Large Vascular Malformations Research Articles

Primer on Embolic Agents and Sclerosants for the Treatment of Vascular Malformations.

Vascular malformations (VMs) are a heterogeneous group of arterial, venous, capillary, and/or lymphatic networks that typically are present at birth and grow with the patient. Signs and symptoms associated with VM range from absent to severe, with a broad spectrum of pain, cosmetic disfigurement, and local tissue destruction. Treatment options for these malformations extend from observation to surgical reconstruction. Each treatment modality has a role in the management of vascular anomalies, but also has specific challenges. Observation is not always sufficient, medical therapies may only be available for a subset of patients and are ultimately not curative, and surgical excision may itself be disfiguring or incomplete/not definitive. Sclerotherapy and/or embolotherapy have become a cornerstone of treatment for many types of VMs. These techniques are applicable to a wide range of VMs, are less invasive, have a shorter recovery period, and are repeatable. Although some VMs may not be amenable to percutaneous or endovascular treatment given their location, and multiple sessions may be required for select large and complex VMs, these minimally invasive treatments are often first line and may be definitive in treating VMs. Various agents may be used in sclerotherapy and embolotherapy, each with their own risks and benefits. Although there is a paucity of Level 1 evidence supporting the superiority of one agent over another, longstanding case series and expert experience exist, informing our knowledge of the safety and efficacy of sclerotherapy and embolotherapy. A brief overview of the most used sclerotherapy and embolotherapy agents and their uses is provided.

Unravelling Anaesthetic Challenges in Neonate with Cavernous Haemangioma: A Case Report

Haemangiomas are developmental vascular abnormalities characterised by hyperplasia of blood vessels, usually veins and capillaries. Haemangiomas show a higher prevalence in females. More than 50% of lesions are found in the head and neck region, with a particular preponderance over the face, lips, buccal mucosa, tongue, palate, and trunk. The aetiology can be neoplastic or reactive, influenced by factors such as hormones, infections, and trauma. Large lingual vascular malformations may present with obstructive symptoms, including difficulty in breathing, chewing, swallowing, and speech, which can lead to delays in linguistic development and compromised airway function. Haemangiomas are more susceptible to trauma, which can result in bleeding and further compromise the airway. Typically, this kind of presentation is managed conservatively until obstructive symptoms arise, at which point surgical removal or local site steroid instillation is considered. Here, a case of a 28-day-old child with a cavernous haemangioma of the tongue who was scheduled for excision and instillation of Kenacort injection at the base of the tongue has been reported. Due to the child’s small age and the presence of a lingual mass, the use of intravenous anaesthetic agents was precluded. In this case, because of the non-availability of a paediatric fibreoptic bronchoscope, blind nasal intubation was considered for securing the airway. Managing a compromised airway is a challenging situation, even for an experienced anaesthesiologist, in a routine operating room set-up. However, maintaining spontaneous ventilation is a crucial element during general anaesthesia.

Management of “Large” Vascular Malformation: An Institutional Review

Background: Large vascular malformations (VM) are complex lesions anatomically, functionally or both. The criteria to define “Large” vascular malformations were unclear. This study aims to retrospectively identify Large VM based on clinical features and discussed about the method we adopted in management. Methods: This is retrospective study of analyzing all patients of vascular malformations treated by us since 2003. The criteria to categorize large vascular malformations were outlined after analyzing the anatomical area of involvement, functional disturbances by these lesions, operability of the lesions, morbidity associated with resection, and the requirement of reconstruction. All cases were managed by a multi-disciplinary team. Various methods utilized to control the bleeding varied from simple wound packing to cardiopulmonary bypass (CPB) assistance. The resection was either curative or palliative. Complete excision in the form of radical resection was carried out in all possible cases intended with curative aim. Vascularized tissue transfer was done to reduce the morbidity following surgical resection. Results: Out of 268 patients, 26 patients fulfilled the criteria for large vascular malformations. The predominant site of involvement is head and neck. 80% had more than one indication for surgery. Majority of the cases 54% were arterio-venous malformations (AVM) followed by 31% of capillary venous malformations (CVM). The commonest indication for surgery is bleeding in 73% followed by cosmetic disfigurement in 61.5%. Compressive wound packing with delayed wound closure and preoperative embolization were the commonly employed methods for bleeding control. CPB assisted resection was carried out in 5 cases. On analysing the recurrence, 7.7% had in complete excision, 36% in incomplete excision and 12% in cases of vascularized tissue transfer. Conclusion: The clinical criteria outlined in our study will be useful in identifying “Large” vascular malformations. Compression wound packing with delayed closure plays a major role in bleeding control immediately after resection. CPB assistance for aiding resection can be considered in selected cases. Complete resection in the form of radical excision will reduce the recurrence. Palliative resection provides symptom free disease when complete resection is not viable. The tissue transfer for the wound cover controls the residual disease in cases of incomplete resection.

34471 Case report: Verrucous venous malformation with thrombocytopenia in a male neonate

Verrucous venous malformations (VVMs) are rare congenital vascular malformations that present at birth or in adulthood as a linear vascular plaque typically unilateral on the lower extremity with reactive skin changes. VVMs are frequently misdiagnosed due to their clinical resemblance to other vascular malformations. Gold standard for diagnosis is histopathologic evaluation of a deep tissue section. Recently, a missense mutation MAP3K3 has been linked to VVM. Large vascular malformations are associated with local intravascular coagulopathy (LIC) that carry an increased thromboembolic risk, especially within low-flow venous malformations.

Use of Intercostal Nerve Block for Chest Wall Pain in a Patient with CLOVES Syndrome

Congenital lipomatous overgrowth, vascular malformations, epidermal nevi and scoliosis/skeletal/spinal anomalies(CLOVES) syndrome is an extremely rare overgrowth syndrome characterized by complex vascular malformations. Management requires aninterdisciplinary approach including debulking operations for tissue overgrowth, embolization therapy for vascular malformations and management of chronic pain due tocongenital and recurrent vascular overgrowth and from scar tissue from surgical interventions. Here, we present a 35-year-old femalewith complex medical history due to CLOVES syndrome, with large vascular malformations on her chest, statuspost debulking/embolization previously and now with continued chronic nociceptive and neuropathic pain, largely due to the recurrent nature of vascular malformations, butnow finding some relief with fluoroscopy-guided intercostal nerve blocks which she never experienced before.

A Study of Iron Status in Pediatric Patients with large complex Vascular Malfomations

Abstract Background Complex vascular malformations involving the gastrointestinal tract (GIT) are expected to develop iron deficiency.However, the haemoglobin and iron status of vascular anomalies unrelated to GIT were not addressed. Our aim was to study the frequency of anemia and iron status among pediatric patients with large/complex vascular malformations without evident bleeding and to correlate the findings with the type, site, severity and extension of vascular malformationand treatment with sirolimus. Patients and method A case control study of participants with large complex vascular malformationregistered at Ain Shams University vascular anomalies clinic during the period from June 2019 to June 2020.They were compared to 20 age, sex and socioeconomic standard matched healthy controls. Clinical and laboratory studies for iron status were performed. Results fifty percentof patients were anemic versus 65% of the control group (p value = 0.33).As regards iron status of patients, 35% has combined iron deficiency versus 40% of controls, 20% has functional iron deficiency versus 20% of controls, 5% has absolute iron deficiency versus 10% of controls (p value = 0.90). There was no significant difference between patients receivingsirolimus and who are not as regards anemia and iron status (p value = 0.36, 0.84, respectively). Conclusion The frequency of anemia and it s iron related classification are comparable in children with large vascular malformations with no bleeding manifestations to normal children.

Proteus Syndrome: A Rare Case in An Adult Ward.

Proteus syndrome is an extremely rare disorder that manifests as an asymmetric, disproportionate overgrowth of any connective tissue.The diagnosis can be difficult because the phenotypes are variable.It is important to keep in mind the importance of anticoagulation because patients are at risk of developing deep vein thrombosis and pulmonary embolism.

Safe and Efficient Removal of Massive Head and Neck Vascular Malformations Using Vessel Sealing Energy Devices

ABSTRACT Large vascular malformations (VMs) pose several difficult clinical challenges to the plastic surgeon. Traditionally surgical dissection was labor-intensive and required a significant amount of time and technique. The advent of more advanced vessel sealing devices has resulted in easier vascular and lymphatic control during resection surgery. The authors present 2 cases of large VMs resected using newer energy devices. First, an infant who was born with a large neck AVM that was acutely bleeding requiring immediate control. The authors utilized the Impact Ligasure device to perform a subtotal resection, stabilize the patient, and returned for definitive resection in the future. In the second case, an adult male with a large complex tongue/lip AVM, presented for elective resection. He underwent staged sclerotherapy, followed by resection using the Harmonic Scalpel. The use of vessel sealing devices allows for a safe and efficient resection for a previously difficult surgery.

S2788 When VCE Is Not Enough: It Was the Blebs!

INTRODUCTION: Although not common, accounting 5-10% of gastrointestinal (GI) bleeding, small bowel bleeding can be clinically significant and challenging to locate and treat. If bleeding is not identified on upper endoscopy (EGD) or colonoscopy, a second look can further exclude upper and lower sources. Push enteroscopy, video capsule endoscopy (VCE), and intraoperative endoscopy are next line diagnostic methods. We describe a challenging case of small bowel bleeding. CASE DESCRIPTION/METHODS: A 79 year-old male with atrial fibrillation, on Rivaroxaban, presented with one week of hematochezia. An EGD and colonoscopy did not reveal a source of bleeding. A VCE found numerous vascular blebs throughout the small intestine and fresh blood distally. Based on appearance, blue rubber bleb nevus syndrome was the initial thought, despite it being unlikely given he had no prior GI bleed. Multiple endoscopies, including push enteroscopy, repeat colonoscopy, and bidirectional balloon enteroscopy revealed scattered non-bleeding vascular blebs but no active bleeding source (Figure 1). CT angiography reported a subcentimeter jejunal lesion without active bleeding. Repeat VCE demonstrated active bleeding and submucosal nodules in the jejunum. He then had a laparotomy-assisted endoscopy that found numerous large vascular malformations (Figure 2) concentrated in the mid-jejunum. Full thickness biopsy was consistent with vascular malformation/hemangioma. Given his age and extent of small bowel involvement, octreotide was given. Due to persistent overt bleeding he ultimately underwent resection of the involved small bowel (approximately 100cm). He had no further episodes of GI bleeding on apixaban and was discharged home. DISCUSSION: Vascular malformations, or hemangiomas, are rare lesions that can cause significant GI bleeding. They generally involve the submucosa to mucosa, can involve the small bowel, and can be localized via CT enterography. They can be seen on endoscopy as soft, dark red to purple masses or blebs; biopsy can cause uncontrollable bleeding and surgical resection is often the treatment of choice. This case was unique given the size and involvement of the small intestine. Our patient's findings initially suggested blue rubber bleb nevus syndrome, however a lack of prior history of GI bleeding made this diagnosis unlikely and the management more challenging. This case highlighted the successful role of surgical intervention in a patient with extensive small bowel involvement of vascular malformations.Figure 1.: Vascular malformations in the jejunum found on anterograde balloon enteroscopy.Figure 2.: Numerous vascular malformations in the jejunum found during laparotomy-assisted endoscopy.

Intracranial hemorrhage in neonates: A review of etiologies, patterns and predicted clinical outcomes.

Intracranial hemorrhage (ICH) in neonates often results in devastating neurodevelopmental outcomes as the neonatal period is a critical window for brain development. The neurodevelopmental outcomes in neonates with ICH are determined by the maturity of the brain, the location and extent of the hemorrhage, the specific underlying etiology and the presence of other concomitant disorders. Neonatal ICH may result from various inherited and acquired disorders. We classify the etiologies of neonatal ICH into eight main categories: (1) Hemorrhagic stroke including large focal hematoma, (2) Prematurity-related hemorrhage, (3) Bleeding diathesis, (4) Genetic causes, (5) Infection, (6) Trauma-related hemorrhage, (7) Tumor-related hemorrhage and (8) Vascular malformations. Illustrative cases showing various imaging patterns that can be helpful to predict clinical outcomes will be highlighted. Potential mimics of ICH in the neonatal period are also reviewed.

Combined Surgery and Intraoperative Sclerotherapy for Vascular Malformations of the Head/Neck

Vascular malformations (VMs) of the head and neck can lead to aesthetic problems as well as cranial nerve damage, airway compromise, and vision loss. Large VMs are typically managed surgically, with sclerotherapy or embolization performed in the perioperative period to decrease the risk of excessive blood loss and minimize the size of the VM. However, this initial treatment is frequently insufficient leading to excessive blood loss intraoperatively, poorer margin visualization for the surgeon, and decreased likelihood of complete resection. As a result, resections of large VMs are often performed in a multistage approach. This article introduces a new hybrid approach for the management of head and neck VMs entailing the use of an endovascular operating room where a neuroendovascular surgeon performs embolization or sclerotherapy intraoperatively as needed in conjunction with surgical excision. Three patients with large VMs in the facial region underwent successful use of the hybrid approach. The hybrid approach improved visualization, leading to complete resection in 1 patient and nearly complete resections (70% and 90%) in the other patients. The technique also helped minimize blood loss because only the youngest patient (23 months old) required a blood transfusion. Implications of these findings include the transition from a multistaged approach for large VMs to a single-stage approach. In addition, decreases in blood loss may allow for the development and use of minimal access techniques, leading to a decrease in visible scarring for patients. We suggest the consideration of the hybrid approach for large head and neck VMs.

4:10 PM Abstract No. 388 Ethylene vinyl alcohol copolymer (Onyx) for treatment of large venous vascular malformations: long-term results

To describe the long-term results and advantages of ethylene vinyl alcohol polymer for treatment of venous vascular malformations. Since 2005, we have used Ethylene vinyl alcohol copolymer (Onyx 18 & 34) in the treatment of 62 patients with large, low-flow venous vascular malformations. There were 34 females and 28 males with ages ranging from 7–59 years (mean 25 years). Fifty-one of the malformations involved the extremities and 11 involved the chest, abdominal wall or pelvis. All patients were evaluated with MRI/MRA pretreatment. Onyx was delivered by direct injection with ultrasound and fluoroscopic guidance under tourniquet control. In a few cases Onyx was also delivered via a transarterial micro catheter positioned in large feeding vessels. Sodium tetradecyl (3%) foam was used also in conjunction with Onyx in six patients where there were associated smaller foci of malformation. Histologic examination of tissues from patients whose embolized lesions were subsequently explanted was performed. Ethylene vinyl alcohol copolymer (Onyx) was found to be useful for occlusion of large venous channels in venous malformations. Unlike sclerosing agents, its visibility allowed direct observation of filling of the venous malformation during injection. When used alone, there was minimal post procedure discomfort. There were two complications: one extrusion of Onyx from a lesion on the sole of the foot, which healed without sequelae and one instance of embolization of a small Onyx fragment to the lung, also without sequelae. On H & E stains, the Onyx was noted to be distributed in the intraluminal spaces with associated mild foreign body type giant cell reaction. Permanency of the copolymer was demonstrated on long-term imaging follow-up. Ethylene vinyl alcohol copolymer (Onyx) is a safe, durable agent for treatment of venous vascular malformations. It is of particular value in malformations with large venous channels. Unlike standard sclerosing agents, Onyx behaves as a filler, and is not dependent upon generation of an inflammatory reaction for occlusion. Its opacity allows visualization of filled channels. It can be used alone or in conjunction with sclerosing agents.

Harmonic devices: The workhorse for surgical resection of vascular malformations

Management of vascular malformations is multimodal with documented role of surgical resection in specific facets of this condition. Surgical resection of these lesions is technically challenging owing to diffuse and relatively ill-defined extent with involvement of multiple tissue planes limitation of access and excessive intra-operative bleeding. An observational study was conducted in 24 cases taken up for surgical resection of vascular malformations. The cases were divided into two groups based on the hemostasis technique used: Group A: Harmonic shears (n = 12) (Ethicon Inc. Somerville, New Jersey, United States). Group B: Electrosurgery (monopolar/bipolar) with standard knot tying (n = 12). We conclude that use of harmonic scalpel in surgical resection causes less parallel tissue damage, secures haemostasis promptly, does not impede vision and aids surgical dissection thereby significantly reducing the operative time and improving the surgical outcome, typically in large vascular malformations of head and neck region.

Multidisciplinary Approach in the Management of Head and Neck Vascular Malformations

Purpose: Arteriovenous malformations (AVM) of the head and neck are rare anomalies, but often present with significant haemorrhage or cosmetic defects. The purpose of this study is to determine the effectiveness of embolization of each type of vascular malformation. Methods: A retrospective review was performed between January 2009 to June 2015 on 36 patients who were diagnosed vascular malformations in the head and neck regions and were referred to our department for transarterial or percutaneous embolization before the surgical approach. Results: All 26 AVM were embolized with a transarterial approach: 18 with Onyx, 8 with a combination of PVA and coils. All patients with an AVM had a single endovascular approach. All 10 venous-lymphatic malformations were treated with a percutaneous sclerotherapy with ethanol injection. Complete healing was obtained in 30 patients (83%). In 6 (17%) there was a recurrence with necessity of a retreatment, 4 at 6 months, 2 at 12 months. Conclusion: Transarterial embolization and percutaneous scleroterapy is a safe treatment for artero-venous malformations before the surgical treatment. A multidisciplinary approach is fundamental to reduce the bleeding risks, the time of the surgical intervention and favouring a lesser demolitive approach particularly in large vascular malformations. Surgical complications occurred in 6 patients (17%); all of them developed infections of the surgical wounds; 3 of these developed necrosis of the skin flap used for the reconstruction with subsequent dehiscence. No other complications including sepsis, hemorrhages, cranial nerve palsies or neuropathies occurred.

Oral Dokuları Etkileyen Arteriovenöz Vasküler Malformasyon Vakalarında Dental Tedavi Yaklaşımı

Large arteriovenous vascular malformations (AVM) of the jaws are relatively rare and potentially life-threatening lesions. When these lesions are not suspected, extraction of teeth or other surgery procedures can be fatal in dentistry. Gingival bleeding seems to be a symptom common to most documented cases. Despite their benign histology, deep lesions can produce serious systemic signs and symptoms due to extensive arteriovenous shunting and soft tissue hypertrophy. Mandibular AVM shows a wide variety of signs and symptoms, such as dental mobility, otalgia, secondary pain due to thrombosis, facial asymmetry, and cosmetic distress. A clinical case report was presented in this study. A 24-year-old girl, seen for gingival bleeding during oral hygiene and facial asymmetry, was found to have a high-flow AVM located around the right mandible. In dental and clinical examination, although she had good oral hygiene, common gingival bleeding was observed. At the mandibular rigth molar region, non-hemorrhagic, gingival granulomatous swelling was noted. Scaling and root planning and polishing was performed to the teeth carefully. The patient was called maintenance phases. During 2 year, no recurrence of the lesion has been seen. Dentists must always consider the possibility and be able to recognize the clinical signs of AVMs in order to propose proper treatment. Because, AVMs of the maxillofacial region give rise to dental emergencies and may cause disfigurement, morbidity, even death.

Abstract: Successful Treatment of a Complex Vascular Malformation with Sirolimus and Surgical Resection

INTRODUCTION: Large complex vascular malformations are rare condition associated to major body deformities and disfiguration, pain, recurrent bleeding, infections, high-output heart failure and, ultimately, death may occur. 1 Typically, management represents a challenge since it may include a wide variety of options such as embolization, laser therapy, sclerotherapy and surgical resection but may lead to significant morbidity. 2 In extreme situations, lesions considered otherwise untreatable have been reported to respond to a new alternative treatment: mammalian target of rapamycin inhibitors (mTORI) 1,3,4.

Clinical analysis of microsurgical treatment of giant intracranial arteriovenous malformations

To explore the clinical features and microsurgical treatment strategies of giant intracranial arteriovenous malformations (AVM). A total of 15 cases of giant intracranial AVM treated with microsurgery were analyzed retrospectively. According to the Spetzler-Martin grade, there were 4 cases of grade Ⅳ, 11 cases of grade Ⅴ. Pre-operative endovascular embolizations were carried out in 3 AVMs. All the included patients were confirmed as giant intracranial AVM by magnetic resonance angiography (MRA), digital subtraction angiography (DSA), 3-dimensional CT angiography (3D-CTA) before surgery. All the resected tissues were sent for pathological examination, and the diagnoses were confirmed as AVM. The average operation time of the 15 patients was 10.3 ±6.9 hours. After 1-3 months, all the patients were rechecked by DSA, the large vascular malformations in 12 cases were completely resected, 3 cases had a small amount of residual further treated with gamma knife treatment. Magnetic resonance imaging (MRI) and MRA examination indicated that the residual AVM was occluded after 12 months. Patients were followed up at 6, 12 and 24 months after operation, and assessed by Glasgow outcome scale (GOS) score: 13 cases good, 1 cases mild disability, 1 cases severe disability; the good rate was 86.6%, with no dead case. Sufficiently preoperative preparation, appropriate operative methods and skills are necessary to treat giant intracranial arteriovenous malformation.

Multidisciplinary management of giant genital tract venous malformations during pregnancy: case report and review of the literature

Abstract Malformation affecting the genital tract is a rare condition. Enlargement of the mass and specific complications may occur following hemodynamic changes associated with pregnancy. A 25-year-old pregnant primigravida affected by an extended vulvar and thigh venous malformation with localised intravascular consumptive coagulopathy was referred to our hospital. Her antenatal care was uneventful until term. She underwent an elective caesarean section because the venous malformation location precluded vaginal birth. At 3 days postpartum, she suffered life-threatening bleeding in the abdominal cavity with disseminated intravascular coagulation requiring multiple blood and platelets transfusions as well as transarterial embolisation. The patient recovered and was discharged after 20 days. Specific counselling and management of pregnant patients with large vascular malformations is essential as it is associated with increased complications rate such as peripartal thrombosis and haemorrhages. These patients should be referred to tertiary centres and should be managed by a multidisciplinary team including an obstetrician, anaesthesiologist, haematologist, interventional radiologist and plastic surgeon.

Management of Severe Craniofacial Vascular Malformation Operated Under Bypass

Cardiopulmonary bypass (CBP) and circulatory arrest as an assist in the surgical excision of a severe facial vascular malformation were first described by Mulliken et al in 1979. Later on, its use had expanded for resection of intracranial vascular malformations. However, to date, there have not been any published series of these procedures being used in the resection of craniofacial vascular malformations. We sought to review the first 10 surgical procedures performed at McGill University Health Centre for large vascular malformations resection using hypothermic CBP with or without circulatory arrest. All consecutive patients at the McGill University Health Centre who had a craniofacial vascular malformation resected with the aid of CBP were reviewed. A comparison of the classic midline sternotomy with cardiac arrest to percutaneous femoral bypass with hypothermic "low flow" was performed. Charts were reviewed for the operative intervention including bypass parameters and short- and long-term complications of the procedure. Cardiopulmonary bypass was used in 9 patients for 10 surgical procedures for the resection of a variety of craniofacial vascular malformations from 1987 to 2001. All lesions had sclerotherapy and embolization of the feeding vessels 72 to 96 hours preoperatively. The average age of our patients was 21 ± 13.4 years (2-37 years). Procedures were conducted via either an open bypass or a closed femoral approach. There were no mortalities. There were 2 major cardiac intraoperative complications and 1 major postoperative complication, which were managed with no sequelae. The average length of postoperative hospital stay was 10 days. All patients went on to full recovery. The blood transfusions varied from 10 U to 0 U for our last patient. The assistance and adjunct of CBP are a useful procedure in the resection of very large vascular malformations, in selected cases. There were no major long-term complications in this series. With the evolution of our approach, the use of complete circulatory arrest was not required in the majority of cases, and an adequate resection was usually possible with the low-flow state alone as we developed this technique with more experience through the process.

Pediatric Headache: A Review

1. Heidi K. Blume, MD, MPH 1. Division of Pediatric Neurology, Seattle Children’s Hospital and Research Institute, Seattle, WA. * Abbreviations: CSF: : cerebrospinal fluid ICH: : intracranial hemorrhage ICP: : intracranial pressure IIH: : idiopathic intracranial hypertension NDPH: : new daily persistent headache NSAID: : nonsteroidal anti-inflammatory drug SVT: : sinus venous thrombosis TAC: : trigeminal autonomic cephalalgia Headaches are common in children; while most are caused by a benign problem or primary headache disorder, headaches can be a sign of a serious underlying condition. Pediatricians must be aware of the most recent recommendations for evaluating and managing headaches. After reading this article, readers should be able to: 1. Understand the evaluation of a child who has headache. 2. Recognize the diagnostic criteria for pediatric migraine. 3. Recognize “red flags” for elevated intracranial pressure or other underlying conditions in the child who has headache. 4. Discuss treatment strategies for migraine, tension, and chronic headache disorders. Headaches are common in children and adolescents and are a frequent chief complaint in office and emergency department visits. The vast majority of childhood headaches are due to a primary headache disorder, such as migraine, or an acute, relatively benign process, such as viral infection. However, clinicians also need to consider other causes of headaches in children. Even when headaches are benign, they may cause significant dysfunction for the child and family and must be managed appropriately to minimize disability and optimize function. In this review, we discuss the epidemiology of childhood headache, evaluation of the child who has headaches, when to consider secondary headache syndromes, and the diagnosis and management of primary headache disorders such as migraine and tension-type headaches. Acute and chronic headaches are relatively common in children and adolescents, although estimates of the precise prevalence of headache and migraine vary widely. Depending on the study definition of headache, population involved, and time periods studied, 17% to 90% of children report headaches, with an overall prevalence of 58% reporting some form of headache in the past year. (1 …