General Data. Case discussion. Present Illness. Chief Complaint. Past History. A 16 year old boy. Growth: Chart No. : Admission date: 9/12

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1 General Data Case discussion A 16 year old boy. Growth: BW: 41.3kg(<3rd percentile) BH: 187cm(>97th percentile) Chart No. : Admission date: 9/ Chief Complaint Sudden onset headache and bilateral neck pain for 1 day Present Illness This 16 year old boy has been diagnosed as Marfan syndrome at childhood due to long extremities. He had received CV OPD and echocardiography follow up. Mitral valve prolapse with trivial mitral regurgitation was noted. Besides, progressive dilated aortic root was noted in recent years. Surgical intervention was suggested by CV surgeon. He was on treatment of Carvedilol 6.25mg/tab 0.5PC BID. 3 4 Present Illness Sudden onset headache, dizziness, mild abdominal discomfort, four limbs weakness, and bilateral neck pain developed after getting up this morning. Past History # Admission history: 2010/06/ /06/24: scoliosis for operation # Operation history: 2010/06/18 posterior fusion and fixation T2 T12 with Moss Miami 5 6

2 Personal History # Birth history G 2 P 2, Gestational age: fullterm via spontaneous delivery BBW: 3100 gram # Newborn screening: normal # Growth BW: 41.3kg(<3rd percentile) BH: 187cm(>97th percentile) # Development: as milestone # Current medication: Carvedilol 6.25mg/tab 0.5PC BID Physical Examination T:36.7/ P:72/min R:18/min BP:100/57 mmhg General Appearance: acute ill looking Appetite: decreased; Activity: decreased Consciousness: clear, E4 V5 M6 HEENT: Sclerae: anicterus Conjunctivae: not injected Eardrum: intact and not injected, no cerumen impaction Nose: no nasal flaring, not boggy nasal turbinates Throat: not injected; no post nasal dripping Tonsil: not injected, no enlargement, no exudate Oral cavity: no ulcer, no vesicle, no thrush Lips: no cyanosis NECK: supple, no lymphadenopathy CHEST: Breath pattern: mild tachypnea, bilateral symmetric expansion No use of accessory muscle, no contraction of sternocleidomastoid muscle No suprasternal retraction, no subcostal retraction Breathing sound: bilateral clear and symmetric breathing sound, no crackle, no wheezing, no stridor, no rhonchi, no bronchial sound HEART: Heart sound: regular heart beat, Gr 2/VI systolic murmur over left sternal border ABDOMEN: Tactile: soft and flat; mild tenderness over periumbilical area; no rebounding pain; no muscle guarding Percussion: dullness Bowel sound: normoactive No palpable mass Hepatosplenomegaly: no BACK: No knocking pain over flank area EXTREMITIES: Freely movable No pitting edema Peripheral and femoral pulse: symmetric SKIN: No rash; no petechiae or ecchymosis; no vesicle; no desquamation Intact without wound 7 8 Blood Pressure Laboratory Findings 97/51 100/57 94/61 103/ CXR 9/12 6/17 Impression 1. Marfan syndrome 2. Mitral valve prolapse with mitral regurgitation 3. Ascending aortic aneurysm 4. Suspect aneurysm rupture or aortic dissection 11 12

3 Consult CVS 2D echo Course and Treatment Ao (mm): 28 LA (mm): 20 LVED (mm): 47 LVES (mm): 25 EF: 77 % Course and Treatment Persistent and severe chest pain and abdomen pain Arrange chest CT No pericardial effusion. Vmax 0.69 m/s for main pulmonary artery. Mitral valve prolapse with trivial mitral regurgitation. Mild tricuspid regurgitation with Vmax 2.94 M/S. Aortic annulus : 28 mm. Aortic root : 51 mm. No definite ASD, VSD and PDA is detected. Vmax 1.06 m/s for ascending aorta with mild aortic regurgitation. Vmax 0.94 m/s for descending aorta. Normal left sided aortic arch CT of Chest 9/12 Presence of radiolucency intimal flap involving ascending and aortic arch and descending thoracic aorta and expanding downward to suprarenal level, consistent with type A aortic dissection. C/W Marfan syndrome with ascending aortic aneurysm, and type A aortic dissection. Operation 9/13 Operative finding: Type A Aortic dissection involving ascending aorta, arch and descending thoracic arota.the tearing site was located 3cm above sinotubular junction with prolapse of aortic valve. No pericardial adhesion. Operative procedure: Resection of AsAo. Aortic valve, interpostion with 26mm woven graft and 25mm St. Jude mechanical valve, reimplantation RCA and LCA, exclusion method Headache 17

4 Warning S/S The abrupt onset of an extremely painful headache (thunderclap, worst headache of my life ) may represent an intracranial hemorrhage (such as from an arteriovenous malformation or aneurysm). A headache that wakens a child from sleep should raise suspicion about a possible brain tumor Tumor Chronic progressive headache (increasing in freqency and severity over time) is a common presenting symptom among children with brain tumors. Over 99 percent of children in the multicenter report and all of the children in the ED series had at least one other symptom or sign (such as nausea/ vomiting, visiual disturbance, ataxia, or abnormal eye movements) Ref: Headache 2000;40:200, J Neuro oncol 1991;10:31 Objectives Neck Complaints in the Pediatric Emergency Department To describe the spectrum of pathologies responsible for neck ailments in a primary care pediatric emergency setting and evaluate their outcome. PEDIATR EMER CARE 2009;25:

5 Methods Methods Children s Hospital of Lausanne Lausanne: a city of 160,000 inhabitants The ED sees approximately 30,000 patients a year, of which 22,000 are triaged to the medical side and 8000 are triaged to the surgical side of the ED. Duration: from October 2004 to September Methods Inclusion criteria: patients aged 16 years or younger with neck complaints Exclusion criteria: toxic appearing children with obvious signs of meningitis were excluded Record: child s demographics, history of fever, previous upper airway illness, or trauma 4 Groups 1. Traumatic: when a fall or direct impact on the neck was recorded, or when a sudden abnormal neck movement initiated the symptoms. 2. Infectious: when the history, physical examination, and/or laboratory studies confirmed a bacterial (a) or viral (b) infection involving the neck Groups 3. Postural: when it was of spontaneous and sudden appearance and when no history of trauma or infection could be identified. 4. Miscellaneous: when congenital, general medical illnesses, or neoplasic lesions were diagnosed. Results 29 30

6 General data Traumatic group Study period: 12 months 170 ED patients with neck complaints Frequency: 1/169 Medical team: 38%, surgical team: 62% Female: male = 61:109 Mean age: 9.05 years, median age: 9 years, range: 7 weeks to 16 years Infectious group Imaging study CT MRI 35 36

7 Treatment NSAIDs: 141 children (83%) Cervical collars: 75 children (44%) Antibiotics: 5 patients (3%) Oral muscle relaxants: 10 patients (5.9%) Surgery: 2 patients (1.2%) Literature reivew Discussion A wide variety of etiologies for neck pain and torticollis in children: depending on the source of information and origin of cases Orthopedic studies review: traumatic or postural torticollis. Pediatric literature: congenital or infectious neck ailments. Our study aimed to describe the most frequent causes of neck complaints that present to a pediatric ED Cervical spine radiographs Traumatic: n = 105, 62% Because even minor trauma is known to produce torticollis, one should always take a detailed history to determine the exact time of onset and mechanism that may have resulted in this complaint. However, a negative history for injury does not rule out trauma because it is sometimes difficult to obtain a clear history in children, especially if the event was not witnessed or if the trauma was considered to be insignificant. Radiological study were performed in 57 (54.3%) children with a history of trauma Chart review alone did not permit the identification of which specific criteria were used to determine which children would receive cervical spine radiographs

8 Criteria for radiographic evaluation? History and physical examination: were sufficient to assess and treat nearly half of the traumatic neck pain presenting to our ED. In children with a history of trauma who had radiographic studies performed, more than 70% were described as normal. This clearly reinforced the need for more specific criteria to guide the need for cervical spine radiographs in children with trauma. 43 C1 C2 rotary subluxation We confirm that a significant trauma to the upper neck is not necessary to cause C1 C2 subluxation. The patient with radiologically confirmed C1 C2 subluxation only described a movement of active extension of the neck before the torticollis. Prompt recognition and medical treatment of this condition are necessary to avoid the complications described with late diagnosis, such as acute rotatory deformity or recurrence of the subluxation. 44 Infectious disease Viral infection (n = 28, 85%) Bacterial infection (n = 5, 15%), 80% have fever Three children with RPA did not have respiratory distress RPA: neck swelling and limitation Our series was in accordance with the literature and showed the advantages of CT imaging for the diagnosis of RPA. 45 Our data suggested that patients with neck ailments of benign origin recover in a few days with conservative, supportive treatment. 46 Main limitations The main limitations of the study were a result of retrospectively obtained data from ED encounters. This resulted in some variability in the quality of information found in the charts. Patients were designated by a triage nurse to be seen on the medical or surgical side of the ED before a complete medical evaluation. This could have lead to selection bias, although only the final ED diagnosis was taken into consideration for group selection. 47 It is worth reemphasizing the need for reevaluation should any acute torticollis not resolve within a week to 10 days. 48

9 Aortic aneurysm, headache and neck pain? Thank you for your attention! 49 50