Neonatal compartment syndrome

Abstract

A term neonate was born with a grossly swollen and discoloured left hand and forearm. He was transferred from the local hospital to the plastic surgical unit, where a diagnosis of compartment syndrome was made and he underwent emergency forearm fasciotomies at six hours of age. Following serial debridements of necrotic tissue, he underwent split-thickness skin grafting of the resultant defects of his forearm, hand and digits. At the clinic follow-up appointment two months after the procedure, he was found to have developed severe flexion contractures despite regular outpatient hand therapy and splintage. He has had further reconstruction with contracture release, use of artificial dermal matrix, and K-wire fixation of the thumb and wrist. Despite this, the long term outcome is likely to be an arm with poor function. The key learning point from this case is that despite prompt transfer, diagnosis and appropriate surgical management, the outcome for neonatal compartment syndrome may still be poor.

Compartment syndrome is defined as an increase in pressure within a fasciocutaneous compartment leading to inadequate capillary perfusion pressure. It is a well recognised complication of closed limb fractures, crush injuries, prolonged extrinsic compression, electrical injuries and revascularisation after ischaemic insult. Upper limb contracture secondary to ischaemia was first noted by Volkmann in 1881¹ with paralytic contracture developing as a consequence of tightly applied bandages.

Although rare, neonatal compartment syndrome has been reported in the literature but it remains poorly understood in terms of aetiology and best practice. It has a broad spectrum of severity. The most severe cases have been noted to have limb length discrepancies at long-term follow-up,² which could be a consequence of ischaemic insult to the growth plates in the affected long bones. Risk factors include prematurity, oligohydramnios, transverse lie, maternal diabetes mellitus and preterm premature rupture of membranes.³ The severity and duration of tissue ischaemia is likely to correlate with the amount of tissue loss as well as the extent of contracture.

Compartment syndrome is often recognised following discolouration and swelling of the limb (indicative of underlying pathology), which develops into more severe skin changes. The differential diagnoses for this presentation should include cellulitis, necrotising fasciitis, vascular injury and amniotic band syndrome.⁴

As in adults, if there is suspicion of compartment syndrome, urgent decompressive fasciotomy should be performed to optimise outcome. The individual’s need for ongoing reconstruction and rehabilitation is variable. In the largest reported series of 24 patients, improved outcomes were noted following early intervention.⁵

Case history

A 3.37kg baby was born at 37+3 weeks’ gestation via spontaneous vaginal delivery with an Apgar score of 10 at five minutes. He had a well demarcated, grossly swollen, discoloured left forearm and hand (Fig 1). There was no difficulty in delivery. No amniotic bands were seen in the placenta, or on the arm or fingers.

Figure 1.

Well demarcated tense and discoloured swelling of the left forearm and hand

There were no antenatal abnormalities identified. The maternal risk factors were smoking, obesity with a booking body mass index of 41.9kg/m² and diet controlled gestational diabetes. The 20-week fetal anomaly scan was negative.

On examination, the patient’s left arm had superficial desquamation distal to the mid-forearm, with fixed staining of the skin from mid-forearm to the wrist crease volarly and to the metacarpophalangeal joints distally. The capillary refill time in the digits of the affected hand was <2 seconds. Doppler examination revealed present brachial and radial pulses; no Doppler signal was detected over the ulnar artery.

Blood tests on day 0 showed a white cell count of 104.6 x 10⁹/l and a platelet count of 75 x 10⁹/l. A blood film showed normal morphological white cells. The impression was that of compartment syndrome secondary to a compressive or thrombotic episode.

Given the diagnosis of compartment syndrome, the patient underwent urgent surgical decompression of all compartments of the hand and forearm (Figs 2–4) as a limb and potentially lifesaving intervention. The wounds were left open and the arm was dressed loosely with non-adhesive moist dressings.

Figure 2.

Preoperative photograph of the hand and forearm showing desquamation of the skin

Figure 3.

Preoperative photograph of the hand and forearm showing desquamation, and deep discolouration of the skin

Figure 4.

Intraoperative photograph demonstrating the ischaemic damage to the extensor muscle compartment

Over the course of the patient’s hospital stay, the insult to his arm declared itself. There was dry necrosis of the fingertips, with loss of length in the index finger and thumb. Owing to the severity of the initial insult, serial debridements (Figs 5 and 6) to reach viable skin led to a significant soft tissue defect. He underwent split-thickness skin grafting before he was discharged to the community with ongoing hand therapy to minimise contracture and maximise function.

Figure 5.

Postoperative view of the dorsum of the hand showing the split-thickness skin graft and the extent of debridement required

Figure 6.

Postoperative view of the volar aspect of the forearm showing the split-thickness skin graft, and the necrosis of the tip of the thumb and index finger

At the two-month follow-up visit, the patient was found to have severe contracture (Figs 7 and 8), worse in the flexor compartment, and loss of the tips of the index finger and thumb. He therefore underwent further surgical management with contracture release, use of an artificial dermal matrix as a skin substitute, and K-wire fixation of the thumb and wrist. The forearm flexor muscle bellies were fibrosed and contributing to the contracture, and so division of their tendons occurred at the same time to allow contracture release. Despite this, long-term limitation of function is the probable outcome. Even with prompt recognition, diagnosis and management, the severity of the contractures is likely to represent a prolonged antenatal insult to blood flow.

Figure 7.

Follow-up photograph at two months showing the severe flexor contracture

Figure 8.

Follow-up photograph at two months showing the severe flexor contracture and dislocation of the index finger

Discussion

Neonatal compartment syndrome is a recognised neonatal emergency but one that is poorly understood. Early fasciotomies are key to its management in order to maximise future limb function. Management advice should be sought as a matter of urgency from paediatric plastic/orthopaedic surgeons (depending on local expertise) in conjunction with neonatal/paediatric intensive care specialists.

Diagnosis is based largely on the clinical features of skin discolouration, poor movement and reduced perfusion. The clinical features of pain on passive stretch and sensory changes that can be useful in adults are not a useful guide in neonates, which can make the diagnosis more challenging.

Long-term follow-up is required because of the effect of the ischaemic insult, which can cause severe contracture and impair bone development.