Limb Salvage in an Extensive, Complicated Vascular Lesion of the Arm in an Infant.

When a vascular lesion involving a limb poses a hazard to the life, treatment options are excision or amputation of the limb. Although excision can be hazardous, limb salvage with vascular control is the best treatment option. We report limb salvage in an infant with an extensive infected congenital hemangioma complicated with consumptive coagulopathy. Copyright:

INTRODUCTION

Extensive vascular lesions can endanger the life of a child by their potential to cause consumptive coagulopathy or cardiac failure. Early aggressive intervention is a must to prevent these potential complications. When they are resistant to medical management, surgical intervention is unavoidable. A conservative surgical approach is not just difficult but can be life-threatening as it can lead to uncontrolled bleeding. However, with good vascular control, limb salvage is still possible. We report a case of congenital hemangioma of the left upper limb, who presented with consumptive coagulopathy and sepsis. He was successfully managed with excision of the lesion.

CASE REPORT

A term male child of birth weight 3.2 kg was born with a swelling over the left arm [Figure 1a]. The antenatal scan had shown a soft-tissue mass measuring 10.8 cm × 6.8 cm × 5.4 cm extending from the left shoulder to the elbow.

Figure 1

The lesion involving the left upper limb (a). Magnetic resonance imaging showing an isointense lesion on T1 with hyperintensities representing hemorrhage (b), blood supply from axillary and brachial arteries (c), and intense postcontrast enhancement with necrotic areas within (d)

On examination, the swelling was not compressible, and there was no bruit heard. Doppler ultrasound of the lesion revealed a hypoechoic lesion with heterogeneous echotexture containing numerous cystic spaces and weak color Doppler signal suggestive of hemangioma. Magnetic resonance imaging showed a lobulated soft-tissue mass measuring 17 cm × 16 cm × 9.5 cm containing large areas of intense enhancement, nonenhancing necrotic and hemorrhagic areas, occupying the flexor as well as extensor compartment of the left arm supplied by the branches of axillary and brachial arteries [Figure 1b–d]. He was diagnosed as hemangioma, started on propranolol at 0.5 mg/kg/day, and gradually increased to 3 mg/kg/day.

As the swelling was increasing in size, prednisolone was started at 1 mg/kg/day and slowly escalated to 3 mg/kg/day. However, he presented at 2 months of age with severe anemia and consumptive coagulopathy refractory to medical treatment. Investigations revealed features of consumptive coagulopathy (hemoglobin: 7.3 g/dL; total leukocyte count: 2160/mm 3; platelet count: 76,000/mm 3; prothrombin time: 35.2 s; international normalized ratio: 3.2; activated partial thromboplastin time: >120 s control-31 s; fibrin degradation product: >800 ng/mL; D-dimer: 4.2 mg/L; fibrinogen: 0.6 g/L). This was accompanied by a sudden increase in size due to infection within the tumor, which got ulcerated and began to discharge a bloody purulent fluid. He was given red blood cell, cryoprecipitate, and fresh frozen plasma transfusions.

At that point, at 4 months of age, surgery became imperative as a lifesaving mode of treatment. The embolization was not considered as the lesion was infected, ulcerated, and was bleeding. After detailed discussion with parents, consent was obtained for excision with a probability of disarticulation of the limb.

Peroperatively, axillary vessels were identified and controlled. Skin flaps elevated, the tumour was gently dissected, and excised in toto. Muscles of flexor and extensor compartments were thinned out, and they were preserved to the extent possible [Figure 2a and b]. The humerus was thinned out. Radial nerve was passing through the tumor. Hence, it was divided and re-anastomosed primarily. Median and ulnar nerve areas were not specifically explored as the medial compartment of the arm was not involved. Postoperative period was uneventful [Figure 2c and d]. Histopathology revealed varying sizes of capillaries with focal papillary endothelial cell hyperplasia, hemosiderin-laden macrophages, and foci of extramedullary hematopoiesis consistent with congenital hemangioma. The infant's arm and hand movements were found to be preserved (finger flexion and wrist flexion are normal [Grade 5]; finger extension, thumb extension, and wrist extension are poor [Grade 0]) at 18-month follow-up. A long-term follow-up is required for final functional assessment.

Figure 2

An intraoperative image showing the axillary vessels under control by a vascular loop (a) and thinned out muscles overlying the lesion (b). Postoperative image taken on postoperative day-10 (c) and 10-month postoperative (d)

DISCUSSION

Most vascular anomalies today are treatable with a predictable outcome. However, there are times when their diagnosis and management can become difficult and complicated.

Vascular lesions are classified into vascular tumors which are characterized by endothelial cell hyperplasia and vascular malformations which show normal endothelial turnover.[1] More than 90% of these lesions can be diagnosed by a good history and thorough clinical examination.[1] Visceral, deep subcutaneous, and intramuscular lesions can be ambiguous warranting radiological investigations.

Congenital hemangioma is a vascular tumor which differs from infantile hemangioma by its nature of completely grown and being present at birth, no gender inequality, and rapid or no involution. This is a rarer entity opposed to infantile hemangioma.[2] Congenital hemangioma usually follows a benign course but rarely can present with complications such as infection, ulceration, bleeding, congestive heart failure, and consumptive coagulopathy which can mimic more severe Kasabach–Merritt phenomenon (seen only with kaposiform hemangioendothelioma and tufted angioma and not with any kind of hemangioma).[2] Ulceration and bleeding in infantile hemangioma are known to be benign, but it is little known about ulcerated or bleeding congenital hemangioma.[3] Massive hemorrhages are actually features of congenital hemangioma rather than infantile hemangioma as the former is found to have superficial large vessels.[3]

There is no standard method of the management of hemangioma in infants. Many of the hemangiomas do not require any treatment as the congenital hemangioma rarely progresses postnatally and the infantile hemangioma involutes over a period of years. The indications for intervention are complication, functional or esthetic.[4] Various options to treat these conditions are propranolol, steroids, systemic and local tranexamic acid, sirolimus, vincristine, chemoembolization with alcohol, laser therapy, and surgery.[3] When medical management fails, surgery is unavoidable. Although the surgery is the last resort, it is the most reliable and final resort. The surgical options include ligation of feeders, excision of the tumor, and rarely, disarticulation or amputation. When a lesion is extensive, amputation of the limb may be warranted to ensure the survival of the infant.

On literature review, we found 25 cases of vascular anomalies involving the limbs in infants who presented with complications. Four of them were tufted angioma while the rest had a hemangioma.[3567891011121314151617181920212223] Twelve (46%) infants had congestive heart failure, seven (27%) had ulceration with three (12%) presenting with hemorrhagic shock, and four (15%) had an infection [Table 1]. Consumption coagulopathy was seen in 15 (58%) cases of limb hemangioma in infancy who presented with complications. Two (8%) infants had intracranial bleed secondary to coagulopathy.[512] Propranolol was used in five patients, but all of them required other modes of therapy. Steroids were used in ten patients, but only two had success. Six were treated with embolization and three of them required surgery to control the disease. The laser was used in two cases who required surgery as final therapy. Other modes of therapy included local tranexamic acid to prevent bleeding, sclerotherapy, and sirolimus [Table 1]. A total of 14 (54%) patients underwent surgical excision. Two patients had recurrence postsurgery and both were managed by amputation. There were four mortalities which include an infant who presented late with hemorrhagic shock, and three were secondary to cardiac failure [Table 1]. The nature and timing of intervention should be individualized to attain the best outcome.

Table 1

Complicated vascular anomalies involving the limbs in infants

Authors	Diagnosis	Site	Manifestation	Nonsurgical therapy	Surgical therapy	Outcome
Stringel and Mercer[5]	Hemangioma	Arm	Congestive heart failure, consumptive coagulopathy, intraventricular bleed	Steroid, chemoembolization, blood products	-	Expired
Stringel and Mercer[5]	Hemangioma	Thigh	Congestive heart failure, consumptive coagulopathy	Blood products, intermittent pneumatic compression	-	Disease free
Weber et al.[6]	Hemangioma	Arm	Congestive heart failure	Steroid	-	Disease free
Miall-Allen et al.[7]	Hemangioma	Arm	Congestive heart failure, consumptive coagulopathy	-	Ligation of the feeder. Excision later	Disease free
Currie et al.[8]	Hemangioma	Arm	Congestive heart failure, consumptive coagulopathy	Steroid, blood products	Excision	Disease free
Nakada et al.[9]	Hemangioma	Arm	Ulceration, bleeding, congestive heart failure, consumptive coagulopathy	Steroid, blood products	Excision	Disease free
McCarthy et al.[10]	Hemangioma	Arm	Congestive heart failure, consumptive coagulopathy	Blood products	Excision	Disease free
Marler et al.[11]	Hemangioma	Thigh	Large foot, congestive heart failure	Steroid	Excision	Multiple recurrences ended up in amputation
Konez et al.[12]	Hemangioma	Shoulder	Congestive heart failure, consumptive coagulopathy, intracranial bleed	Steroid, chemoembolization, blood products	-	Expired
Agesta et al.[13]	Hemangioma	Thigh	Ulceration, bleeding	Steroid	Excision	Disease free
Baselga et al.[14]	Hemangioma	Arm	Congestive heart failure, consumptive coagulopathy	Steroid, chemoembolization	-	Disease free
Powell et al.[15]	Hemangioma	Knee	Ulceration, bleeding	Local tranexamic acid	-	Disease free
Vildy et al.[3]	Hemangioma	Knee	Ulceration, bleeding, hemorrhagic shock	Propranolol, chemoembolization	Excision	Disease free
Vildy et al.[3]	Hemangioma	Thigh	Ulceration, bleeding, hemorrhagic shock	Propranolol, chemoembolization	Excision	Disease free
Lu et al.[16]	Hemangioma	Forearm	Restricted wrist and elbow movements	Sclerotherapy	Excision	Multiple recurrences ended up in amputation
Salhi et al.[17]	Hemangioma	Arm	Ulceration, bleeding, hemorrhagic shock	Dressing, blood products	-	Expired
McCuaig et al.[18]	Hemangioma	Forearm	Infection, necrotizing fasciitis, consumptive coagulopathy	Dressing, blood products, antibiotics, propranolol, steroids	Fasciotomy	Disease free
Kumagai et al.[19]	Hemangioma	Thigh	Infection, cellulitis, consumptive coagulopathy	Sirolimus, antibiotics	-	Disease free
Lei et al.[20]	Tufted angioma	Thigh	Consumptive coagulopathy	Steroid	Excision	Disease free
Lei et al.[20]	Tufted angioma	Thigh	Consumptive coagulopathy	Laser	Excision	Disease free
Lei et al.[20]	Tufted angioma	Leg	Infection, consumptive coagulopathy	Laser, antibiotics	Excision	Disease free
Lei et al.[20]	Tufted angioma	Thigh	Consumptive coagulopathy	Chemoembolization	Excision	Disease free
Ren et al.[21]	Hemangioma	Arm	Congestive heart failure	Propranolol	Ligation of the feeder	Disease free
Knöpfel et al.[22]	Hemangioma	Arm	Postnatal rapid growth	-	-	Not known
Darouich et al.[23]	Hemangioma	Arm	Congestive heart failure, hydrops, lung hypoplasia	-	-	Expired
Our infant	Hemangioma	Arm	Infection, ulceration, consumptive coagulopathy	Propranolol, steroids, blood products	Excision	Disease free

The patient was thought to have a hemangioma as it was suggested by the radiological findings, postnatal rapid proliferation, consumptive coagulopathy, and normal adjacent bone. Hence, he was started on propranolol and then steroids. As he responded only partially, vincristine was planned. The rapid growth, infection of the lesion with sepsis, and consumptive coagulopathy warranted removal of the lesion as it posed a threat to the survival of the infant. Peroperatively, the axillary vessels were controlled; hence, the limb salvage was possible, and the risk of torrential hemorrhage was avoided. Histopathology was suggestive of congenital hemangioma.

CONCLUSION

A conservative limb salvage surgery is possible even in the case of an extensive vascular anomaly. A good vascular control is must to prevent life-threatening hemorrhage in a conservative surgery.

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.

We acknowledge the help of Dr. Vidya, Consultant Pathologist, for helping reach the correct histopathological diagnosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.