discoloration of the feet and hands. Child 5 had bluish discoloration of all upper digits at presentation, and after the blocks, all digits were salvaged with five blocks (Tables 2 and 3).

Table 2.

Pre- and postprocedure parameters

	Pain Score		Discoloration/Gangrene		Temp	SpO2
Case No.	Before	After	Before	Recent Follow-Up	After	Before	After
1	9–10	2–3	Blue/black discoloration Wet gangrene—FL	No progress of gangrene Dry gangrene	↑	None/↓ Poor waveform	Improved waveform ↑ SpO2
2	9	0–1	Black discoloration Gangrene—BUL	No discoloration Loss of all digit tips	↑	None/↓ Poor waveform	Improved waveform ↑ SpO2
3	7–9	0–1	Black discoloration—FL	No discoloration Loss of digits left hand	↑	None/↓ Poor waveform	Improved waveform ↑ SpO2
4	6	0–1	Black discoloration—FL Gangrene	Complete reversal	↑	None/↓ Poor waveform	Improved waveform ↑ SpO2
5	5	0–1	Black discoloration Ulcer—BUL	Complete reversal	↑	None/↓ Poor waveform	Improved waveform ↑ SpO2
6	6	1–2	Blue/black discoloration—BUL	No discoloration Loss of digit tips right hand	↑	None or poor waveform	Improved waveform ↑ SpO2

SpO₂ = oxygen saturation; FL = four limbs; BUL = bilateral upper limbs.

Table 3.

Pre- and post-block pictures along with number of blocks given at variable intervals

	Before Injection	Time Interval of Blocks	Total Injections/BLUSGB (Total USGB)/ULUSGB/IT	After Injection
Case 1: 13 years / M Cutaneous PAN			22/8 (16)/0/6
Case 2: 13 years / M Cutaneous PAN			14/7 (14)/0/0
Case 3: 13  years  / F MCTD			10/3 (6)/1/3
Case 4: 10  years / M SS			9/4 (8)/0/1
Case 5: 14 years / F PAN			5/2 (4)/1/0
Case 6: 9  years / M Systemic PAN			8/4(8)/0/0

BLUSGB= bilateral ultrasound stellate ganglion block; USGB= ultrasound stellate ganglion block; ULUSGB= unilateral ultrasound stellate ganglion block; IT= intrathecal; Bupi= bupivacaine; PAN= polyarteritis nodosa; MCTD= mixed connective tissue disorder; SS= systemic sclerosis.

Procedures in all children were undertaken at different time intervals according to the presenting symptom of NRS >3 (Table 2). Case 1, Case 3, and Case 4 had blocks at two admissions with gaps of 7, 1, and 4 months, respectively (Table 3). Follow-up of all children was up to October 2021.

Transient hoarseness and dysphagia were reported in two USGBs. No other side effects were reported.

Discussion

Sympathetic overactivity in RP increases norepinephrine and other chemical mediators like Substance-P and interleukin -6, which causes painful edematous tissues, painful nerve endings, and vasoconstriction leading to severe pain and gangrene [2]. Sympathetic blocks are a known treatment to stop the vicious cycle of RP [3].

For the upper extremity, the stellate ganglion, located at the level of C7–T1, has to be blocked, and this has been shown to increase blood flow, decrease the resistance index of blood vessels, and increase the temperature of the upper extremity [5]. With the advent of USGB, reported complications of the landmark-guided technique, which were due to proximity to vital arteries and the lungs, have been reduced because of better visualization of vital vessels, soft tissue, needle trajectory, and injectate [6].

Isolated reports of stellate ganglion block in children have been found in the literature, of which none were reported for RP [7]. Bilateral stellate ganglion block has not been reported in children before and is seldom described even in adults, mainly because of concerns about catastrophic respiratory distress due to bilateral recurrent laryngeal nerve palsy, bilateral phrenic nerve palsy, and decreased sympathetic tone [8]. In the present series, bilateral stellate ganglion blocks were required in all referred children. For safety, all blocks were undertaken by an expert with ultrasound guidance, which resulted in minimal complications. Moreover, a minimum gap of 2 hours was kept between blocks to monitor for any side effects.

Sympathectomy of the lower extremity is typically performed by blocking lumbar sympathetic ganglion at the L2–L3 vertebral level under image guidance with a large volume of local anaesthetic solution on the affected side. Neuraxial blocks, like epidural and intrathecal blocks, are an alternative for sympathectomy and have shown an increase in skin capillary blood flow, wherein a single midline injection results in a bilateral effect [9]. Intrathecal injections were preferred in the present referred children because of bilateral lower involvement and the requirement of a small volume of local anesthetic (0.5 mL) for lower-extremity sympathectomy compared with the large volumes of local anesthetic solution required in lumbar sympathectomy and epidural block. This ensured a permissible local anesthetic volume for the child’s weight to minimize complications.

Clonidine was the preferred adjuvant in the present series because of its vasodilative properties, in addition to the prolongation of analgesia. Diligence is needed with regard to side effects like sedation, bradycardia, and hypotension [10].

General anesthesia is the ideal setting for performing a procedure on a child. However, this may not be possible in these children, as multiple blocks are often required. Thus, for execution of blocks without general anesthesia, the atmosphere should be made child friendly by the presence of the parents during the block and reassurance given to the child in calming easy words. For minimal discomfort, all blocks should be performed with smallest-gauge needles in a single prick by an experienced anesthetist. Sedation with drugs like ketamine, midazolam, or propofol may be used if required.

After sympathetic blocks, all children in the present series had an immediate reduction in pain and a gradual halt of discoloration and gangrene of digits with no major amputation. However, in longstanding disease and RP, fibrosis of vessels can occur, which compromises the blood circulation of the digits. In these patients, after sympathectomy, there is relief of pain, but auto-amputation might not be prevented because of the inability to achieve complete vasodilation due to vessel fibrosis. This was seen in some children (Cases 2, 3, and 6) in whom a few metacarpopharyngeal digits were lost. Although formal rehabilitation has not been possible in these children, they are managing their manual functions well.

A limitation of the present series is that no objective measurements for gains of sympathectomy blocks were conducted. However, the improvement in clinical parameters of all children points to a clear benefit of these interventions.

Thus, to conclude, timely reporting by the patient, timely referral by the pediatric rheumatologist, and timely intervention by the treating physician is a very vital aspect of management. Sympathetic blockade for RP in pediatric rheumatological disorders is an invaluable adjunct when performed with meticulous planning and precautions. For safety of multiple invasive blocks, all USGBs should be undertaken by an expert, the volume of cumulative required local anesthetic volume should be calculated, and vigilant monitoring should be undertaken in the periprocedure period.

Funding sources: No funding was received for this research.

Conflict of interest: There are no conflicts of interest for any author in relation to this article.