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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
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Year : 2020  |  Volume : 25  |  Issue : 2  |  Page : 121-125

Limb salvage in an extensive, complicated vascular lesion of the arm in an infant

1 Department of Paediatric Surgery, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India
2 Department of Paediatric Surgery, Manipal Hospital, Bengaluru, Karnataka, India
3 Department of Plastic Surgery, Manipal Hospital, Bengaluru, Karnataka, India

Date of Submission26-Jan-2019
Date of Decision26-Apr-2019
Date of Acceptance25-May-2019
Date of Web Publication28-Jan-2020

Correspondence Address:
Dr. Chittur Narendra Radhakrishnan
Department of Paediatric Surgery, Manipal Hospital, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_16_19

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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.

Keywords: Consumptive coagulopathy, hemangioma, limb salvage

How to cite this article:
Radhakrishna V, Radhakrishnan CN, Kabilan HK, Chandrappa AB, Vasudevan S. Limb salvage in an extensive, complicated vascular lesion of the arm in an infant. J Indian Assoc Pediatr Surg 2020;25:121-5

How to cite this URL:
Radhakrishna V, Radhakrishnan CN, Kabilan HK, Chandrappa AB, Vasudevan S. Limb salvage in an extensive, complicated vascular lesion of the arm in an infant. J Indian Assoc Pediatr Surg [serial online] 2020 [cited 2022 Aug 19];25:121-5. Available from: https://www.jiaps.com/text.asp?2020/25/2/121/276934

   Introduction Top

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 Top

A term male child of birth weight 3.2 kg was born with a swelling over the left arm [Figure 1]a. 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)

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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 1]b, [Figure 1]c, [Figure 1]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 2]a and [Figure 2]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 2]c and [Figure 2]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)

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   Discussion Top

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.[3],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22],[23] 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.[5],[12] 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

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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 Top

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 theater staff, OPD staff, ward, and ICU staff in managing this child.

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

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Kulungowski M, Fishman SJ. Vascular anomalies. In: Coran AG, Adzick NS, Krummel TM, Laberge JM, Shamberger RC, Caldamone AA, editors. Paediatric Surgery. 7th ed. Philadelphia: Elsevier; 2012. p. 1613-30.  Back to cited text no. 1
Merrow AC, Gupta A, Patel MN, Adams DM. 2014 revised classification of vascular lesions from the international society for the study of vascular anomalies: Radiologic-pathologic update. Radiographics 2016;36:1494-516.  Back to cited text no. 2
Vildy S, Macher J, Abasq-Thomas C, Le Rouzic-Dartoy C, Brunelle F, Hamel-Teillac D, et al. Life-threatening hemorrhaging in neonatal ulcerated congenital hemangioma: Two case reports. JAMA Dermatol 2015;151:422-5.  Back to cited text no. 3
Umpton J 3rd, Marler JJ. Vascular anomalies of the upper extremity. In: Mathes SJ, Hentz VR, editors. Plastic Surgery. 2nd ed. Philadelphia: Elsevier; 2006. p. 369-416.  Back to cited text no. 4
Stringel G, Mercer S. Giant hemangioma in the newborn and infant. Complications and management. Clin Pediatr (Phila) 1984;23:498-502.  Back to cited text no. 5
Weber TR, West KW, Cohen M, Grosfeld JL. Massive hemangioma in infants: Therapeutic considerations. J Vasc Surg 1984;1:423-8.  Back to cited text no. 6
Miall-Allen VM, Morgan B, Cooper P, Shinebourne EA. Peripheral arteriovenous fistula as a cause of neonatal cardiac failure. Int J Cardiol 1986;10:177-9.  Back to cited text no. 7
Currie BG, Schell D, Bowring AC. Giant hemangioma of the arm associated with cardiac failure and the Kasabach-Merritt syndrome in a neonate. J Pediatr Surg 1991;26:734-7.  Back to cited text no. 8
Nakada K, Kawada T, Fujioka T, Kitagawa H, Enami T, Kuwabara M, et al. Hemangioma of the upper arm associated with massive hemorrhage in a neonate. Surg Today 1993;23:273-6.  Back to cited text no. 9
McCarthy RE, Lytle JO, Van Devanter S. The use of total circulatory arrest in the surgery of giant hemangioma and Klippel-Trenaunay syndrome in neonates. Clin Orthop Relat Res 1993;289:237-42.  Back to cited text no. 10
Marler JJ, Fishman SJ, Upton J, Burrows PE, Paltiel HJ, Jennings RW, et al. Prenatal diagnosis of vascular anomalies. J Pediatr Surg 2002;37:318-26.  Back to cited text no. 11
Konez O, Burrows PE, Mulliken JB, Fishman SJ, Kozakewich HP. Angiographic features of rapidly involuting congenital hemangioma (RICH). Pediatr Radiol 2003;33:15-9.  Back to cited text no. 12
Agesta N, Boralevi F, Sarlangue J, Vergnes P, Grenier N, Léauté-Labrèze C. Life-threatening haemorrhage as a complication of a congenital haemangioma. Acta Paediatr 2003;92:1216-8.  Back to cited text no. 13
Baselga E, Cordisco MR, Garzon M, Lee MT, Alomar A, Blei F. Rapidly involuting congenital haemangioma associated with transient thrombocytopenia and coagulopathy: A case series. Br J Dermatol 2008;158:1363-70.  Back to cited text no. 14
Powell J, Blouin MM, David M, Dubois J. Bleeding in congenital hemangiomas: Crusting as a clinical predictive sign and usefulness of tranexamic acid. Pediatr Dermatol 2012;29:182-5.  Back to cited text no. 15
Lu H, Chen Q, Shen H, Ye G. A rare atypical rapidly involuting congenital hemangioma combined with vascular malformation in the upper limb. World J Surg Oncol 2016;14:229.  Back to cited text no. 16
Salhi A, Drali W, Cheddani N, Tounsi T, Djeridane A, Aitbenamar A, et al. Fatal hemorrhage from a rapidly involuting congenital hemangioma in an infant. Proceedings of the 22nd International Workshop of the International Society for the Study of Vascular Anomalies Conference. Amsterdam, Netherlands; 29 May, 1 June, 2018. Available from: http://www. issva.org/UserFiles/file/ISSVA2018-Program-AbstractBook.pdf. [Last accessed on 2018 Dec 30].  Back to cited text no. 17
McCuaig C, David M, Bortoluzzi P, Laberge L, Rousseau E, Hatami A, et al. Controversy RICH or KHE? What constitutes a true Kasabach Merritt syndrome? Necrotizing fasciitis in vascular anomalies: Seeking other centers experience, prevention? Proceedings of the 22nd International Workshop of the International Society for the Study of Vascular Anomalies Conference. Amsterdam, Netherlands; 29 May, 1 June, 2018. Available from: http://www. issva.org/UserFiles/file/ISSVA2018-Program-AbstractBook.pdf. [Last accessed on 2018 Dec 30].  Back to cited text no. 18
Kumagai C, Ozeki M, Nozawa A, Kakuda H, Fukao T. Efficacy of sirolimus in an infant with Kasabach-Merritt phenomenon. Pediatr Int 2018;60:887-9.  Back to cited text no. 19
Lei HZ, Sun B, Ma YC, Li MM, Wang LF, Jiang SW, et al. Retrospective study on the outcomes of infantile tufted angioma complicated by Kasabach-Merritt phenomenon. Clin Chim Acta 2018;486:199-204.  Back to cited text no. 20
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Knöpfel N, Wälchli R, Luchsinger I, Theiler M, Weibel L, Schwieger-Briel A. Congenital hemangioma exhibiting postnatal growth. Pediatr Dermatol 2019. doi: 10.1111/pde.13813. [Epub ahead of print].  Back to cited text no. 22
Darouich S, Bellamine H, Mkaouar L, Ayachi A, Mourali M. Congenital large cutaneous hemangioma with arteriovenous and arterioarterial malformations: A novel association. Fetal Pediatr Pathol 2019;38:85-90.  Back to cited text no. 23


  [Figure 1], [Figure 2]

  [Table 1]


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