CARE OF THE PEDIATRIC ASPLENIC PATIENT. Michael Siegenthaler, MD and Nadine Khouzam, MD

Transcription

1 CARE OF THE PEDIATRIC ASPLENIC PATIENT Michael Siegenthaler, MD and Nadine Khouzam, MD

2 CASE OVERVIEW 11-year-old Arabic speaking female who recently immigrated from Jordan presented to the office as a walk-in with fevers, abdominal pain, and generalized malaise Patient had been running a fever for the past 24 hours Had been hallucinating the previous evening Also complaining of a headache Has had several episodes of non-bloody, non-bilious vomiting Reports watery diarrhea Denies URI symptoms, cough, sore throat, or new skin rash

3 Past Medical History: Nocturnal enuresis, eczema PAST MEDICAL HISTORY Past Surgical History: Splenectomy in Jordan performed secondary to a congenital splenic cyst not currently on antibiotic prophylaxis Medications: Triamcinolone 0.1% cream, polyethylene glycol 17 g daily, loratadine 10 mg daily Allergies: NKDA

4 IMMUNIZATION HISTORY Had received MMRV, Hep A, Hep B, IPV, Meningococcal, and Tdap vaccines through the clinic Was given a dose of Prevnar 13 No record of a PPSV23

5 PHYSICAL EXAM VS: HR 128, RR 18, BP 105/73, Temp 102.2ºF General Appears ill on exam; she is lying flat on the exam table and is tearful due to abdominal discomfort and headache HEENT NCAT, right TM and external ear canal are unremarkable; left ear canal is obstructed by cerumen; mucous membranes appear moist, no oral lesions; no tonsillar exudate; PERRL; mild photophobia Neck Supple; full range of motion; no adenopathy; no tenderness to palpation Cardiovascular Tachycardic; S1, S2 normal; no murmurs, rubs, or gallops Respiratory Effort normal; no decreased breath sounds; no wheezing, rales, or rhonchi Abdominal Soft; generalized tenderness to palpation without rebound rigidity, or guarding; large healed horizontal incision over the LUQ of the abdomen Neurologic Alert; cranial nerves intact; no focal deficits Skin Warm and dry

6 INITIAL LABORATORY EVALUATION % neutrophils on differential Rapid influenza - negative ESR 39, CRP UA SG 1.030, 1+ bilirubin, 1+ protein, 1+ ketones, 2+ blood, Neg LE, Neg Nitrite

7 SUBSEQUENT EVALUATION The patient was given a dose of IV ceftriaxone and an IV fluid bolus Due to concerns about the patient s immunocompromised state and possibility of sepsis, she was transferred to the Upstate Pediatric ED Appendicitis was ruled out with an abdominal ultrasound She was admitted for observation and continued empiric antibiotics Her fever resolved and she was stable for discharge on hospital day 2 Blood and urine cultures remained negative Given her GI symptoms, stool cultures were sent She tested positive for Campylobacter Pediatric Infectious Disease was consulted and recommended that she be discharged on 250 mg of amoxicillin daily for prophylaxis

8 SPLENIC CYSTS Rare clinical entity with approximately 800 case reports worldwide The most common causes are parasitic infections, congenital cysts, and post traumatic cysts They are usually discovered incidentally, but can cause symptoms secondary to compression if they are large enough Smaller cysts can be observed, but surgical intervention is generally indicated for cysts greater than 4-5 cm in diameter, as well as any cyst that is causing symptoms Potential complications include rupture, hemorrhage, or abscess formation Traditionally, total splenectomy has been considered the treatment of choice Attention is now being focused on developing spleen sparing techniques to preserve immunologic function

9 An example of a splenic cyst as seen on CT scan. This image is from a 26-year-old female. The cyst was discovered during work up of chronic abdominal pain. It measures 11.5 x 10.7 x 8.0 cm.

10 IMMUNE DEFICIENCIES IN ASPLENIC PATIENTS Impaired clearance of bacteria from the blood stream The spleen is normally the most efficient organ for clearing IgG coated bacteria from the blood stream Asplenic patients also have deficits in humoral immunity with decreased serum IgM levels to polysaccharides and a reduction in memory B cells This results in a diminished immune response to vaccination

11 ASPLENIA IN PEDIATRIC PATIENTS Can result from congenital absence of the spleen, surgical removal, or medical conditions that cause reduced splenic function (sickle cell disease, hereditary spherocytosis, immune thrombocytopenic purpura, etc.) Highest frequency of sepsis is in the first three years following splenectomy Mortality rates for asplenic patients with sepsis from an encapsulated organism are 50-70%, with the highest mortality rates in children under two years of age

12 RESPONSIBLE ORGANISMS Streptococcus pneumonia is the responsible organism in 50% of cases Other important organisms are Haemophilus influenza type B Neisseria meningitidis Salmonella E. coli Staphylococcus aureus Capnocytophaga species from animal bites Some data also suggest asplenic patients are at higher risk for malaria and Babesia infections

13 SEPSIS IN ASPLENIC PATIENTS Usually has a nonspecific presentation without localizing symptoms May present with fevers, chills, sore throat, muscle aches, vomiting, and diarrhea Clinical decompensation and shock can develop within a few hours of initial presentation Overwhelming bacteremia can develop to the point where gram positive cocci can be observed in stained smears of peripheral blood

14 IMMUNIZATION IMPLICATIONS

15 PNEUMOCOCC AL VACCINATION Asplenic children should receive the traditional four dose series of PCV13 For previously unvaccinated children If months of age only two doses needed If greater than 24 months only one dose needed The PPV23 vaccine should be administered to asplenic children as soon as possible after 24 months of age, followed by a booster dose five years after the first dose The PCV13 is administered first, has it is thought to prime the effect of the PPV23 vaccine

16 MENINGOCOCCUS VACCINATION Asplenic infants should receive a four dose series of a quadrivalent meningococcal vaccine, while children identified as asplenic after 12 months of age only require two doses at least eight weeks apart Some data suggests giving a booster vaccine every five years If available, asplenic patients also benefit from vaccinations aimed against serotype B strains

17 HAEMOPHILUS INFLUENZA TYPE B Children should receive the traditional three vaccine schedule for Hib B Patients greater than 5 years of age who have never been vaccinated or who have missed a dose should receive an extra dose Some experts recommend an additional dose for asplenic patients after age 5 even if previously fully immunized

18 OTHER VACCINATION CONSIDERATIONS Asplenic patients should receive the yearly influenza vaccine to lower the rate of subsequent bacterial infections Individuals traveling to parts of the word where Salmonella typhi is endemic should consider vaccination prior to travel When possible vaccinations should be administered at least two weeks (ideally 4-6 weeks) prior to splenectomy

19 ANTIBIOTIC PROPHYLAXIS The exact timing and duration of antibiotic prophylaxis is controversial The American Academy of Pediatrics recommends prophylaxis until the age of five and a minimum of one year of prophylaxis postsplenectomy, assuming the patient is up-to-date on ageappropriate immunizations. Some organizations advocate lifelong antibiotic prophylaxis Individuals with an episode of overwhelming post splenectomy infection (OPSI) should be on lifelong prophylaxis Penicillin is the drug of choice, but Augmentin can be used in children less than three months due to concern for E. Coli and Klebsiella Erythromycin can be used for PCN allergic patients, but it does have higher rates of pneumococcal resistance, desensitization should be considered

20 ANTIBIOTIC PROPHYLAXIS

21 PATIENT EDUC ATION Very important in conjunction with proper vaccination and antibiotic prophylaxis Parents must be educated about the importance of seeking medical attention for any illness or fever Alert bracelets are available for patients with asplenia

22 MANAGEMENT OF A FEBRILE EPISODE

23 REFERENCES 1. Iorga C, Strambu V, Popa F, Puscu C, Radu P. Congenital splenic cyst case study. Journal of Medicine and Life. 2011;4(1): Geraghty M, Khan IZ, Conlon KC. Large primary splenic cyst: A laparoscopic technique. Journal of Minimal Access Surgery. 2009;5(1): doi: / Salvadori MI, Price VE, Canadian Paediatric Society, Infectious Diseases and Immunization Committee. Preventing and treating infections in children with asplenia or hyposplenia. Paediatrics & Child Health. 2014;19(5): Rubin LG, Schaffner W. Care of the asplenic patient. New England Journal of Medicine 2014; 371(4):349-56

24 IMAGES COURTESY OF