Hemangiomas Flashcards
A 2-week-old premature male twin develops a red, rapidly enlarging lesion of the posterior trunk. Which of the following is the most important factor suggesting a diagnosis of infantile hemangioma?
A) Growth of the lesion
B) Location of the lesion
C) Patient gender
D) Premature birth
E) Twin gestation
The correct response is Option A.
Infantile hemangioma is the most common tumor of infancy. These lesions typically present between 2 weeks and 2 months of life and have a predictable clinical course, including rapid proliferation during the first 9 months of life (proliferative phase), followed by gradual involution until 3.5 years of age. Infantile hemangiomas most commonly occur in the skin, but can also occur in other sites, with the liver being the most common extracutaneous site. For cutaneous lesions, most are located in the head and neck, followed by the trunk, and then by the extremities. >95% of infantile hemangiomas are diagnosed by history and physical examination; <5% require imaging or biopsy for diagnosis. The onset of a red lesion at 2 weeks of age followed by rapid growth is pathognomonic for infantile hemangioma. The tumor affects both sexes, but is 3 to 4 times more common in females. Infantile hemangioma is also more frequent in premature infants and twins who are low birth weight. Some infantile hemangiomas can ulcerate during the proliferating phase.
2017
A 14-year-old boy is brought to the office for evaluation because of recurrent, severe nosebleeds that require visits to the emergency department. Dermatologic examination shows no skin discolorations. Neurologic examination shows no abnormalities. Family history includes frequent nosebleeds. A diagnosis of hereditary hemorrhagic telangiectasias (HHT) is suspected. A mutation of which of the following genes is most likely in this patient?
A) ENG
B) KRIT1
C) PIK3CA
D) PTEN
E) RASA1
The correct response is Option A.
In patients with hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, characteristic abnormal arteriovenous shunting is noted in the mucosae of the naso- and oropharynx, and pulmonary, GI/hepatic, and CNS systems. They are at risk for bleeding and anemia, and even stroke (CNS manifestation). Several genes have been associated with HHT, including the endoglin gene, ENG. ACVRL1 and SMAD4 may also be associated.
RASA1 mutation has been associated with capillary malformations with or without AVMs. Oval, macular port-wine staining on the skin is a common finding.
PTEN mutation has been associated with Bannayan-Riley-Ruvalcava syndrome, an autosomal dominant condition that presents with macrocephaly, genital lentiginosis (speckled penis), and GI polyps. A subset of patients may develop arteriovenous anomalies (arteriovenous malformations and arteriovenous fistulae).
KRIT1 mutation is an autosomal dominant condition associated with cavernous malformation in the brain (venous malformation, no fast-flow component). Affected patients are at risk for cerebral hemorrhage. Cutaneous manifestations are hyperkeratotic vascular malformations (slow-flow malformations).
CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies/scoliosis syndrome) syndrome can occur as a result of a mutation in PIK3CA. It is an overgrowth syndrome where vascular malformations can also occur. Patients with CLOVES syndrome should have a spinal MRI to screen for CNS AVM. They may also have other slow-flow vascular malformations.
2017
A 6-month-old infant is brought to the clinic for evaluation of an expanding vascular cutaneous lesion that partially obstructs the visual axis. The presence of which of the following histologic markers is most likely to confirm the diagnosis of infantile hemangioma in this patient?
A) Fibroblast growth factor receptor 3 (FGFR-3)
B) Glucose transporter 1 (GLUT-1)
C) Transforming growth factor beta (TGF-B)
D) Tumor necrosis factor 1 (TNF-1)
E) Vascular endothelial growth factor (VEGF)
The correct response is Option B.
GLUT-1 is a specific marker for infantile hemangioma and is often used by pathologists to confirm the diagnosis.
VEGF and TGF-B are incorrect, because while these markers may often be present in hemangiomas, they are not as specific as GLUT-1 in confirming the diagnosis of hemangioma.
Staining for FGFR-3 is not routinely used in confirming the diagnosis of hemangioma. FGFR-3 mutations are implicated in a number of syndromes, including Muenke syndrome, epidermal nevus, and achondroplasia.
Staining for TNF-1 is also not routinely used in confirming the diagnosis of hemangioma.
2017
A 7-month-old female infant is brought to the office because of a large mass on the left side of the neck. Her parents report that the mass has been present since birth and has remained consistent proportionately in size with the child’s growth. It has become firmer after a recent upper respiratory tract infection. Examination shows an active child in no distress. The mass measures 3 × 2 cm at the child’s left mandibular body and angle going down into the upper neck. It is easily compressible, has no pulse, and causes no pain. Which of the following is the most appropriate next step?
A) Admission of the infant and propranolol therapy
B) Angiography and injection of a sclerosing agent
C) CT angiography
D) Fine-needle aspirate biopsy
E) MRI
The correct response is Option E.
Differential diagnosis for congenital head and neck mass in a child includes malignancy, branchial remnants, and malformations. History and physical examination suggest a vascular malformation vs. malignancies. Due to the non-emergent nature of the mass, further imaging in the form of an MRI will help elucidate the nature of the mass, specifically to see whether it is a lymphatic malformation or a venous-lymphatic malformation. MRI will also assist in demonstrating which deeper tissues are involved, such as the parotid and the neck vessels. Invasive measures which are also treatment may eventually be required; however, at this time, there is no indication for urgent treatment in the form of angiography and either embolism or sclerosis. Propranolol currently does not have a role in treatment of vascular malformations, although it does have a role in hemangioma management. CT angiography can help define whether it is an arteriovenous malformation; however, clinical examination puts that further down the differential.
2017
A 2-month-old female infant is brought to the office for evaluation of a hemangioma of the right mid cheek that has been enlarging progressively since 3 weeks of age. Physical examination shows a 1-cm-diameter and 1-cm raised lesion. Which of the following is the most appropriate management at this time?
A) Intralesional steroid injection
B) Surgical resection
C) Systemic sirolimus therapy
D) Systemic steroid therapy
E) Observation
The correct response is Option E.
Hemangiomas are proliferative lesions that occur most often in the first weeks of life and then proceed into a proliferative phase. Involution and spontaneous regression occur for most lesions. The lesions frequently occur on the face, scalp, chest, and back but can occur on the extremities as well. The indications for treatment are related to symptoms or secondary effects such as: bleeding/ulceration, visual obstruction, and impingement on the mouth prohibiting proper feeding. Small lesions without symptoms should be observed and resolution is anticipated. However, if treatment is required, the options include intralesional or systemic steroids, systemic propranolol therapy, and surgical resection. If propranolol is used, it must be initiated cautiously with inpatient observation due to risks of hypoglycemia and bradycardia.
Sirolimus has been found to be helpful for complex lymphatic and vascular malformations. It is not indicated for hemangioma treatment.
2017
A 3-month-old male infant is brought to the office for evaluation of a 10 × 10-cm purple lesion of the right thigh that was present at birth. Medical history includes nosebleeds. On physical examination, bruising over the trunk is noted. Complete blood cell count shows thrombocytopenia. Which of the following is the most likely diagnosis?
A) Arteriovenous malformation
B) Capillary malformation
C) Congenital hemangioma
D) Infantile hemangioma
E) Kaposiform hemangioendothelioma
The correct response is Option E.
The most likely diagnosis is kaposiform hemangioendothelioma, which is a vascular tumor that is usually present at birth. The lesion can cause Kasabach-Merritt phenomenon: extreme thrombocytopenia (<25,000/mm3) from platelet trapping within the tumor which leads to spontaneous bleeding and bruising. Treatment is either vincristine or rapamycin. Infantile hemangioma, congenital hemangioma, capillary malformation, and arteriovenous malformation do not cause Kasabach-Merritt phenomenon or any other type of coagulopathy.
2016
A 12-year-old boy has had the lesion of the right index finger shown in the photograph since birth. The lesion has been growing in proportion to his overall growth. On physical examination, there is no thrill or bruit. Which of the following is the most likely diagnosis?
A) Arteriovenous malformation
B) Hemangioma
C) Kaposiform hemangioendothelioma
D) Lymphatic malformation
E) Port-wine stain
The correct response is Option D.
In 1982, Mulliken and Glowacki proposed a binary classification system for vascular anomalies based on pathologic features. This system divides vascular anomalies into two primary biological categories: 1) vasoproliferative or vascular neoplasms and 2) vascular malformations.
Vasoproliferative neoplasms, such as hemangioma and Kaposiform hemangioendothelioma, have increased endothelial cell turnover (i.e., they proliferate and undergo mitosis) because they are neoplasms. Vascular malformations do not have increased endothelial cell turnover. Instead, vascular malformations are structural abnormalities of the capillary, venous, lymphatic, and arterial systems that grow in proportion to the child.
The lesion shown in the photographs grew in proportion to the child; therefore, it is a malformation, not a vasoproliferative neoplasm. Given the absence of a bruit or thrill, it is unlikely to be an arteriovenous malformation; therefore, a slow-flow lesion such as a lymphatic malformation is the most likely diagnosis.
Although a port-wine stain is a type of venous malformation, it is characterized by its very superficial location, which causes the skin to be discolored red (hence the name). Such findings are not present in the photographs shown.
2016
A 4-month-old male infant is brought to the office because of a rapidly enlarging mass in the eyebrow region. The patient’s mother says she first noticed the lesion at 1 month of age and that it was not present at birth. A photograph is shown. Treatment with administration of propranolol is planned. Which of the following adverse effects is most likely in this patient?
A) Adrenal failure
B) Decrease in height
C) Hypertension
D) Hypoglycemia
E) Spastic diplegia
The correct response is Option D.
First-line treatment of a small problematic infantile hemangioma is intralesional corticosteroid (triamcinolone 3 mg/kg). If the tumor is too large to inject, then oral corticosteroid (prednisolone 3 mg/kg daily) or propranolol is initiated. Interferon is no longer used to treat infantile hemangioma because it may cause spastic diplegia when administered to infants.
The common side effects of propranolol include gastrointestinal effects (vomiting, diarrhea, and constipation), rash, fatigue, and hypersomnia. Severe side effects may include bradycardia, hypotension, chest pain, shortness of breath, bleeding, bronchospasm, and glaucoma. In pediatric patients, propranolol has been associated with hypoglycemia that may occur without the characteristic jitteriness.
Proliferating hemangiomas should not be treated with pulsed-dye laser therapy because accelerated involution does not occur, and patients are at risk for ulceration, pain, bleeding, hypopigmentation, and scarring. The pulsed-dye laser is indicated, however, to treat residual telangiectasias after the tumor has involuted.
Corticosteroid complications (e.g., adverse neurodevelopment, aseptic necrosis of the femoral head, diabetes mellitus, osteoporosis, adrenal insufficiency, cataracts, glaucoma, infection, gastric irritation) have not been observed in patients treated with corticosteroid for infantile hemangioma. Although increased blood pressure has been observed, the clinical significance of this is unclear, and no adverse effects have been reported. Twenty percent of infants develop a temporary cushingoid appearance that disappears during tapering of the medication. Approximately one third of infants exhibit decreased gain in height but return to their growth curve by 24 months of age.
2015
A 3-year-old boy is brought to the office because of recurrent swelling, discoloration, and occasional discomfort of the right forearm. Discoloration and swelling have been present since birth, but these features have become more pronounced with growth and are exacerbated by limb dependency. A photograph is shown. Which of the following is the most appropriate initial treatment in this patient?
A) Interferon alpha therapy
B) Propranolol therapy
C) Reassurance that the condition will resolve with time
D) Sclerotherapy
E) Systemic corticosteroid therapy
The correct response is Option D.
The lesion shown is a venous malformation. Unlike infantile hemangioma, these lesions are present at birth and grow proportionately with the child, and therefore, cannot be expected to resolve over time. Venous malformations can become more symptomatic with growth and swelling or symptoms are exacerbated by dependency. In addition, rapid swelling and pain can result from phlebothrombosis (clotting) in the anomalous venous channels. The most effective treatment for symptomatic lesions is sclerotherapy. Operative excision is generally reserved for focal lesions or those with significant functional effects; because the lesions involve critical tissues and are poorly circumscribed, operative excision is rarely curative. The other options presented have been used to treat infantile hemangioma, but have not demonstrated effectiveness for venous, arteriovenous, or lymphatic malformations.
2015
A 7-year-old boy is evaluated because of capillary malformations in the right leg and thigh, which have been present since birth. Physical examination shows venous varicosities over the anterior leg and medial thigh. Girth of the affected lower limb is increased compared with the unaffected limb. Duplex imaging discloses no sign of arteriovenous fistulae. This patient is at greatest risk for which of the following conditions?
A) Chondrosarcoma
B) Disseminated intravascular coagulation
C) High-output cardiac failure
D) Limb-length discrepancy
E) Pathologic fracture
The correct response is Option D.
The presence of capillary malformations, varicosities, and limb hypertrophy paired with the absence of arteriovenous (AV) fistulae is classic for Klippel-Trénaunay syndrome. Up to 67% of patients with Klippel-Trénaunay syndrome will exhibit limb-length discrepancy, most commonly with the affected limb being hypertrophic.
Pathologic fractures may occur through enchondromas found in Maffucci syndrome. Since the lesions have been present since birth and the child is young, presentation is less consistent with Maffucci syndrome, which presents in adolescence in roughly 80% of patients. These enchondromas also hold a 15 to 30% risk for malignant transformation to chondrosarcoma.
High-output cardiac failure is a feature of vascular malformations that include AV fistulae; the classic example is Parkes-Weber syndrome.
Disseminated intravascular coagulation can result in life-threatening thrombocytopenia. Referred to as Kasabach-Merritt syndrome, this finding is more common in abnormalities such as kaposiform hemangioendothelioma.
2015
An otherwise healthy 4-year-old boy is brought to the office for evaluation of a large scalp hemangioma. A photograph is shown. The family has requested removal of the hemangioma before he enters school. Which of the following is the most appropriate management in this patient?
A) Deferral of surgery for 2 years
B) Excision and skin grafting
C) Single-stage excision and closure
D) Staged excision and closure
E) Tissue expansion
The correct response is Option C.
Although the hemangioma shown is sizable, judicious mobilization of the scalp and galeal scoring allow the resultant defect to be easily closed without resorting to delayed rotational flaps, tissue expanders, or skin grafting. Single-staged excision and linear closure is the simplest, most predictable manner of closing small to intermediate-sized scalp defects such as the one in the patient described. The maximum defect using this technique is not well defined, but at least one series has demonstrated successful primary closure of defects in infants of up to 7 cm without using the other alternatives listed or tissue expansion. Delaying the resection for another 2 years might be productive in a younger child, but further involution is unlikely in a 4-year-old. Staged excision is possible, but unnecessary for this lesion; moreover, repair along the edge of the friable hemangioma can lead to wound problems.
A 22-year-old woman comes to the office because of oral bleeding and a 20-year history of a radiologically defined arteriovenous malformation. Embolization and resection of the tumor are planned. Which of the following factors is most likely to be associated with an increased risk for recurrence?
A) Enlargement of the malformation
B) Patient age
C) Patient gender
D) Quiescence of the malformation
E) Ulceration
The correct response is Option E.
According to the Children’s Hospital of Boston’s experience with arteriovenous malformations, the Schobinger stage of the lesion is most important when considering the individual’s recurrence risk for these lesions. In their experience, lesion recurrence was more associated with advanced Schobinger staging than with quiescent lesions, whether these were treated with embolization alone or embolization followed by resection.
Enlargement of the malformation describes a Schobinger Stage I lesion, which has the lowest risk for recurrence in the Boston Children’s series. The presence of these lesions, while concerning to parents, typically has a recurrence risk of 80% with embolization alone and 21% with embolization followed by resection.
Patient sex, age, and lesion location did not correlate with lesion recurrence in the Boston Children’s series.
Quiescence of the malformation represents a Schobinger Stage III lesion, which is the highest Schobinger stage description among the options listed. This stage lesion has a recurrence risk of 99% with embolization alone and 81% with embolization followed by resection.
2015
A 12-year-old girl has a 7 × 7-cm venous malformation of the thigh that is slowly enlarging and causing pain. The lesion involves the skin, subcutaneous tissue, and muscle. Which of the following is the most appropriate first step in management?
A) Corticosteroid therapy
B) Embolization of the lesion
C) Propranolol therapy
D) Resection of the lesion
E) Sclerotherapy
The correct response is Option E.
The most appropriate first step in management is sclerotherapy. First-line intervention for a large symptomatic venous malformation is sclerotherapy. Sclerotherapy is the injection of an inflammatory substance into a lesion which causes endothelial damage, fibrosis, and shrinkage of the malformation. Sclerotherapy is more effective and less morbid than resection. Propranolol and corticosteroids are treatment options for a problematic infantile hemangioma, but have no efficacy for vascular malformations. Embolization is first-line intervention for an arteriovenous malformation, and is not a treatment option for venous malformation. Resection is second-line therapy for a large problematic venous malformation. Extirpation can cause significant morbidity (i.e., bleeding, nerve injury, infection, wound breakdown). In addition, excision leaves a cutaneous scar and recurrence is common because a venous malformation can rarely be completely removed.
2014
An 8-month-old male infant is evaluated for a lip mass. A photograph is shown. Treatment with propranolol is initiated. Which of the following adverse effects is most important to monitor?
A) Drooling
B) Hypertension
C) Hypoglycemia
D) Lethargy
E) Tachycardia
The correct response is Option C.
Vascular anomalies are a common source of pediatric morbidity, potentially resulting in cosmetic and functional abnormalities. Two main categories of lesions are hemangiomas and vascular malformations. Vascular malformations are generally named after the vessel types that are involved and are further subdivided into low-flow and high-flow lesions. Low-flow lesions include capillary, lymphatic, venous, and mixed lesions. High-flow lesions include arteriovenous malformations. Both high- and low-flow vascular malformations are almost always present at birth and either grow commensurately with the child or slowly enlarge over a period of years.
Treatment is guided by the degree of functional impairment, and many require surgical therapy.
Hemangiomas are rarely present at birth. They tend to appear between 2 and 8 weeks of life and grow rapidly. These lesions comprise of rapidly proliferating endothelial cells and follow a predictable clinical course. They usually undergo an aggressive proliferative phase that lasts several months before reaching a plateau phase, when they grow very little. Finally, at about 1 year of life, hemangiomas begin a process of spontaneous involution which may last for up to 4 years. Completion of involution does not mean complete involution, and many hemangiomas may leave cosmetically unacceptable residua. Hemangiomas found in inconspicuous areas can be observed. Those noted in cosmetically or functionally sensitive areas require more aggressive treatment. Treatment options have included topical, intralesional, and systemic corticosteroids, laser treatment, interferon-alfa, and surgical resection. However, each of these treatments carries marked risks and still does not provide consistent, reliable success.
In June 2008, a French group reported rapid resolution of hemangiomas in children treated with propranolol for pediatric cardiomyopathy. This serendipitous finding led to a landmark paper and has resulted in a paradigm shift in the clinical care of these patients. Many groups have now confirmed their findings with very promising results. The use of propranolol for hemangiomas remains off-label, but there are centers of excellence with institutional review board protocols in place to further study its clinical effects. Recommended doses vary but frequently start at 0.5 mg/kg/day divided three times daily and slowly titrated over 1 to 2 weeks to 2 mg/kg/day. Most centers recommend obtaining pretreatment electrocardiography (ECG); if normal, then therapy can be initiated on an outpatient basis. If the ECG is abnormal, then pretreatment cardiology evaluation is warranted. Dose titration, blood pressure, and heart rate are checked regularly. The most common adverse effect is lethargy. Other adverse effects include hypoglycemia, hypotension, and bradycardia. All children drool, and this has not been reported as a complication of propranolol administration. Hypoglycemia can be a potentially life-threatening complication that can also cause seizures. Parents are counseled to give food with medication. Many centers regularly check blood glucose.
An image of a child with upper lip hemangioma treated with propranolol for 14 months is shown.
A male newborn has a large macrocystic lymphatic malformation of the neck. Which of the following is the most appropriate first step in management?
A) Embolization of the lesion
B) Prednisolone therapy
C) Propranolol therapy
D) Resection of the lesion
E) Sclerotherapy
The correct response is Option E.
The most appropriate first step in management is sclerotherapy. Lymphatic malformation is a type of vascular anomaly that results from aberrant formation of lymphatic vessels. Lymphatic malformation most commonly affects the neck and axilla. There are two major types of lymphatic malformations: macrocystic and microcystic. Macrocystic lesions have cysts large enough to be cannulated by a needle and treated with sclerotherapy. Microcystic lesions have cysts that are too small for treatment with sclerotherapy. First-line management of macrocystic lymphatic malformations is sclerotherapy, which is the injection of an inflammatory substance into the lesion that causes scarring of the cyst walls together and shrinkage of the malformation. The most commonly used sclerosants are doxycycline, sodium tetradecyl sulfate, and ethanol.
Prednisolone and propranolol are drugs used to treat problematic proliferating infantile hemangioma, and have no efficacy for vascular malformations. Embolization is used to treat arteriovenous malformations and involves delivering a substance through an artery to occlude blood flow.
Resection is second-line therapy for a problematic macrocystic lymphatic malformation. Extirpation can cause marked morbidity (i.e., bleeding, nerve injury, infection, wound breakdown). In addition, excision leaves a cutaneous scar and recurrence is likely because a lymphatic malformation can rarely be completely removed. Resection is considered if a lesion remains symptomatic following sclerotherapy, or for microcystic lymphatic malformations that cannot be sclerosed.
2014
An otherwise healthy 68-year-old man comes to the office because of an enlarging mass over the right ear for the past 30 years. A photograph is shown. He had no prior treatment of the mass. MRI confirms the diagnosis of arteriovenous malformation. In the past 3 months, he had bleeding from the mass requiring hospital admission and blood transfusions. Bleeding is controlled after prolonged direct pressure. Which of the following is the most appropriate management for this lesion?
A) Corticosteroid therapy
B) Laser therapy
C) Resection
D) Sclerotherapy
E) Observation
The correct response is Option C.
The most appropriate treatment for this lesion is radical resection, with the goal of eradicating the lesion. Although the lesion is prone to recurrence, this approach maximizes the chances for a cure. This patient has recurrent bleeding and is at risk for death due to exsanguination. Observation is not acceptable in this otherwise healthy 68-year-old. Laser therapy is futile as it will only affect superficial dermal structures, and this lesion clearly involves large vessels and deeper tissues. Corticosteroid therapy is appropriate for management of infantile hemangioma but has no role in the treatment of arteriovenous malformations. Sclerotherapy is not indicated in this patient.
2014
A 7-month-old male infant is referred because of the lesion shown in the photograph. Ophthalmologic examination shows mild astigmatism, for which he was being treated with corrective lenses. No amblyopia is noted. Which of the following is the most appropriate next step in management?
A) Administration of propranolol 2 mg/kg/day in divided doses
B) Magnetic resonance angiography of the brain
C) Pulsed-dye laser (595 nm) therapy
D) Surgical resection
E) Observation only
The correct response is Option A.
The patient described has focal hemangioma. Medical therapy is the first line of treatment in this case when there are minimal ophthalmologic findings. There is a subset of periorbital hemangiomas for which surgical resection is indicated: well-localized hemangioma amenable to resection, greater than 2 diopters of astigmatism, visual obstruction, or nonresponsive to medical therapy. In a study by Arneja and Mulliken, the best ophthalmologic improvement occurred when the hemangioma was resected at less than age 3 months.
Laser therapy can treat discoloration only and will not decrease the bulk of the lesion.
Surgical debulking is indicated if the hemangioma does not clinically respond (softer, lightening in color, no improvement in/worsening of astigmatism).
2014
A newborn female presents with a lesion on the occipital scalp/posterior neck. A photograph and x-ray are shown. MRI has been performed. It is most appropriate to order which of the following studies before formulating a treatment plan?
A) Brain MRI to rule out structural anomalies
B) Doppler ultrasonography to assess flow of the lesion
C) Echocardiography to assess cardiac function
D) Incisional biopsy for genetic analysis
E) Lymphatic mapping with isosulfan blue to identify the sentinel lymph node
The correct response is Option C.
This patient has a rapidly involuting congenital hemangioma (RICH). These are often large and fast-flow and can cause high-output cardiac failure. Therefore, it is important to assess cardiac function to ascertain whether this patient needs cardiac support (eg, digoxin). While Doppler ultrasonography may have been indicated as a first-line imaging study, the MRI and magnetic resonance angiography demonstrated the extent of the lesion and flow; Doppler ultrasonography will not give additional information at this time. Incisional biopsy may be indicated if there are concerns about the diagnosis. However, performing a biopsy to obtain tissues for genetic analysis is not indicated, as the genetic information will not give information to direct treatment. Brain MRI to rule out structural anomalies is indicated when a patient is suspected of having posterior fossa brain malformations, hemangioma, arterial lesions, cardiac abnormalities, and eye abnormalities (PHACE) syndrome, a large segmental infantile hemangioma in the face. Lymphatic mapping for sentinel node is used for head and neck malignancies and has no role here.
In this case, the patient did develop mild cardiac failure. This lesion was embolized one day before she underwent resection of this lesion.
2021
A 2-month-old female infant presents with a raised, nonpulsatile, vascular lesion of the lower lip that has worsened progressively since it first appeared soon after birth. A photograph is shown. Her parents deny history of trauma, but since monitoring, report increased tenderness, bleeding, and difficulty with feeds. Which of the following is the most appropriate first step in treatment?
A) Beta-adrenergic blocker administration
B) Embolization
C) Laser therapy
D) Surgical excision
E) Topical corticosteroid administration
The correct response is Option A.
Beta-adrenergic blockers, including oral propranolol (Hemangeol), would be the best first-line treatment due to the size and location of the hemangioma. Such medications can act to limit growth, decrease bulk, and prevent complications of the hemangioma in a time-sensitive manner.
Embolization is often employed in treatment of arteriovenous and lymphatic malformations. It is typically the treatment of choice for complications of larger vessels, rather than the capillaries comprising a hemangioma.
Laser therapy is beneficial in the treatment of port-wine stains and telangiectasia post involution of hemangiomas. The hemangioma described in the vignette is clearly in the proliferative phase, making it too large, thick, and deep to respond well to laser therapy alone.
Surgical excision is not a viable option in this case due to the extensive size and location of the hemangioma. The treatment is not emergent, since the patient described remains stable, and more conservative options should be exhausted before resorting to resection.
Topical corticosteroid is not preferred in this case due to the large size and location of the hemangioma. Topicals should not be applied to the intraoral mucosa, and corticosteroids would be too slow-acting to improve significant symptoms such as bleeding and poor feeding.
2022
