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Karaman Ilić, Maja and Kern, Josipa and Babić, Irena and Šimić, Diana and Kljenak, Antun and Majerić Kogler, Višnja (2011) Application of the procedural consolidation concept to surgical treatment of children with epidermolysis bullosa: a retrospective analysis. Croatian Medical Journal, 52 (4). English abstract AIM: To assess the efficacy of the procedural consolidation concept (PCC) at reducing the number of sessions of general anesthesia necessary for treating children with epidermolysis bullosa (EB). ----- METHODS: We examined the records of children treated at Children's Hospital of Zagreb between April 1999 and December 2007. Children treated before the introduction of PCC in January 2005 (n=39) and after (n=48) were analyzed in order to determine the effect of PCC on the occurrence of complications, days of hospitalization, and number of hospitalizations. ----- RESULTS: During the study period, 53 patients underwent 220 sessions of general anesthesia for a total of 743 surgical interventions per session. Before the introduction of PCC (n=39 patients, 83 sessions), the median number of interventions per session was 2 (range 1-5), and after the introduction of PCC (n=48 patients, 137 sessions) it was 4 (range 3-7, P<0.001). After the introduction of PCC, the median number of complications per anesthesia session increased from 2 (range 0-10) to 3 (range 0-10) (P=0.027), but the median number of complications per surgical procedure decreased from 1 (range 0-10) to 0.6 (range 0-2.5) (P<0.001). PCC lengthened each anesthesia session from a median of 65 minutes (range 35-655) to 95 minutes (range 50-405), (P<0.001). Total length of hospitalization was similar before (median 1, range 1-4) and after (median 1, range 1-3) introduction of PCC (P=0.169). The number of hospitalization days per procedure was 3 times lower after the introduction of PCC (median 0.3, range 0.2-3) than before (median 1, range 0.75-1.7) (P<0.001). ----- CONCLUSION: PCC should be considered an option in the surgical treatment of children with EB. Actions (login required)
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Karaman Ilić, Maja (2011) Povoljni učinak vremenskog usklađivanja elektivnih kirurških zahvata u bolesnika s buloznom epidermolizom. PhD thesis, Sveučilište u Zagrebu. Croatian abstract Bulozna epidermoliza (EB) spada u grupu rijetkih genetskih bolesti koju karakterizira kutana i ekstrakutana krhkost, stvaranje mjehura i nakon blage mehaničke traume. Ozljede većinom zarastaju stvaranjem ožiljka. Vodeće obilježje nasljedne bulozne epidermolize je osjetljivost na mehanički pritisak. Bolest je kronična degenerativna, progresivna i smrtonosna. Bule koje najčešće viđamo na koži bolesnika mogu nastati i na bilo kojoj mukoznoj površini. Nakon pucanja mjehura zaostaje ranjiva površina koja zarasta stvarajući atrofične ožiljke. Tradicionalno buloznu epidermolizu prema fenotipu, načinu nasljeđivanja i genotipu dijelimo na tri glavna oblika: EB Simplex, Junctional EB i Dystrophic EB. U novije vrijeme ovoj podjeli dodan je još jedan, glavni oblik – Hemidemosomal EB. Svi glavni oblici bulozne epidermolize imaju svoje podtipove. Dva glavna podtipa distrofičnog oblika bulozne epidermolize su Dominant Dystrophic EB i Recessive Dystrophic EB. Bolesnici koji boluju od distrofičnog oblika najčešće su kirurški liječeni oblik bulozne epidermolize. Bulozna epidermoliza je multisistemna bolest s brojnim prijeoperacijskim, perioperacijskim i poslijeoperacijskim izazovima. Ožiljci, strikture i stenoze kože i sluznica samo su neki od problema koji zahtijevaju kirurško liječenje. Bolesnici s buloznom epidermolizom, s anesteziološkog stajališta, predstavljaju grupu visoko rizičnih bolesnika zbog postojeće malnutricije, anemije, hipoproteinemije, relativno česte kardiomiopatije i gotovo uvijek prisutnog ograničenog otvaranja usta. Multisistemne bolesti treba liječiti multidisciplinarni tim liječnika. Prijeoperacijska priprema treba biti individualizirana, a anesteziološki problemi objektivizirani. Da bi se izbjegle ponavljane anestezije, u planiranju kirurških aktivnosti tijekom jedne anestezije treba razmatrati rješavanje što većeg broja potrebnih kirurških intervencija. Djeca s buloznom epidermolizom, koja se liječe u Klinici za dječje bolesti Zagreb, godinama su se javljala liječniku u trenutku kada su komplikacije primarnih i sekundarnih lezija postale hitne (nemogućnost hranjenja, bol, kaheksija, karcinom). Bolesnici su sami indicirali kirurško liječenje. U siječnju 2005., formiranjem multidisciplinarnog tima, stvoreni su uvjeti za implementaciju vremenskog usklađivanja elektivnih kirurških zahvata. Praksa od maksimalno dva kirurška zahvata zamijenjena je Konceptom koji podrazumijeva veći broj kirurških intervencija tijekom jedne opće anestezije. Uvođenjem vremenskog usklađivanja elektivnih kirurških zahvata smanjuje se broj općih anestezija tijekom kojih se izvodi značajno veći broj zahvata. U ovoj doktorskoj disertaciji ponudili smo hipotezu povoljnog učinka vremenskog usklađivanja elektivnih kirurških zahvata u djece/bolesnika s buloznom epidermolizom na smanjenje broja općih anestezija bez značajnog utjecaja na broj komplikacija ili duljinu hospitalizacije. Analizirali smo dokumentaciju pedeset i troje djece/bolesnika s buloznom epidermolizom koji su se od travnja 1999. do prosinca 2007. kirurški liječili u Klinici za dječje bolesti Zagreb. Deskriptivna statistika, uključujući srednju vrijednost i standardnu devijaciju, korištena je za kirurške zahvate i komplikacije. Frekvencije zahvata i komplikacija izražene su kao apsolutne vrijednosti, postoci i omjeri. Značajna razlika između razdoblja prije i nakon uvođenja Koncepta vremenskog usklađivanja elektivnih kirurških zahvata testirana je primjenom Kruskal-Wallisova testa. Dobiveni rezultati potkrijepili su pretpostavku da uvođenje vremenskog usklađivanja elektivnih kirurških zahvata u djece s buloznom epidermolizom ima pozitivni učinak u smanjenju broja općih anestezija bez utjecaja na vrstu razvijenih komplikacija. Zabilježeni porast broja komplikacija po općoj anesteziji nije utjecao na dužinu boravka u bolnici. Mislim da se Koncept vremenskog usklađivanja elektivnih kirurških zahvata treba razmatrati kao mogućnost u liječenju djece s buloznom epidermolizom. English abstract Epidermolysis bullosa (EB) refers to a group of rare genetic disorders characterized by cutaneous and extracutaneous fragility, blistering and subsequent scaring even after a mild mechanical stress. The hallmark feature of inherited EB is mechanical fragility. The illness is chronic, degenerative, difficult to treat and lethal. Although the most common symptom of EB is bullae of skin epithelium, blisters may arise on virtually any mucosal surface. Bullae usually heal, leaving atrophic scars. Digital fusion following scar formation is common, rendering the hands useless. Traditionally, EB is divided into three major types based on phenotype, mode of inheritance and genotype: EB-Simplex, Junctional-EB and Dystrophic-EB. More recently, a fourth form, Hemidesmosomal-EB, was introduced to describe EB in which blisters emerge in the dermal-epidermal circuit. Each major EB type has subtypes. Two major subtypes of Dystrophic EB are Dominant Dystrophic EB and Recessive Dystrophic EB. Dystrophic EB is probably the most common form of surgically treated EB. EB has become recognized as a multisystem disorder that poses a number of pre-, peri-, and postoperative challenges. Scars, strictures and stenoses are some of the problems that require surgical treatment. In addition, EB affects the oral-gastrointestinal system, creating the need for operative treatment and significantly affecting perioperative care. Patients with EB are a high-risk group for anesthesiology because of malnutrition, anemia, hypoproteinemia, dilated cardiomyopathy, and limited mouth opening. A multidisciplinary team approach to EB treatment is recommended. Preoperative preparation must be individualized, with special attention given to the potential anesthetic problems of patients with EB. When procedures under anesthesia are planned, it is best to coordinate as many surgical procedures as possible to avoid repeated anesthesia. Surgical care for EB patients at the Children's Hospital of Zagreb was for many years quite basic and was not codified into a uniform and standard procedure. In January 2005, the hospital adopted a team approach to treating EB, which led to implementation of a procedural consolidation concept (PCC).The standard approach of repeated sessions of anesthesia with no more than two surgical procedures performed each time was replaced by PCC, which involved scheduling multiple surgical approaches and procedures during the same session of anesthesia. PCC is based on multidisciplinary team-based treatment planning aimed at reducing the number of times that patients with EB are subjected to general anesthesia. In this study we analyzed whether PCC has been successful at reducing the number of anesthesia sessions without increasing complications or length of hospitalization. Records of 53 children with EB surgically treated at Children's Hospital of Zagreb between April 1999 and December 2007 were examined. Descriptive statistics, including average ±SD and range for number of surgical procedures, were used. Frequencies of complications were presented as absolute values and percentages. For not normal distributions of variables under considerations the Kruskal-Wallis test was used to test the significance of pre- and post-intervention outcomes. Results show that introduction of PCC reduced the number of general anesthesia sessions and the number of hospitalizations. It did not affect the range of complications and although it did increase the mean number of complications per anesthesia session, it did not affect the total hospitalization time. PCC should be considered an option in the treatment of children with EB. Actions (login required)