Tonsillectomy, and Adenoidectomy T & A is the most common surgical procedure in children in the US today with annual expenditure of $500 million. Trends are toward selective performance of T & A, with 259,000 surgeries in 1987, 1/4 the 1970 figure. Recurrent infection has always been the #1 indication, recently an increasing % are performed for OSA (approx 19% 1986 study at Mt Sinai).

History Celsus 50 A.D. Caque of Rheims Philip Syng Wilhelm Meyer 1867 Samuel Crowe 4/16/2017 rhinoplastyman@yahoo.com

Embryology 8 weeks: Tonsillar fossa and palatine tonsils develop from the dorsal wing of the 1st pharyngeal pouch and the ventral wing of the 2nd pouch; tonsillar pillars originate from 2nd/3rd arches Crypts 3-6 months; capsule 5th month; germinal centers after birth 16 weeks: Adenoids develop as a subepithelial infiltration of lymphocytes The first 8 weeks constitutes the period of greatest embryonic development of the head and neck. There are 5 arches (pharyngeal or branchial). Btw these arches are the clefts externally and the pouches internally. Each pouch has a ventral or dorsal wing. The derivatives of arches are usually mesoderm origin. The cleft is lined by ectoderm, the pouch is lined by endoderm The adenoids are colonized with bacteria soon after birth, enlarge early and middle childhood form antigenic challenges and should regress by early adulthood. Hypertrophic tonsils are rare in adults and suggest chronic infection or lymphoma. 4/16/2017 rhinoplastyman@yahoo.com

Anatomy Tonsils Adenoids Plica triangularis Gerlach’s tonsil Fossa of Rosenmüller Passavant’s ridge The tonsil is nestled in a fossa formed by the muscular anterior and posterior tonsillar pillars (palatoglossus and palatopharyngeus) and lying superficial to the superior constrictor muscle; preservation of these muscular condensations and the overlying mucosa is critical to maintaining physiologic function of the palate postoperatively. The tonsil is contiguous inferiorly with the lingual tonsil. The point of attachment (plica triangularis) must be transected during tonsillectomy. In pts with marked hypertrophy, this extension is freq quite large and can result in troublesome bleeding at the pt of transection at the base of the tongue. The adenoid is positioned in the midline of the posterior wall of the NP immediately inferior to the rostrum of the sphenoid and extending laterally to but not onto the lateral wall of the NP. It makes up the most rostral portion of the pharyngeal lymphoid tissue termed Waldeyer’s ring. The space created lateral to the adenoid and posteromedial to the ET orifice is termed the FOSSA of Rosenmuller. Gerlach’s tonsil is lymphoid tissue within lip of the fossa of Rosenmuller; goes into ET. Inferiorly, the adenoid extends nearly to the superior margin of the superior constrictor…Passavant’s ridge. 4/16/2017 rhinoplastyman@yahoo.com

Blood Supply Tonsils Adenoids Ascending and descending palatine arteries Tonsillar artery 1% aberrant ICA just deep to superior constrictor Adenoids Ascending pharyngeal, sphenopalatine arteries Tonsillar branch of the facial artery is the main supply of the entire tonsil. Facial artery: Tonsillar art Ascending palatine art Lingual art dorsal lingual branch IMA Desceding palatine Greater palatine Ascending pharyngeal (ECA) Venous drainage of the tonsil is thru lingual and pharyngeal veins which empty into the IJ. In most people the ICA lies 2cm posterolateral to the deep surface of the tonsil; however in 1% of the population, it is found just deep to the superior constrictor. Adenoids: Ascending palatine, ascending phayrngeal, pharyngeal br of IMA, ascending cervical branch of thyrocervical trunk 4/16/2017 rhinoplastyman@yahoo.com

Common Diseases of the Tonsils and Adenoids Acute adenoiditis/tonsillitis Recurrent/chronic adenoiditis/tonsillitis Obstructive hyperplasia Malignancy 4/16/2017 rhinoplastyman@yahoo.com

Acute Adenotonsillitis Etiology 5-30% bacterial; of these 39% are beta-lactamase-producing (BLPO) Anaerobic BLPO GABHS most important pathogen because of potential sequelae Throat culture Treatment 1. MC bacteria: Beta streptoccoci, staphylococci, streptoccocus pneumoniae, hemophilus 2. Prevalence of beta-lactamase producing organisms is rising: from 2 % in 1980 to 44% in 1989 (FIND STUDY) 3. Prevalence of anaerobic org is also rising Asymptomatic streptococcal pharyngitis responsible for at least 1/3 of ARF in 3rd world. Gold std is throat culture. Blood agar plate with septra more sensitive than plain agar plate. Culture both tonsils; if only one, may miss 25%. Rapid streptococcal antigen test, 12 min.; highly specific but variable sensitivity; must confirm negative result with a throat cx. Newer solid-phase enzyme immunoassay Older latex agglutination test Treat with 10 day course of PCN if high clinical suspicion (augmentin, clinda, pcn + rifampin for recurrence) Post treatment culture: high risk RF, remain symptomatic, recurring symptoms; if asymptomatic but positive cx, treat if h/o RF or if FH of RF Suspect infectious mononucleosis if sore throat and malaise persist despite abx treatment; order WBC and Paul-Bunnell. Characterized by white membrane covering one or both tonsils and hypersensitivity to ampicillin. Look for atypical mononuclear cells and positive Paul-Bunnell blood test. 4/16/2017 rhinoplastyman@yahoo.com

Microbiology of Adenotonsillitis Most common organisms cultured from patients with chronic tonsillar disease (recurrent/chronic infection, hyperplasia): Streptococcus pyogenes (Group A beta-hemolytic streptococcus) H.influenza S. aureus Streptococcus pneumoniae Tonsil weight is directly proportional to bacterial load. Study by Brodsky et al (1988) taking cultures from core specimens (not surface). Core species do not always correlate with surface bacteria. 90% correlation with H.influenza, 73% strept pyogenes 4/16/2017 rhinoplastyman@yahoo.com

Acute Adenotonsillitis Differential diagnosis Infectious mononucleosis Malignancy: lymphoma, leukemia, carcinoma Diptheria Scarlet fever Agranulocytosis 4/16/2017 rhinoplastyman@yahoo.com

Medical Management PCN is first line, even if throat culture is negative for GABHS For acute UAO: NP airway, steroids, IV abx, and immediate tonsillectomy for poor response Recurrent tonsillitis: PCN injection if concerned about noncompliance or antibiotics aimed against BLPO and anaerobes For chronic tonsillitis or obstruction, antibiotics directed against BLPO and anaerobes for 3-6 weeks will eliminate need for surgery in 17% Co-amoxiclav or clindamycin or PCN+Rifampin 4/16/2017 rhinoplastyman@yahoo.com

Obstructive Hyperplasia Adenotonsillar hypertrophy most common cause of SDB in children Diagnosis:snoring, restless sleep, FTT, daytime symptoms… poor mentation, decreased attn span, poor scholastic performance, dysphagia, nocturnal enuresis, chronic mouth breathing Indications for polysomnography Interpretation of polysomnography Perioperative considerations Diagnosis of OSA is based on H & P (snoring, restless sleep, FTT, daytime symptoms… poor mentation, decreased attn span, poor scholastic performance, dysphagia, nocturnal enuresis, chronic mouth breathing; predisposing conditions craniofacial abnormalities, NM disorders, FTT, cor pulmonale, Downs syndrome) MC symptom in kids is snoring (adults is daytime somnolence). Obtain sleep study when PE does not correlate with history ($1600), or when suspect central component. Apnea (10s breathing pause)from complete obstruction is uncommon in children. Children tend to have a continuous partial obstructive hypoventilation that is characterized by decreased oxygen saturation, hypercapnia, labored paradoxical resp efforts, and snoring. Controversy over how to interpret sleep study in kids… few normative data. Marcus et al.(1992) studied normal resp patterns in children during sleep. Abnormal values: >1 obstructive apnea of any duration per hour central apnea assoc with desat <90% Pco2>53 or Pco2>45 for more than 60% test time fall of o2 sat < 92% No consensus on indications for surgery for those without severe obstruction/apnea. 4/16/2017 rhinoplastyman@yahoo.com

Interpretation of polysomnography: Indications for surgical intervention >1 apnea in 1 hour Central apnea + o2sat<90% O2sat<92% CO2>53 CO2>45 in more than 60% of test time 4/16/2017 rhinoplastyman@yahoo.com

Unilateral Tonsillar Enlargement Apparent enlargement vs true enlargement Non-neoplastic: Acute infective Chronic infective Hypertrophy Congenital Neoplastic Apparent: tonsil sits in more medial position, displacement medially by PTA or parapharyngeal space mass. Chronic infections: tubercular tonsillitis, actinomycosis, and congenital syphilis Congenital include teratoma, hemangioma, lymphangioma, and cystic hygroma. Neoplastic: Benign papillomas Lymphoma (usually non-Hodgkins B-cell) and squamous cell 4/16/2017 rhinoplastyman@yahoo.com

Peritonsillar Abscess Displacement of tonsil and uvula medially, trismus, dysphagia, pain referred to the ear, malaise, fever, cervical adenopathy. Initial mgmt is needle aspiration, IM penicillin, oral penicillin. Quinsy tonsillectomy for uncooperative, toxic patient, bleeding. Peritonsillar Abscess 4/16/2017 rhinoplastyman@yahoo.com

ICA Aneurysm 4/16/2017 rhinoplastyman@yahoo.com This patient came to the ER for sore throat ICA Aneurysm 4/16/2017 rhinoplastyman@yahoo.com

Pleomorphic Adenoma 4/16/2017 rhinoplastyman@yahoo.com Consider masses in the parapharyngeal space for apparent UTE including tumors of the deep lobe of the parotid gland (ie pleomorphic adenoma), chemodectomas, neurofibromata, and enlargement of the parapharyngeal lymph nodes. Pleomorphic Adenoma 4/16/2017 rhinoplastyman@yahoo.com

Other Tonsillar Pathology Hyperkeratosis, mycosis leptothrica Tonsilloliths Yellow spicules due to hyperkearatineized areas of epithelium are sometimes extensive over the tonsil. It is important to probe the tonsil to be certain these areas are not exudate. No treatment is required unless assoc with tonsillitis. Tonsilloliths are yellow gritty particles in crypts, more commonly seen in adults with a h/o recurrent tonsillitis. Elongated styloid process causes pain exacerbated during maximal deglutition and deep breathing…. 2nd branchial arch derivitative, approx 2.5 cm long, located btw internal and ECA just lateral to tonsillar fossa. 4/16/2017 rhinoplastyman@yahoo.com

Candidiasis 4/16/2017 rhinoplastyman@yahoo.com A fungal infection of the pharynx and one of the most common upper respiratory tract manifestation of AIDS. Also seen in neonates and may complicate treatment with broad spectrum antibiotics. Characterized by extensive white areas (either continuous or punctate) covering the entire oropharynx and not limited to the tonsil. Swab shows candida albicans. Candidiasis 4/16/2017 rhinoplastyman@yahoo.com

Retention Cysts 4/16/2017 rhinoplastyman@yahoo.com These are common on the tonsil and appear as sessile yellow swellings. If small, they can be ignored. Also seen after tonsillectomy in region of the fauces. Retention Cysts 4/16/2017 rhinoplastyman@yahoo.com

Supratonsillar Cleft 4/16/2017 rhinoplastyman@yahoo.com This recess near the superior pole of the tonsil tends if large to collect debris. A mass of yellow fetid tissue can be extruded from the tonsil with pressure, and discomfort, halitosis are symptoms. Tonsillectomy may be necessary. Supratonsillar Cleft 4/16/2017 rhinoplastyman@yahoo.com

Indications for Tonsillectomy; Historical Evolution 4/16/2017 rhinoplastyman@yahoo.com

Indications for Tonsillectomy Paradise study Frequency criteria: 7 episodes in 1 year or 5 episodes/year for 2 years or 3 episodes/year for 3 years Clinical features (one or more): T 38.3, cervical LAD (>2cm) or tender LAD; tonsillar/pharyngeal exudate; positive culture for GABHS; antibiotic treatment From 1971-1994 the Children’s Hospital of Pittsburgh conducted parallel randomized and nonrandomized clinical trials to determine 1. efficacy of tonsillectomy in reducing the frequency and severity of episodes of pharyngitis, 2. the efficacy of adenoidectomy in reducing the freq/severity of OM, and 3. the effect of adenoidectomy of the course of nasal obstruction due to large adenoids Findings: 1. Histories of recurrent throat infections that are undocumented do not validly predict recurrence; need documentation by physician before performing tonsillectomy 2. Using the selection criteria, the incidence of throat infection during the first 2 years of f/u was significantly lower in the surgical groups 3. Many pts in the nonsurgical group had fewer than 3 episodes of infx, and most cases were mild… therefore, treatment should be individualized, taking into consideration pt/parental preference, anxieties, tolerance of illness, tolerance of antimicrobial drugs, child’s school performance in relation to illness-related absence, accessability of health-care services, out-of-pocket costs, nature of available anesthetic and surgical services/facilities 4/16/2017 rhinoplastyman@yahoo.com

Indications for Tonsillectomy AAO-HNS: 3 or more episodes/year Hypertrophy causing malocclusion, UAO PTA unresponsive to nonsurgical mgmt Halitosis, not responsive to medical therapy UTE, suspicious for malignancy Individual considerations Contraindications: Tonsillectomy Acute infection Anemia Disorders of hemostasis 4/16/2017 rhinoplastyman@yahoo.com

Recurrent tonsillitis in patient with heart valve dis Also: Recurrent tonsillitis in patient with heart valve dis In patient with febrile convulsion Unresponsive carrier Obstructive IMN unresponsive to medical therapy Carrier of diphtheria Chronic tonsillitis with never-ending sore throat OSAS FTT Disphagia phonation disorders Malocclusion Craniofacial growth abnormalities Corpulmonale Suspicious malignancy 4/16/2017 rhinoplastyman@yahoo.com

Pathophysiology of corpulmonale in OSA: URT obstruction Alveolar hypovantilation Chronic hypoxia and hypercapnea Respiratory acidosis Vasoconstriction of alveolar capillaries Right atrial dilatation Pulmonary hypertension Corpulmonale and CHF 4/16/2017 rhinoplastyman@yahoo.com

Pathophysiology of enuresis: SDB REM abnormalities Irregularities in ADH secretion Pathophysiology of FTT: SDB REM abnormalities Irregularities in GH secretion, may sometimes stop totally Both return to normal following A&T 4/16/2017 rhinoplastyman@yahoo.com

ADHD (Attention Deficit Hyperactivity Disorder) is the most Common psychiatric diagnosis in children. In direct correlation with Sleep Disordered Breathing(SDB) Pathophysiology uncertain, Maybe due to abnormal sleep pattern Neurocognitive development delays: Healthy sleeping pattern: important component of brain growth Frontal cortex growth continues up to puberty Most critical age: 3-7 4/16/2017 rhinoplastyman@yahoo.com

Indications for Adenoidectomy Paradise study 28-35% fewer acute episodes of OM with adenoidectomy in kids with previous tube placement Adenoidectomy or T & A not indicated in children with recurrent OM who had not undergone previous tube placement Gates et al Recommend adenoidectomy with M & T as the initial surgical treatment for children with MEE > 90 days and CHL > 20 dB Paradise: Parallel randomized and nonrandomized clinical trials of 213 children who developed recurrence of OM after extrusion of t-tubes; In both trials, over a period of 2 years, 28-35% fewer episodes than controls. Gates: 578 children with chronic middle ear effusion. Adenoidectomy combined with myringotomy or with t tube placement proved to be more effective thatn myringotomy or tube placement alone in preventing recurrences of OM over a 2 year period * differences were small (31 vs 36 weeks as mean cumulative times with effusion in 2 treatment groups over 2 yr f/u). TT surgery alone is assoc with higher rate of repeat surgeries, increased rate of otorrhea, greater expense and human cost of illness than initial adenoidectomy and myringotomy 4/16/2017 rhinoplastyman@yahoo.com

Indications for Adenoidectomy Obstruction: Chronic nasal obstruction or obligate mouth breathing SDB with FTT, cor pulmonale Dysphagia Speech problems Severe orofacial/dental abnormalities Infection: Recurrent/chronic adenoiditis (3 or more episodes/year) Recurrent/chronic OME (+/- previous BMT) Contraindications: Adenoidectomy Overt or submucous CP Neurologic or neuromuscular abnormalities with impaired palatal function Anemia Disorders of hemostasis 4/16/2017 rhinoplastyman@yahoo.com

Chronic sinusitis: first step Also: Chronic sinusitis: first step biofilm in 95% adenoid specimen vs. 2% in SDB Chronic recurrent AOM COM Chronic otorrhea VT and persistent discharge Suspecious malignancy 4/16/2017 rhinoplastyman@yahoo.com

PreOp Evaluation of Adenoid Disease Triad of hyponasality, snoring, and mouth breathing Rhinorrhea, nocturnal cough, post nasal drip “Adenoid facies” “Milkman” & “Micky Mouse” Overbite, long face, crowded incisors 4/16/2017 rhinoplastyman@yahoo.com

PreOp Evaluation of Adenoid Disease Differential diagnoses Allergic rhinitis Sinusitis GERD For concomitant sinus disease, treat adenoids first 4/16/2017 rhinoplastyman@yahoo.com

PreOp Evaluation of Adenoid Disease Evaluate palate Symptoms/FH of CP or VPI Midline diastasis of muscles, bifid uvula CNS or neuromuscular disease Preexisting speech disorder? Speech path consult for speech disorder. Submucous cp 1 in 1200 4/16/2017 rhinoplastyman@yahoo.com

PreOp Evaluation of Adenoid Disease Lateral neck films are useful only when history and physical exam are not in agreement. Accuracy of lateral neck films is dependent on proper positioning and patient cooperation. 4/16/2017 rhinoplastyman@yahoo.com

PreOp Evaluation of Adenoid Disease 4/16/2017 rhinoplastyman@yahoo.com

PreOp Evaluation of Tonsillar Disease History Documentation of episodes by physician FTT Cor pulmonale Poststreptococcal GN Rheumatic fever 4/16/2017 rhinoplastyman@yahoo.com

PreOp Evaluation of Tonsillar Disease TONSIL SIZE 0 in fossa +1 <25% occupation of oropharynx +2 25-50% +3 50-75% +4 >75% Avoid gagging the patient 4/16/2017 rhinoplastyman@yahoo.com

PreOp Evaluation of Tonsillar Disease Down syndrome 10% have AtlantoAxial laxity Obtain lateral cervical films (flexion/extension) when positive findings on history, PE If unstable, need neurosurgical evaluation preoperatively Large tongue and small mandible… difficult intubation Prone to cardiac arrhythmias/hypotension during induction Instability is caused by laxity of transverse ligament. Neck pain, muscular problems. Hyperactive DTR, clonus atlas-dens interval > 4.5mm 4/16/2017 rhinoplastyman@yahoo.com

PreOp Evaluation for Adenotonsillar Disease Coagulation disorders Historical screening CBC, PT/PTT, BT, vWF activity Hematology consult von Willebrand’s disease ITP Sickle cell anemia Von Willebrand’s disease is the most common inherited coagulopathy (AD with variable expression) (1% population) and is caused by a deficiency in Factor VIII:VW complex necessary in platelet activation. 3 types… type 1 is the most common (80-90%) with subnormal levels of qualitatively normal vWF and most will respond to desmopressin. Type 2 is a defect in the factor, type 3 is complete absence of the factor. DX elevated PTT, BT, decreased vWF antigen, factor VIII procoagulant activity, ristocetin cofactor activity; measure response of levels to desmopressin (0.3microg/kg IV) RX give IV over 30 min preop (peak levels 45-60 min), 12 hr postop, then q am until eschar completely sloughed and fossae completely healed; also give aminocaproic acid or tranexamic acid preop and postop to decrease fibrinolysis (oral cavity high conc of fibrinolytic enzymes); not useful type 2/3 adverse effects… Na <132 or tachyphylaxis, d/c desmopressin, give cryoprepipitate or vWF-containing antihemophilic factor ITP: 4/16/2017 rhinoplastyman@yahoo.com

Principles of Surgical Management Numerous techniques: Guillotine Tonsillotome Beck’s snare Dissection with snare (Scissor dissection, Fisher’s knife dissection, Finger dissection Electrodissection Laser dissection (CO2, KTP) Coblation RadioFrequency … Surgeon’s preference 4/16/2017 rhinoplastyman@yahoo.com

Post Operative Managment Criteria for Overnight Observation Poor oral intake, vomiting, hemorrhage Age < 3 Home > 45 minutes away Poor socioeconomic condition Comorbid medical problems Surgery for OSA or PTA Abnormal coagulation values (+/- identified disorder) in patient or family member MC reasons for inpt stays… emesis, dehydration, hemorrhage, obstruction, pulm edema < 3years: 7% airway complications (2.3 times other kids), 4% dehydration, 1.5 % hemorrhage; less likely to cooperate with oral intake and more likely to have surgery for airway obstruction Conditions associated with a complicated postop course (resp compromise): CP, seizures, age <3, congenital heart disease, prematurity, chromosomal abnormalities, loud snoring with apnea, difficulty breathing during sleep Excessive adenotonsillar tissue obstructs airway and increases resistance to inspiration/expiration… maintains PEEP with increased intrathoracic venous and hydrostatic pressure. Sudden relief of excess PEEP by intubation or T & A results in transudation of fluid into interstitial and alveolar spaces….pulm edema. Treatment… intubation and reestablishment of PEEP. 4/16/2017 rhinoplastyman@yahoo.com

Complications #1 Postoperative bleeding Other: Sore throat, otalgia, uvular swelling Respiratory compromise Dehydration Burns and iatrogenic trauma Dental problems and trauma Mortality 1 in 16,000 to 35,000 (anesthetic and hemorrhage); Hemorrhage 0.1-8.1%; 76% occur within first 6 hrs; 0.04% require transfusion; 0.002% mortality (mc for primary); Etiology: retained adenoid tissue, damage to post pharyngeal wall muscle; Increased incidence winter, age > 20 Anesthetic: kinking, extubation, fire, laryngospasm Resp compromise: sudden loss of PEEP… pulmonary edema; avoid sedating analgesics Assess for loose teeth… post op CXR to r/o aspiration if loss of tooth Draping to avoid burns… avoid towel clips (penetration); avoid tape (accidental extubation when take drapes off) Sore throat: increased with increased age, electrocautery, KTP/ less with CO2 lasere and periop/postop antibiotics (4.4 to 3.3 days) Otalgia: referred from IX, r/o otitis, ET tube injury or edema Fever: normal in 1st 36 hr… watch for dehydration Dehydration: n/v 2nd to anesth, swallowed blood; decreased po intake with pain, esp younger kids less cooperative and smaller volume reserve; single intraoperative steroid earlier return to nl diet 4/16/2017 rhinoplastyman@yahoo.com

Rare Complications Velopharyngeal Insufficiency Nasopharyngeal stenosis Atlantoaxial subluxation/ Grisel’s syndrome Regrowth Eustachian tube injury Depression Laceration of ICA/ pseudoaneursym of ICA VPI: usu transient; sig in 1 in 1500-3000; only 1/3 identified preop as increased risk; > 2mo speech therapy; > 6-12mo pharyngeal flap NP stenosis: circumferential contracture of pharynx Waldeyer’s ring, T AND A; syphilis; increased risk with excessive mucosal excision; difficult to rx AA subluxation.. Grisel’s syndrome vertebral body decalcification and laxity of anterior transverse ligament secondary to infection in the nasopharynx… may cause spontaneous subluxation 1 week postoperatively…pain and torticollis (traumatic adenoidectomy or injection of local anesthestic into prevertebral space) 15-28% tonsil tags; 6% recurrent pharyngitis adenoids may grow from adjacent lymphoid tissue… incomplete removal? Laceration of ICA usu occurs medially and near the skull base. Pseudoanerusym of ICA requires embolization and proximal ligation. 4/16/2017 rhinoplastyman@yahoo.com

Management of Hemorrhage Ice water gargle, afrin Overnight observation and IV fluids Dangerous induction ECA ligation Arteriography Anesthetic induction is hazardous…. Hypovolemic, underestimated blood loss (T &C). Risk of aspiration, stomach full of swallowed blood… tracheotomy if active hemorrhage prevents intubation. ECA ligation via lateral neck incision, retraction of SCM posteriorly if unable to stop bleeding. Angiography if ECA ligation fails… ICA and ECA communicate via opthalmic/angular nasal arteries and via middle meningeal artery 4/16/2017 rhinoplastyman@yahoo.com

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Case study 13 year old female referred by PCP for frequent throat infections “She’s always sick. She’s been on four different antibiotics this year.” You call her pediatrician… he is out of town and his nurse can’t find the chart 4/16/2017 rhinoplastyman@yahoo.com

Case study No known medical problems, no prior surgical procedures Takes motrin for menustrual cramps No personal history of bleeding other than occasional nose bleeds and extremely heavy periods. Family history unknown. Patient is adopted. 4/16/2017 rhinoplastyman@yahoo.com

Case study Physical exam is unremarkable. Mom breaks down in tears when you tell her you do not have enough documentation of illness to warrant T & A. “I had to go on welfare because I’ve missed so much work from her being out sick.” You hesitate. She adds, “Her grades have dropped from all A’s to all F’s. If she misses any more school, she’ll be held back.” 4/16/2017 rhinoplastyman@yahoo.com

Case study You confirm with her pediatrician that she has had 4 episodes of tonsillitis this year and agree to T & A. Because of her history of epistaxis and menorrhagia, you order a PT, PTT, CBC, BT. She has a mild microcytic anemia and prolonged bleeding time. You order vWF activity level and consult hematology 4/16/2017 rhinoplastyman@yahoo.com

Case study She has a subnormal level of vWF, which responds to a DDAVP challenge (rise in vWF and Factor VII greater than 100%). You advise her to stop taking motrin. Before surgery, she receives desmopressin 0.3 microg/kg IV over 30 min and amicar 200mg/kg. 4/16/2017 rhinoplastyman@yahoo.com

Case study She receives the same dose of DDVAP 12 hours postoperatively and every morning. Amicar is given 100mg/kg PO q 6 hr. Before each dose of DDAVP, serum sodium is drawn. Sodium levels drop to 130. Desmopressin is discontinued and substituted with cryoprecipitate. 4/16/2017 rhinoplastyman@yahoo.com

Case study Patient presents to the ER on POD # 7 complaining of intermittent bleeding from her mouth. You order cryoprecipitate, draw a Factor VII level and CBC, and call her hematologist. Hemoglobin has dropped from 11.9 to 9.6. 4/16/2017 rhinoplastyman@yahoo.com

Case study PE reveals no active bleeding; an old clot is present You establish IV access, admit the patient for overnight observation, have her gargle with ice water, and administer crypoprecipitate No further bleeding occurs, patient is discharged the next day 4/16/2017 rhinoplastyman@yahoo.com