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Multinodüler guatrýn (MNG) torasik giriþ yoluyla mediasten içine doðru büyümesi veya direk olarak mediasten içindeki ektopik tiroid dokularý Substernal Guatr (SG) olarak tanýmlanmaktadýr. Ancak bu taným ile ilgili literatürde net bir konsensus bulunmamaktadýr.
Literatürde SG için bir çok taným bulunmaktadýr. Çoðu taným birbirine benzer veya örtüþmektedir. Tiroid normal anatomik pozisyonda, boyunda torasik giriþin süperiorunda yerleþtiðinden, onun torasik giriþin aþaðýsýna inip mediastinal organlarla komþu olmasý ile ilgili tanýmlardan klinik kullanýmý en basit olan tercih edilmelidir. Amerikan Tiroid Birliði (ATA) kýlavuzunda SG; supin pozisyonda boynu hafif ekstansiyonda olan hastada klinik veya radyolojik olarak tiroidin sternal çentiði veya klavikulayý geçmesi olarak tanýmlanmýþtýr.
SG’lar orijinine göre primer veya sekonder olarak sýnýflandýrýlabilir. Ayrýca servikal tiroid bezinin mediastene doðru büyümesinden oluþan primer SG ve ektopik mediastinal kitle olarak tanýmlanan sekonder SG’nin birlikte büyümesinden ortaya çýkan SG’lar da bulunmaktadýr. Biz bu tür SG’larýn mikst SG olarak tanýmlanmasýný uygun bulmaktayýz ve bu yazýda da bu þekilde tanýmladýk.
Etyoloji ve etyopatogenezi servikal guatrla ayný olan bu hastalýkta bazý anatomik faktörler sebebiyle tiroid bezinin mediastene inmesi fizyopatolojiyi açýklamaktadýr. Lokalizasyonu itibariyle mediastinal majör vasküler yapýlara, trakea ve özefagusa yaptýðý basý semptomlarý SG’larýn semptom ve bulgularýna sebep olmaktadýr.
Ayrýca SG’larýn olasý malignite riski ve hipertiroidi ile iliþkisi uzun zamandýr tartýþýlmakta ve yapýlacak tedavi endikasyonlarýný ve yöntemini etkilemektedir.
Biz de bu çalýþmamýzda literatürde güncelliðini koruyan SG’larýn tanýmlarý, sýnýflandýrmasý, fizyopatolojisi, taný için kullanýlan laboratuvar ve görüntüleme yöntemlerini, SG’ýn hipertiroidi ve malignite ile iliþkisini ve kýsaca tedavi yöntemlerini güncel literatür bilgileri ýþýðýnda deðerlendirmeyi amaçladýk. (SETB-2022-06-162)

The enlargement of multinodular goiter into the mediastinum through the thoracic inlet or ectopic thyroid tissues directly in the mediastinum is defined as Substernal Goiter (SG). However, there is no clear consensus in the literature on this definition. There are many definitions for SG in the literature. Most definitions are similar or overlapping. Since the thyroid is located in the neck above the thoracic inlet in its normal anatomical position, the simplest clinical definition should be preferred among the definitions regarding its descent below the thoracic inlet and adjacent to the mediastinal organs. In the American Thyroid Association guideline, SG is defined as clinical or radiological protrusion of the thyroid gland over the sternal notch or clavicle in a patient with a slightly extended neck in the supine position. SGs can be classified as primary or secondary according to their origins. In addition, there are combined SGs resulting from the enlargement of the primary SG, which is the growth of the cervical thyroid gland toward the mediastinum, and the secondary SG, which is defined as an ectopic mediastinal mass, together. We find it appropriate to define such SGs as mixed SGs. In this disease, which has the same etiology and etiopathogenesis as cervical goiter, the descent of the thyroid gland into the mediastinum due to some anatomical factors explains the physiopathology. Compression symptoms of mediastinal major vascular structures, trachea, and esophagus cause the symptoms and findings of SGs due to its localization. In addition, the relationship of SGs with possible malignancy risk and hyperthyroidism affecting the indications and methods of treatment has been discussed for a long time. In this study, we aimed to evaluate the definitions, classification, physiopathology, laboratory and imaging methods used for diagnosis, the relationship of SG with hyperthyroidism and malignancy, and briefly the treatment methods, according to the current studies from literature.

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Transkateter aort implantasyonu uygun aort kapaðý belirlenerek deðiþimi gerçekleþtirilen kapalý cerrahi bir iþlemdir. Aort kökü, aort kapaðý ölçümleri bunlarýn dýþýnda aort aðacýnýn, koroner damarlarýn, femoral, subklaviyan arterlerin ölçümleri kritik öneme sahiptir. TAVI iþleminde hastaya yerleþtirilecek kapaðýn boyutlarý Bilgisayarlý Tomografi yöntemi ile belirlenir. Koroner skorlamasý ve koroner arterlerin TAVI görüntülemesine dahil edilmesi için uygun protokoller seçilmeli ve çekimden sonra koroner arterlerin Curved MPR, VRT gibi görüntüleri iþlenmeli ve bu görüntüler iþlemi yapacak hekime rehberlik edecek þekilde hazýrlanmalýdýr. Görüntülemede kullanýlacak cihaz, en az 64 MCDT kesitli bir tomografi cihazý olmalýdýr. EKG tetikleme kullanan bu çekimler için iki yöntem vardýr. Bu yöntemler þunlardýr: Retrospektif Tarama ve Prospektif taramadýr. TAVI görüntüleme için Bolus Ýzleme yöntemi, kullanýlabilecek en doðru kontrast verme yöntemlerinden biridir. Otomatik doz kalibrasyonu kullanýlýr. Yöntemin günden güne baþarýsý ile hekimlere yöntem sýrasýnda rehberlik eden "Bilgisayarlý Tomografi de TAVI’nin” önemi artmýþtýr. (SETB-2022-03-060)

Transcatheter aortic valve implantation is a procedure in the context of non-suitable for open surgery. Measurements of aortic root width, aortic valve surface area, and measurements of the aortic tree, coronary vessels, femoral, and subclavian arteries are of critical importance. In the TAVI procedure, the dimensions of the valve to be placed on the patient are determined by the computed tomography method. Appropriate protocols should be selected for coronary scoring and inclusion of coronary arteries in TAVI imaging and after the shooting, images of coronary arteries such as curved MPR and VRT should be processed, and these images should be prepared to guide the physician who will perform the procedure. The device to be used in imaging must be a tomography device with at least 64 MCDT sections. There are two methods for these shots using ECG triggering. These methods are as follows: Retrospective scan and prospective scan. Bolus tracking method for TAVI imaging is one of the most accurate contrast giving methods that can be used. Automatic dose calibration is used. With the success of the method day by day, the importance of “Computerized Tomography TAVI,” which guides physicians during the method, has increase.

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Amaç: SYNTAX II skoru’nun (SS-II) prognostik önemi kronik koroner sendromlu hastalarda iyi bilinmektedir. Bununla birlikte, primer perkütan koroner giriþim (PKG) uygulanan ST-segment yükselmeli miyokard enfarktüsü (STYMI) hastalarýnda mortaliteyi öngörme yeteneði belirsizliðini koruyor. Bu nedenle, primer PKG uygulanan STYMI hastalarýnda SS-II'nin prognostik doðruluðunu araþtýrmayý amaçladýk.
Yöntemler: Primer PKG ile tedavi edilen 743 STYME hastasý geriye dönük olarak analiz edildi. Çalýþma popülasyonu SS-II'ye göre üç gruba ayrýldý ve SS-IIDÜÞÜK ≤ 22,5 (n = 245), 22,5 < SS-IIORTA ≤ 31 (n = 243) ve SS-IIYÜKSEK > 31 (n = 255) olarak tanýmlandý. Hastane içi ve uzun süreli mortalite, çalýþmanýn klinik sonlaným noktalarý olarak tanýmlandý.
Bulgular: Hastane içi mortalite (%15 - %0,4 - %0,8, p<0,001) ve takip sýrasýnda tüm nedenlere baðlý ölüm (%32,2 - %6,6 - 2, %9, p<0,001) SS-IIYÜKSEK grubunda, diðer iki grupla karþýlaþtýrýldýðýnda anlamlý olarak daha yüksekti. Ayrýca Kaplan-Meier analizi, SS-II > 31 grubunun önemli ölçüde daha yüksek ölüm insidansýna sahip olduðunu gösterdi (p [log-rank] < 0.001). SS-II > 31, tüm nedenlere baðlý mortalitenin baðýmsýz bir öngördürücüsü olarak tanýmlandý (Hazard oraný 5,22 %95 güven aralýðý 2,11–12,87 p<0,001). Tüm nedenlere baðlý ölüm için SS-II, anatomik ve lojistik klinik SS, modifiye Yaþ-Kreatinin-Ejeksiyon Fraksiyonu (ACEF) skoru ve GRACE skorlarýnýn ROC analizinden elde edilen eðri altýnda kalan alan deðerleri sýrasýyla 0.82, 0.71, 0.81, 0.82 ve 0.82 idi. (tümü için p < 0,001)
Sonuç: SS-II’nin, p-PKG uygulanan STYME hastalarýnda hastane içi ve uzun dönem mortalite için artan bir tahmin yeteneðine sahiptir. (SETB-2021-11-322)

Objectives: The prognostic significance of SYNTAX Score II (SS-II) is well-known in patients with chronic coronary syndromes. However, its predictive ability for mortality in ST-segment elevation myocardial infarction (STEMI) patients undergoing primary percutaneous coronary intervention (p-PCI) remains unclear. Therefore, we aimed to investigate the prognostic accuracy of SS-II in STEMI patients who underwent p-PCI.
Methods: A total of 743 STEMI patients treated with p-PCI were retrospectively analyzed. Study population was divided into three groups according to SS-II and defined as SS-IILOW ≤22.5 (n=245), 22.5 <SS-II MID ≤31 (n=243) and SS-II HIGH >31 (n=255). In-hospital and long-term mortality at long-term follow-up were defined as clinical endpoints of the study.
Results: The incidence of in-hospital (15% vs. 0.4% vs. 0.8%, p<0.001) and all-cause mortality (32.2% vs. 6.6% vs. 2.9%, p<0.001) were significantly higher in SS-IIHIGH group compared with the other two groups. In addition, Kaplan–Meier analysis showed statistically significantly increased incidence of death in SS-II > 31 group (P [log-rank] <0.001). SS-II >31 was defined as an independent predictor of all-cause mortality (hazard ratio 5.22 95% confidence interval 2.11–12.87 p<0.001). Area under the curve values derived from ROC analysis to evaluate the predictive accuracy of SS-II, anatomical and clinical SS, modified ACEF score, and Global Registry of Acute Coronary Events risk scores for all-cause mortality were 0.82, 0.71, 0.81, 0.82, and 0.82, respectively (p<0.001).
Conclusion: SS-II has an increased predictive ability for in-hospital and long-term mortality in STEMI patients undergoing p-PCI.

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Amaç: COVID-19 nedeni ile Yoðun bakým ünitesinde (YBÜ) yatarak favipiravir ve lopinavir-ritonavir ile tedavi edilen hastalarda YBÜ ve hastane mortalitesinin karþýlaþtýrýlmasýdýr.
Metot: 10 Mart ve 10 Mayýs 2020 arasýnda YBÜ’ye yatan COVID-19’lu hastalarýn verileri geçmiþe yönelik toplanmýþtýr. Favipiravir ve lopinavir-ritonavir alan hastalar arasýnda laboratuvar verileri, klinik özellikler, YBÜ ve hastane mortalitesi, YBÜ ve hastanede kalýþ süresi karþýlaþtýrýlmýþtýr.
Bulgular: Toplam 100 hastanýn verisi incelenmiþtir. Hastalarýn %85’inde favipiravir, geri kalanýnda lopinavir-ritonavir kullanmýþtýr. Her iki grubun klinik ve laboratuvar verileri benzerdir. Favipiravir alan hastalarýn hastane yatýþ süresi 16 (9-24) gün iken lopinavir-ritonavir alanlarýnki 8.5 (5-12.5) gündür (p=0.002). YBÜ yatýþ süresi favipiravir ve lopinavir-ritonavir alan hastalarda sýrasý ile 8 (5-15) ve 4 (3-9) gündür (p=0.011). YBÜ mortalitesi favipiravir için %65.9, lopinavir-ritonavir için %80’dir (p=0.002). Hastane mortalitesi favipiravir ve lopinavir-ritonavir için sýrasý ile % 67.1 ve % 80’dir (p=0.001).
Sonuç: Favipiravir ile tedavi edilen hastalarýn mortalitesi lopinavir-ritonavir ile tedavi edilenlerden azdýr. Favipiravirin etkisinden emin olunabilmesi için daha fazla çalýþmaya ihtiyaç vardýr. (SETB-2021-07-212)

Objectives: The aim of this study was to compare intensive care unit (ICU) and overall hospital mortality in patients treated with favipiravir and lopinavir-ritonavir for COVID-19.
Methods: Data were collected retrospectively between March 10 and May 10, 2020, from patients’ records admitted to ICU due to COVID-19. Laboratory data, clinical characteristics, ICU and hospital mortality, ICU and hospital length of stay were compared in patients treated with favipiravir and lopinavir-ritonavir.
Results: A total of 100 patients’ data were investigated. Favipiravir was used as the treatment for 85% of patients, with the rest treated with lopinavir-ritonavir. Clinical and laboratory data of both antiviral treatment groups were similar. Length of hospital stay was 16 (9–24) days with favipiravir and 8.5 (5–12.5) days with lopinavir-ritonavir (p=0.002). Length of ICU stay for favipiravir and lopinavir-ritonavir groups were 8 (5–15) days and 4 (3–9) days, respectively (p=0.011). ICU mortality was 65.9% for the favipiravir and 80% for lopinavir-ritonavir (p=0.002). Hospital mortality for favipiravir and lopinavir-ritonavir was 67.1% and 80%, respectively (p=0.001).
Conclusion: The mortality in patients treated with favipiravir was less than patients treated with lopinavir-ritonavir. Favipiravir needs more attention and trials for its effect to be confirmed.

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Amaç: Yüzeyel optik disk druseni (ODD) olan eriþkin hastalar ile saðlýklý eriþkinler arasýnda, optik koherens tomografi anjiyografi (OKT-A) ile deðerlendirilen peripapiller ve makuler vasküler akým deðiþikliklerini karþýlaþtýrmak.
Gereç ve Yöntemler: Bu retrospektif çalýþmaya nörooftalmoloji birimimizde takip edilen 22 yüzeyel ODD hastasýnýn 44 gözü ve rutin oftalmolojik muayene için kliniðimize baþvuran 27 saðlýklý bireyin 54 gözü dahil edildi. Üst, alt, nazal ve temporal radyal peripapiller kapiller pleksus (RPK) damar yoðunluðu (DY) deðerleri; üst, alt, nazal ve temporal parafoveal ve foveal bölge yüzeyel kapiller pleksus (YKP) ve derin kapiller pleksus (DKP) DY deðerleri; koryokapillaris akým alaný ve foveal avasküler zon (FAZ) alaný OKT-A ile deðerlendirildi.
Bulgular: Gruplar arasýnda yaþ ve cinsiyet açýsýndan istatistiksel olarak anlamlý fark yoktu. ODD'li olgularda temporal RPK DY deðerlerinde anlamlý bir azalma olurken (p = 0.02), diðer kadranlarda anlamlý bir fark saptanmadý. Gruplar arasýnda parafoveal bölge 4 kadran, foveal YKP ve DKP DY deðerlerinde veya foveal avasküler zon ölçümlerinde anlamlý farklýlýk bulunmazken, koryokapillaris akým alaný deðerleri çalýþma grubunda anlamlý olarak yüksekti (p = 0,012).
Sonuçlar: Sonuçlarýmýz, yüzeyel ODD'li olgularda RPK temporal kadran DY deðerlerinde azalma ve koryokapillaris akým alanýnda artýþ olduðunu gösterdi. ODD'nin optik sinir baþý ve makula perfüzyonu üzerindeki etkilerini deðerlendirmek ve altta yatan mekanizmalarý ve ikincil komplikasyonlarý anlamak için daha geniþ vaka serileri ile daha uzun takip süreli çalýþmalara ihtiyaç vardýr. (SETB-2021-05-139)

Objectives: The purpose of this study was to compare the peripapillary and macular vascular flow changes between healthy adults and adult patients with superficial optic disk drusen (ODD), as evaluated by optical coherence tomography angiography (OCT-A).
Methods: In this retrospective study, 44 eyes of 22 patients with superficial ODD followed-up in our neuro-ophthalmology unit and 54 eyes of 27 healthy individuals admitted to our clinic for routine ophthalmological examination were included in the study. The superior, inferior, nasal, and temporal radial peripapillary capillary plexus (RPC) vessel density (VD) values; superior, inferior, nasal, and temporal parafoveal and foveal region superficial capillary plexus (SCP) and deep capillary plexus (DCP) VD values; and choriocapillaris flow (CCF) area and foveal avascular zone area were evaluated by OCT-A.
Results: There was no statistically significant difference between groups in terms of age and sex. While there was a significant decrease in the temporal RPC VD values in cases with ODD (p=0.02), no significant difference was observed in other quadrants. No significant differences in either parafoveal zone four quadrant, foveal SCP and DCP VD values, or foveal avascular zone measurements were found between groups, while CCF area values were significantly higher in the study group (p=0.012).
Conclusion: Compared to healthy controls, our results showed a decrease in the RPC temporal quadrant VD values, and an increase in CCF area in cases with superficial ODD. To evaluate the effects of ODD on optic nerve head and macular perfusion, and to understand its underlying mechanisms and secondary complications, longer follow-up studies with larger case series are needed.

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Amaç: Audiovestibuler semptomlar nedeniyle tetkik edilen hastalarýn çok azýnda bu semptomlarla iliþkili retrokoklear bir patoloji saptanýr. Bu hastalarýn, internal akustik kanal manyetik rezonans görüntülemelerinin (IAK-MRG) çoðu ya tamamen normaldir ya da eþlik eden rastlantýsal bulgularý (RB) içerir. Çalýþmamýzýn amacý, IAK-MRG'lerde, olasý retrokoklear lezyonlarla birlikte RB’lerin varlýðýný ve sýklýðýný ortaya koymak ve bu RB'lerin klinik önemini vurgulayarak, saptandýklarýnda nasýl bir klinik yaklaþýmda bulunulmasý gerektiðini tartýþmaktýr.
Yöntem: 374 olgunun IAK-MRG tetkiklerinin retrospektif olarak incelenmesiyle gerçekleþtirilmiþtir.
Bulgular: Cinsiyet daðýlýmý: 201 erkek, 173 kadýn. Yaþ aralýðý: 2-82 yýl. Yetmiþ iki olgunun MRG tetkiki (%19.25) tamamen normaldi. On beþ olguda (%4.01) sadece pontoserebellar açý bulgularý (PSAB) saptandý. Elli yedi olguda (%15.24) PSAB ve RB varlýðý birlikteydi. MRG’lerin 230'unda (%61.50) sadece RB mevcuttu. Tüm IAK-MRG'lerde RB insidansý %76.74 idi (374 olgunun 287'sinde). Otuz dört olguda (%9.09) klinik ileri deðerlendirme ve inceleme gerektirebilecek kritik bulgular mevcuttu. Olgularýn 253’ünde (%67.65) ise, daha ileri inceleme gerektirmeyen RB'ler gözlendi.
Sonuç: IAK-MRG ile, PSAB’ye benzer semptomlara yol açabilecek, çok çeþitli RB’ler saptanabilir. Ve ne yazýk ki bu RB’lerin bazýlarý ciddi klinik önem arz edebilir. Hastalarýndan IAK-MRI tetkiki isteyen tüm klinisyen hekimler, bu audiovestibuler semptomlarýn, iþitsel yolun herhangi bir yerinde, RB olarak ortaya çýkabileceðini ve klinik yaklaþýmda nasýl bir yol izlenmesi gerektiðini iyi bilmelidir. (SETB-2021-06-192)

Objectives: Retrocochlear pathology associated with audiovestibular symptoms is detected in very few of the patients, and most of the internal acoustic canal magnetic resonance imaging examinations (IAC-MRIs) are either completely normal or include accompanying incidental findings (IFs). The aim of our study is to reveal the presence and frequency of IFs in IAC-MRIs, together with retrocochlear lesions. In addition, we intend to emphasize the clinical importance of these IFs.
Methods: A retrospective analysis of 374 serial IAC-MRI scans.
Results: Gender distribution: 201 males and 173 females. Age range: 2–82 years. Seventy-two scans (19.25%) were totally normal. Fifteen scans (4.01%) demonstrated only pontocerebellar angle findings (PCAFs). The presence of PCAF and IF was together in 57 scans (15.24%). In 230 (61.50%) of the scans, only IFs were present. The incidence of IFs in all IAC-MRIs was 76.74% (287 of 374). Critical findings that may require clinical further evaluation and examination were present in 34 scans (9.09%). IFs that did not require further examination were observed in 253 scans (67.65%).
Conclusion: Various IFs can be detected with IAC-MRI that may cause similar symptoms with PCAF. And unfortunately, some of these IFs may be of very high clinical importance. All referral clinicians should know well that these audiovestibular symptoms can appear as IFs anywhere in the auditory pathway, and how they should be followed in their clinical approach.

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Amaç. Total diz artroplastisinde kullanýlan metal yüzeyli Kobalt-Krom içerikli protezlerin iþitme ve kulak çýnlamasý üzerine uzun dönemli etkilerini belirlemeyi amaçladýk.
Yöntem. 5 yýldan uzun süredir Kobalt-Krom içeren total diz protezi kullanan 44 hasta ve benzer demografik özelliklere sahip implant uygulanmamýþ 44 hasta olmak üzere otoskopik muayenesi ve kan B12 düzeyi normal olan toplam 88 hasta, rastgele seçilmiþ ve çalýþmaya dahil edilmiþtir. Tüm katýlýmcýlar saf ses odyometrisi, distorsiyon product otoakustik emisyon (DPOAE), tinnitus handikap envanteri ve tinnitus reaksiyon anketi ile deðerlendirildi ve iki grup karþýlaþtýrýldý.
Bulgular. 88 hastanýn yaþ ortalamasý 67,22±10,4 yýl, en az 5 yýl önce total diz artroplastisi uygulanan 44 hastanýn yaþ ortalamasý 72.89±7.18 yýl idi. Bu hastalarýn %75'inde bilateral protez vardý ve ortalama protez süresi 11.00±5.08 yýl (5-25 arasý) idi. Protezli ve protezsiz hasta gruplarý karþýlaþtýrýldýðýnda, iþitme ve kulak çýnlamasý açýsýndan iki grup arasýnda anlamlý bir fark saptanmadý. (p>0.05).
Sonuç. Çalýþmamýzda metal yüzeyli Kobalt-Krom total diz protezlerinin uzun süreli kullanýmýnýn sanýlanýn aksine ototoksik etkisinin olmadýðýný gözlemledik. Ayrýca protez süresinin uzamasýnýn veya bilateral cerrahi geçirmenin iþitme ve kulak çýnlamasýný kötüleþtirmediðini bulduk. (SETB-2022-04-097)

Objectives: We aimed to determine the long-term effects of metal-on-metal (MoM) cobalt (Co)-chromium-containing prostheses used in total knee arthroplasty (TKA) on hearing and tinnitus.
Methods: A total of 88 patients with the normal otoscopic examination and normal blood B12 levels, consisting of 44 patients who had been using MoM Co-chromium-containing total knee prosthesis for more than 5 years, and 44 non-implanted patients with similar demographic characteristics, were randomly selected and included in the study. Patients with previous ear surgery, chronic middle ear disease, or conductive hearing loss were excluded from the study. All participants were evaluated with pure-tone audiometry (PTA), distortion product otoacoustic emission (DPOAE), tinnitus handicap inventory (THI), and tinnitus reaction questionnaire (TRQ).
Results: The mean age of the 88 patients was 67.22±10.4 years. The mean age of 44 patients who underwent TKA at least 5 years ago was 72.89±7.18 years, 75% of these patients had bilateral prostheses, and the mean prosthesis duration was 11.00±5.08 years (range 5–25). The two groups were compared statistically using PTA, DPOAE, THI, and TRQ scores. When the patient groups with and without prostheses were compared in terms of hearing and tinnitus, we did not find a significant difference between the two groups (p>0.05).
Conclusion: In our study, we observed that the long-term use of MoM Co-chromium total knee prostheses did not have an ototoxic effect, contrary to what was believed. We also found that prolonged prosthesis duration or undergoing bilateral surgery did not worsen hearing and tinnitus.

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Amaç: Jinekolojik cerrahide pleth deðiþkenlik indeksine (PVI) dayalý hedefe yönelik sývý yönetiminin (GDFM) yararlýlýðýna iliþkin veriler eksiktir. Bu çalýþmanýn amacý, PVI (pleth deðiþkenlik indeksi) temelli hedefe yönelik sývý yönetimi (GDFM) ile geleneksel sývý yönetiminin (CFM) jinekolojik cerrahi geçiren hastalarda intraoperatif hemodinamik deðiþiklikler ve laktat düzeyleri üzerindeki etkisini karþýlaþtýrmaktýr.
Gereç ve Yöntemler: Bu randomize, kontrollü çalýþma, elektif jinekolojik cerrahi geçiren 70 hasta üzerinde gerçekleþtirildi. Denekler rastgele olarak CFM veya GDFM'ye atandý. Hemodinamik veriler ve arteriyel kan gazý analizinin sonuçlarý ve infüze edilen toplam sývý miktarý ameliyat boyunca birer saatlik aralýklarla kaydedildi.
Bulgular: Toplam sývý miktarý, GDFM grubuna göre CFM grubunda anlamlý olarak daha yüksekti (p <0.001). Cerrahinin 2. saatinde kaydedilen ortalama arter basýncý, GDFM grubuna göre CFM grubunda anlamlý derecede düþüktü (p = 0.047). GDFM grubunda bazal ve 2. saat laktat seviyeleri arasýnda anlamlý bir fark bulunmazken, CFM grubunda laktat seviyesi baþlangýçtan 2. saate anlamlý olarak arttý (p = 0.010).
Sonuçlar: Jinekolojik cerrahi geçiren hastalarda PVI tabanlý GDFM uygulamasý, CFM'ye göre daha iyi intraoperatif hemodinamik stabilite ve daha düþük laktat seviyeleri saðlar. (SETB-2021-01-012)

Objectives: Data concerning the usefulness of pleth variability index (PVI)-based goal-directed fluid management (GDFM) in gynecologic surgery is limited.
This study purposed to compare the impact of PVI-based GDFM to conventional fluid management (CFM) on intraoperative hemodynamics and lactate levels in subjects undergoing gynecologic surgery.
Methods: This randomized and controlled trial was conducted on 70 patients undergoing elective gynecologic surgery. Subjects were randomly assigned to CFM or GDFM. Hemodynamic data and results of the arterial blood gas analysis, and total amount of the fluid infused were recorded throughout the surgery at 1-h intervals.
Results: The amount of the total fluids was significantly higher in the CFM group compared to that of the GDFM group (p<0.001). Mean arterial pressure recorded at the 2nd h of the surgery was significantly lower in the CFM group compared to that of the GDFM group (p=0.047). While there were no significant differences between the baseline and the 2nd h lactate levels in the GDFM group, the lactate level significantly increased from baseline to the 2nd h in the CFM group (p=0.010).
Conclusion: Implementation of PVI-based GDFM provides better intraoperative hemodynamic stability and lower lactate levels compared to the CFM in subjects undergoing gynecologic surgery.

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Giriþ: Bu çalýþmada, kendi merkezimizde canlýdan ve kadavradan yapýlan karaciðer nakillerinde bilyer komplikasyonlarý etkileyen cerrahi teknik ve biyokimyasal parametreleri araþtýrmayý amaçladýk.
Yöntem: Bu çalýþmaya, Ocak 2018 - Ocak 2020 tarihleri arasýnda XXX Hastanesi organ nakli merkezinde karaciðer nakli yapýlan 141 hasta dahil edildi. Hastalar, 12-24 ay boyunca izlendi. Bu çalýþmamýzda, hastalar geriye dönük olarak incelendi. Safra yolu komplikasyonlarýna neden olabilecek faktörler ve komplikasyonlara yönelik tedavi modaliteleri incelendi.
Bulgular: Bu çalýþmada, 124 canlý donörden ve 17 kadavradan karaciðer nakil iþlemi gerçekleþti. Yirmi üç hasta 18 yaþýn altýndaydý. Toplam 39 safra komplikasyonunun sadece yedisi ameliyat edildi. Safra komplikasyonu olan hastalarda sað greft bulunma oraný daha yüksekti (p < 0,05). Sol, sol lateral ve tüm greft varlýðý açýsýndan gruplar arasýnda istatistiksel olarak anlamlý fark yoktu (sýrasýyla p = 0,561, p = 0,172 ve p = 0,057). Biliyer komplikasyon grubunda çift biliyer anastomoz daha yüksek bulundu ancak istatistiksel olarak anlamlý bir fark yoktu (p = 0,086).
Sonuç: Biliyer komplikasyonlar, özellikle sað lobdan alýnan karaciðer nakillerinde yaygýndýr. Erken teþhis ve uygun tedavi yaklaþýmý ile önemli hasta saðkalýmý saðlanabilir. (SETB-2021-06-184)

Objectives: In this study, we aimed to investigate the surgical technique and biochemical parameters that affect biliary complications in liver transplants from live and cadaver in our center.
Methods: In this study, 141 patients who underwent liver transplants at Istanbul Yeni Yüzyýl Universty Gaziosmanpaþa Hospital organ transplant center between January 2018 and January 2020 were included in the study. The patients were monitored for 12–24 months. The patients included in the present study were examined retrospectively. Factors that may cause biliary tract complications and treatment modalities for complications were examined.
Results: In this study, liver transplantation from 124 living donors and 17 cadavers was performed. Twenty-three patients were under the age of 18. Only seven of 39 biliary complications were operated on. The rate of finding the right graft in patients with biliary complications was higher (p<0.05). There was no statistically significant difference between the groups concerning left, left lateral, and whole graft presence (p=0.561, p=0.172, and p=0.057, respectively). Double biliary anastomosis was to be higher in the biliary complication group, but there was no statistically significant difference (p=0.086).
Conclusion: Biliary complications are common, especially in liver transplants taken from the right lobe. Significant patient survival can be achieved with an early diagnosis and an appropriate treatment approach.

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Amaç: Minimal invaziv cerrahide deneyimli kliniklerde, kýz çocuklarýnda kasýk fýtýðý (KF) tedavisinde “perkütan internal ring suturing (PIRS)” tekniði uygulamasý artmaktadýr. Bizim çalýþmamýzýn amacý; kýz çocuklarýnda KF tedavisinde, laparoskopik yapýlan PIRS prosedürü ile açýk yöntemin sonuçlarýnýn karþýlaþtýrýlmasý.
Metod: Çalýþmamýzda 2012-2017 yýllarý arasýnda KF nedeniyle opere edilen kýz hastalarýn sonuçlarýný geriye dönük olarak deðerlendirildi. Ýstatistiksel deðerlendirme yapýldý.
Bulgular: KF nedeniyle 293 kýz çocuðu kliniðimizde opere edildi. PIRS prosedürü 164 hastaya uygulandý ve grup 1 olarak isimlendirildi. Grup 1’in 73’üne sað, 26’sýna sol, 65’ine (%39.6) iki yanlý KF onarýmý yapýldý. Bu 65 hastanýn, ameliyat öncesi deðerlendirmesinde 16’sýnda sadece sað 11’ine de sol KF mevcuttu. Açýk cerrahi 129 hastaya uygulandý ve grup 2 olarak isimlendirildi. 78’ inde sað, 38’ inde sol ve 13’ ünde (%10) iki yanlý KF mevcuttu. Grup 1 de 7, grup 2 de 21 hastada fallop tüp kaymasý mevcuttu (p<0.001). Over kaymasý grup 1 de 7, grup 2 de 16 hastada mevcuttu (p<0.015). Fýtýk tekrarý grup 1 de bir hastada geliþti (p>0.05).
Sonuçlar: PIRS yapýlýrken yapýlan laparoskopik deðerlendirmede yüksek oranda iki taraflý kasýk fýtýðý tanýsý koyma ihtimali halen bu yöntemin bir problemi, ama çalýþmamýzda iki taraflý PIRS uygulamasý sonucu komplikasyonlarda artýþ geliþmedi. Açýk ameliyatta yüksek olan over kaymasýndaki artýþ, PIRS yönteminde görülmedi, bunun sebebi açýk ameliyatta fýtýk kesesinin çekilmesine baðlý olabilir. PIRS yöntemi yapýlýrken, iç inguinal kanal çevresinde geliþen hematom, ameliyatýn laparoskopik bitirilmesi için ciddi bir problem. Çalýþmamýzda cerrahlarýn seçiminin PIRS yönünde olduðu da görüldü. Ek olarak, PIRS yaklaþýmý iç genital organlar için daha koruyucu yöntem olarak görünüyor ama bu konuda yeni çalýþmalarýn yapýlmasý gerekli. (SETB-2021-06-164)

Objectives: Percutaneous internal ring suturing (PIRS) method for inguinal hernia (IH) repair in girls is increasingly used in clinics especially experienced in minimal invasive surgery. We aimed to evaluate and compare our results of laparoscopic PIRS and the open procedure for IH repair in girls in our series.
Methods: We retrospectively evaluated female patients in our pediatric surgery clinic who underwent IH surgery between 2012 and 2017 and results were assessed statistically.
Results: We identified 293 girls operated in our clinic with IH. In 164 of them, PIRS procedure (group 1) was performed; 73 had right, 26 had left, and 65 (39.6%) had bilateral IH. Among these 65 patients, 16 had only right and 11 had only left IH according to preoperative examination, which turned out to be bilateral during laparoscopy. In the open surgery group (Group II), there were 129 patients. Seventy-eight patients had right, 38 had left, and 13 (10%) had bilateral IH. Sliding fallo-pian tubes were present in seven of Group I versus 21 of group II (p<0.001), while sliding ovaries were present in seven of Group I versus 16 of Group II (p=0.015). Recurrence was observed in only one patient in Group I (p>0.05).
Conclusion: The higher rate of bilaterality is still the problem for laparoscopic procedures. Despite over-diagnosis, no more complications were not detected due to PIRS. High ratio of sliding tubes and ovaries can be due to traction for preparing the sac in open surgery. Hematoma can be a serious problem to complete PIRS procedure. Preferred technique from the surgeon’s point of view is in favor of PIRS procedure. In addition, PIRS pro-cedure can be more protective for internal genitalia, but more studies with higher numbers and longer follow-up period are needed.

Abstract | English Full Text

Amaç: Pankreatikoduodenektomi (PD) diseksiyon bölgelerinin zorlayýcý olmasý ve kompleks rekonstrüksiyon ihtiyacý nedeni ile gastrointestinal sistem cerrahisinin en zorlu operasyonlarýndan birisidir. Bu çalýþmanýn amacý minimal invaziv PD için öðrenme periyodundan olduðumuz vakalar ile benzer fistül risk skoruna (FRS) sahip açýk PD yaptýðýmýz vakalarýn morbidite, POPF ve mortalite oranlarý karþýlaþtýrmaktýr.
Yöntem: Benzer; yaþ, ASA skoru, ameliyat öncesi drenaj ve FRS'ye sahip hastalar çalýþmaya dahil edildi. Çalýþmaya 48'i açýk cerrahi, 23'ü minimal invaziv yöntemlerle ameliyat edilen toplam 71 hasta dahil edildi.
Bulgular: Yapýlan istatistiksel analizde açýk cerrahi ile minimal invaziv cerrahi yapýlan grup arasýnda yaþ, cinsiyet, ASA skoru, preoperatif drenaj yapýlmasý, pankreas kývamý, pankreas kaçaðýnýn tedavisi açýsýndan istatistiksel anlanmlý fark saptanmamýþtýr. (Sýrasýyla;p= 0.27, p=0.09, p=0.4, p=0.39, p=0.76 ve p=0.36). Klinik iliþkili pankreas anastomoz kaçaðý( grade-B ve grade-C fistül) açýsýndan iki grup arasýnda istatistiksel anlamlý fark saptanmýþ olup (p=0.11) minimal invaziv cerrahi grubunda daha fazla hastada Grade-BL ve B kaçak görülürken Grade-C fistül hiçbir hastada izlenmemiþtir(p=0.002). Ancak pankreas kaçaðýnýn yönetimi ve buna baðlý morbidite açýsýndan iki grup arasýnda istatistiksel olarak anlamlý fark saptanmamýþtýr (p=0.36). Tümör çapý, çýkarýlan lenf nodu sayýsý, fistül risk skoru, peroperatif kanama miktarý, wirsung veya koledok çapý açýsýndan da iki grup arasýnda anlamlý fark saptanmamýþtýr (sýrasýyla, p=0.15, p=0.20, p=0.145, p=0.80 ve p=0.073)
Operasyon süresine bakýldýðýnda minimal invaziv cerrahi tedavisi uygulanan hastalarda operasyon süresinin daha uzun olduðu ve bunun istatistiksel olarak anlamlý olduðu saptanmýþtýr (p<0.0001).
Sonuç: Sonuç olarak öðrenme aþamasýnda MIPD ile benzer FRS’ye sahip olan OPD karþýlaþtýrýlmasýnda benzer ciddi morbidite ve kabul edilebilir CR-POPF ile gerçekleþtirilebilineceðine inanmaktayýz. (SETB-2021-06-172)

Objectives: Pancreaticoduodenectomy (PD) is one of the most challenging operations in gastrointestinal system due to the difficulty of dissection areas and the need for complex reconstruction. The aim of this study is to compare the morbidity, post-operative pancreatic fistula (POPF), and mortality rates of the cases we have from the learning period for minimally invasive PD and our previous open PD cases with similar fistula risk scores (FRSs).
Methods: Patients with similar age, ASA score, pre-operative drainage, and FRS were included in the study. A total of 71 patients, 48 of whom were operated with open surgery and 23 with minimally invasive methods, were included in the study.
Results: When the statistical analysis performed, no statistically significant difference was found between open surgery and minimally invasive surgery groups in terms of age, gender, ASA score, pre-operative drainage, pancreatic texture, and treatment of pancreatic leakage (p=0.27, p=0.09, p=0.4, p=0.39, p=0.76, and p=0.36, respectively). There was a statistically significant difference between two groups in terms of clinically relevant pancreatic anastomotic leakage (Grade-B and Grade-C fistula) (p=0.11). The rate of Grade-BL and B leakage was higher in the minimally invasive surgery group, while Grade-C fistula was not observed in any patient (p=0.002). However, there was no statistically significant difference between the two groups in terms of the management of pancreatic leakage and related morbidity (p=0.36). There was no significant difference between the two groups in terms of tumor size, number of lymph nodes removed, FRS, amount of intraoperative bleeding, and diameter of Wirsung and common bile duct (p=0.15, p=0.20, p=0.145, p=0.80, and p=0.073, respectively). Considering the operation time, it was found that the operation time was longer in patients who received minimally invasive surgical treatment and this was statistically significant (p<0.0001).
Conclusion: As a result, we believe that minimally invasive PD operation can be performed with similar morbidity and acceptable CR-POPF rates when compared with the open PD with similar FRS at the learning stage.

Abstract | English Full Text

AMAÇ: Feokromositoma tanýsýyla laparoskopik adrenalektomi yapýlan ≥5 cm ve <5 cm adrenal kitlesi olan hastalarýn sonuçlarýný karþýlaþtýrmayý amaçladýk.
YÖNTEM: Demografik deðiþkenler, tümör özellikleri, perioperatif ve postoperatif sonuçlar retrospektif olarak deðerlendirildi ve gruplar arasýnda karþýlaþtýrýldý.
BULGULAR: Þubat 2008 ve Aðustos 2020 tarihleri arasýnda 54 hasta (27 kadýn, 27 erkek) çalýþmaya dahil edildi ve tümör boyutuna göre grup L ≥5 cm (28 hasta) ve grup S <5 cm ( 26 hasta) olarak iki gruba ayrýldý. ASA skorlarý, vücut kitle indeksi, tümör lokasyonlarý, yaþlý (≥ 60 yaþ) hasta oraný ve cinsiyet oraný açýsýndan S ve L gruplarý benzerdi (p = 0,572, p = 0,516, p = 0,6, p = 0.331, p = 0.207, sýrasýyla). Ortalama ameliyat süresi (p = 0.266), ortalama tahmini kan kaybý (p = 0.587), ortalama hastanede kalýþ süresi (p = 0.374) gruplar arasýnda benzerdi. Maksimum ve preoperatif sistolik basýnç farký, maksimum ve preoperatif diyastolik basýnç farký, S ve L gruplarý arasýnda benzerdi (sýrasýyla, p = 0.852 ve p = 0.526). Sistolik kan basýncý> 160 mmHg, sistolik kan basýncý baþlangýc deðerine göre % 30 dan fazla artanlarýn oraný, Kalp hýzý> 110 olanlarýn oranlarý (p = 0,307, p = 0,609, p = 0,296) benzerdi. Diyastolik kan basýncý baþlangýç deðerine göre en az 10 epizotta % 30 dan fazla düþenlerin oranlarý L grubunda daha fazla olmakla birlikte istatistiksel olarak farklý deðildi (p = 0,077).
SONUÇ: Feokromasitoma hastalarýnýn hazýrlýðýnda endokrinologlar ve anestezistler ile koordineli olarak çalýþmalýdýr. Feokromasitoma tedavisinde laparoskopik adrenalektomi tümör boyutundan baðýmsýz olarak multidisipliner yaklaþým ile deneyimli merkezlerde uygulanmalýdýr. (SETB-2021-03-084)

Objectives: We aimed to compare the outcomes of patients who underwent laparoscopic adrenalectomy (LA) for pheochromacytoma (PHE) ≥5 cm versus <5 cm in diameter.
Methods: Demographic variables, tumor characteristics, perioperative, and post-operative outcomes were evaluated retrospectively and compared between groups.
Results: Between February 2008 and August 2020, 54 patients (27 female and 27 male) enrolled to the study and divided into two groups according to the tumor size as group L ≥5 cm (28 patients) and group S as <5 cm (26 patients). Groups compared in the aspect of American Society of Anesthesiologists scores, body mass index, tumor locations, ratio of elder (≥60-years-old) patients, and gender ratio were similar between groups S and L (p=0.572, p=0.516, p=0.6, p=0.331, and p=0.207, respectively). Mean duration of surgery (p=0.266), mean estimated blood loss (p=0.587), and mean length of hospital stay (p=0.374) were similar between groups. Difference between maximum and pre-operative systolic pressure and the difference between maximum and pre-operative diastolic pressure were similar between S and L groups (p=0.852 and p=0.526, respectively). Patients whose systolic blood pressure >160 mmHg, systolic blood pressure >30% of baseline, and heart rate >110 (p=0.307, p=0.609, and p=0.296) were similar. Diastolic blood pressure <30%, there is a difference between groups in favor of group L, but not statistically different (p=0.077).
Conclusion: It is necessary to work in coordination with endocrinologists and anesthesiologists and LA for PHE should be performed in experienced medical centers regardless of tumor size with multidisciplinary approach.

Abstract | English Full Text

Amaç: Eþlik eden tiroid hastalýðý, primer hiperparatiroidizm (PHPT) hastalarýnýn yaklaþýk olarak yarýsýný etkiler. PHPT hastalarýnda tiroid kanserinin erken teþhisi için tiroid bezinin preoperatif deðerlendirilmesi önemlidir. Bu çalýþmada, PHPT ve eþlik eden tiroid hastalýðý olan ancak özellikle tiroid kanseri olan hastalarda cerrahinin tipini ve yaygýnlýðýný etkileyen klinikopatolojik özellikleri araþtýrmayý amaçladýk.
Yöntem: Altý yýllýk bir süre içinde PHPT nedeniyle paratiroidektomi yapýlan ardýþýk hastalarýn dosyalarý geriye dönük olarak incelendi. Paratiroidektomi ve eþ zamanlý tiroidektomi yapýlan olgular çalýþmaya alýndý. Çalýþma kriterlerine uyan toplam 84 hasta son histopatolojik incelemeye göre benign tiroid hastalýðý (Grup 1) ve malign tiroid hastalýðý (Grup 2) olarak iki gruba ayrýldý. Gruplar arasýnda demografik ve klinikopatolojik özellikler karþýlaþtýrýldý.
Bulgular: PHPT nedeniyle ameliyat edilen 284 hastanýn 158'inde (%55,6) eþlik eden tiroid hastalýðý saptandý. 84 (%29,6) hastaya eþ zamanlý total tiroidektomi veya lobektomi yapýldý ve 29 (%10,2) hastada tiroid karsinomu tespit edildi. Total tiroidektomi ve komplikasyon oranlarý Grup 2'de daha yüksekti (p<0.05). Preoperatif ince iðne aspirasyon biyopsisi hastalarýn %58,3'ünde alýndý ve histopatolojik olarak doðrulanmýþ tiroid karsinomunun sadece %26,3'ünü tanýmladý. Grup 1'de sadece ameliyat öncesi serum fosfor düzeyi daha yüksek bulundu (p<0.05) ancak çok deðiþkenli analizde çalýþma parametrelerinin hiçbiri tiroid malignitesi için baðýmsýz bir risk faktörü olarak bulunmadý (p>0.05).
Sonuç: PHPT hastalarýnda eþlik eden tiroid karsinomunu öngörmek için henüz kabul edilmiþ bir parametre yoktur. Bununla birlikte, PHPT hastalarýnda benign biyopsi tiroid malignitesini dýþlayamaz ve papiller mikrokarsinom nedeniyle taný konmamýþ tiroid kanserine neden olabilir. (SETB-2022-01-028)

Objectives: Concomitant thyroid disease affects almost half of the primary hyperparathyroidism (PHPT) patients. Pre-operative evaluation of the thyroid gland for the early diagnosis of thyroid carcinoma is essential in PHPT patients. Herein, we aim to investigate the clinicopathologic features that affect the type and extent of surgery in patients having PHPT and concomitant thyroid disease but especially thyroid cancer.
Methods: The files of consecutive patients who underwent parathyroidectomy for PHPT during a 6-year period were retrospectively reviewed. The cases who underwent parathyroidectomy and simultaneous thyroidectomy were enrolled in the study. A total of 84 patients who met the study criteria were divided into two groups as benign thyroid disease (Group 1) and malignant thyroid disease (Group 2) according to the final histopathological examination. The demographic and clinicopathological characteristics were compared between groups.
Results: Concomitant thyroid disease was found in 158 (55.6%) of 284 patients who were operated on for PHPT. Simultaneous total thyroidectomy or lobectomy was performed for 84 (29.6%) patients and thyroid carcinoma was detected in 29 (10.2%) patients. Total thyroidectomy and complication rates were higher in Group 2 (p<0.05). Pre-operative fine-needle aspiration biopsy was obtained in 58.3% of patients and it identified only 26.3% of histopathologically confirmed thyroid carcinoma. Only pre-operative serum phosphorus level was found higher in Group 1 (p<0.05), but none of the study parameters was found as an independent risk factor for thyroid malignancy (p>0.05) in multivariate analysis.
Conclusion: There is no accepted parameter yet to predict the accompanying thyroid carcinoma in PHPT patients. However, a benign biopsy cannot exclude thyroid malignancy in PHPT patients and may cause undiagnosed thyroid carcinoma due to papillary microcarcinoma.

Abstract | English Full Text

Amaç: Primer hiperparatiroidi (PHPT) yaygýn bir endokrin hastalýktýr. Ektopik adenomlar, baþarýsýz bir cerrahi sonrasý persistans veya nükse neden olabilir. Ýntratiroidal paratiroid adenomu (ÝTPA), PHPT'nin nadir bir nedeni ve nadir bir ektopik odaktýr. Burada üçüncü basamak bir referans merkezinde ÝTPA’lu hastalarýn klinik ve görüntüleme özelliklerini ve lokalizasyon için radyolojik araçlarýn etkinliðini araþtýrmayý amaçladýk.
Yöntem: Ocak 2007-Aralýk 2021 tarihleri arasýnda kliniðimizde PHPT nedeniyle paratiroidektomi yapýlan ardýþýk 708 hastanýn dosyalarý retrospektif olarak incelendi. ÝTPA'lu PHPT hastalarý çalýþmaya dahil edildi. Sekonder veya tersiyer hiperparatiroidizmi olan ve verisi eksik olan hastalar çalýþma dýþý býrakýldý. Hastalarýn klinikopatolojik özellikleri ve radyolojik bulgularý araþtýrýldý.
Bulgular: Yirmi sekiz (28/708: %3,9) hasta çalýþmaya dahil edildi. Komplet intratiroidal bez ve subkapsüler paratiroid bezi sýrasýyla 8 (%1.1) ve 20 (%2.8) hastada görüldü. Paratiroid ultrasonu ve sintigrafisi, sýrasýyla 25 (%89.3) ve 18 (%64.3) hastada ÝTPA'nun doðru lokalizasyonunu belirledi. Konvansiyonel lokalizasyon çalýþmalarýnýn uyumsuz veya sonuçsuz olduðu 10 hasta için ek görüntüleme yöntemleri uygulandý. ÝTPA'larý en sýk alt bez (n=20) lokalizasyonunda bulundu. Tüm hastalara baþarýlý bir paratiroidektomi yapýldý ve çalýþma grubunda persistans veya nüks görülmedi.
Sonuç: ÝTPA, PHPT'nin nadir bir nedenidir. Deneyimli ellerde ultrasonografinin tanýsal deðeri yüksektir. Negatif veya uyumsuz lokalizasyon yöntemleri varlýðýnda, ikinci basamak görüntüleme yöntemleri uygun olabilir. Preoperatif lokalizasyon çalýþmalarý çoðu hastada ÝTPA’larý tespit edebilir, bu nedenle tanýsal tiroidektomiden kaçýnýlmalýdýr. (SETB-2022-03-075)

Objectives: Primary hyperparathyroidism (PHPT) is a common endocrine disease. Ectopic adenomas may cause a failed surgery which results in persistence or recurrence. Intrathyroidal parathyroid adenoma (ITPA) is a rare reason for PHPT and site of ectopia. Herein, we aimed to investigate the clinical and imaging features of patients with ITPAs and the effectiveness of radiological tools for localization at a tertiary reference center.
Methods: The files of 708 consecutive patients who underwent parathyroidectomy for PHPT in our department between January 2007 and December 2021 were investigated retrospectively. PHPT patients with ITPA were included in the study. Patients with missing data were excluded from the study. Clinicopathological features of the patients and radiological evaluation findings were investigated.
Results: Twenty-eight (28/708: 3.9%) patients were included in the study. The complete intrathyroidal gland and subcapsular parathyroid gland were observed in 8 (1.1%) and 20 (2.8%) patients, respectively. The ultrasound and parathyroid scintigraphy revealed the accurate localization of ITPA in 25 (89.3%) and 18 (64.3%) patients, respectively. Additional imaging modalities were applied for 10 patients in which conventional localization studies were discordant or inconclusive. ITPAs were most commonly found in the lower gland (n=20) localization. All patients had a successful parathyroidectomy and neither persistence nor recurrence was occurred in the study group.
Conclusion: The ITPAs are rare in PHPT. The ultrasound has a high diagnostic rate in experienced hands. The second-line imaging methods may be favorable in the presence of negative or discordant scans. The pre-operative localization studies can detect the ITPAs in most patients, so blind thyroidectomy should be avoided.

Abstract | English Full Text

Amaç: Total tiroidektominin en sýk komplikasyonu olan hipokalseminin etyolojisinde esas faktör hipoparatiroidizmdir. Postoperatif hipoparatiroidizmin etyolojisi multifaktöryel olup, literatürede hipoparatiroidizm üzerine etkili bazý faktörler ortaya koyulmuþ, bazý faktörlerle ilgili çeliþkili sonuçlar bulunmaktadýr. Bu çalýþmada hipoparatiroidizm geliþimini etkileyen preoperative ve intraoperative faktörleri deðerlendirmeyi amaçladýk.
Gereç ve Yöntem: 2012-2020 tarihleri arasýnda total tiroidektomi ± santral diseksiyon (±lateral diseksiyon) uygulanan ve postoperatif parathormon ölçümlerine ulaþýlabilen 542 hastanýn verileri prospektif olarak toplandý ve retrospektif olarak deðerlendirildi. Postoperatif 4. saatte paratiroid hormon (PTH) deðerinin <15 pg/mL olmasý hipoparatiroidizm, postoperatif 1. gün kalsiyum (Ca) deðerinin <8 mg/dl olmasý biyokimyasal hipokalsemi olarak tanýmlandý. Hastalar postoperatif hipoparatiroidizm olan (Grup 1) ve hipoparatiroidizm olmayanlar (grup 2) olarak 2 gruba ayrýldý. Ayrýca postoperatif 6. ayda PTH deðeri referans deðerin altýnda olmasý ve/veya hala kalsiyum tedavisine ihtiyaç duyulmasý kalýcý hipoparatiroidi olarak tanýmlandý. Hastalarýn demografik verileri, preoperatif biyokimyasal deðerleri, cerrahi endikasyonlarý, intraoperatif bulgularý, postoperatif 4. saat PTH deðerleri, postoperatif 1. gün kalsiyum deðerleri, spesmenin patolojik incelemesinde istenmeden çýkarýlmýþ paratiroid bezi olup olmadýðý hipoparatiroidizm için risk faktörleri olarak deðerlendirildi. Hipoparatiroidizm geliþimi ile ilgili risk faktörleri lojistik regresyon analizi ile deðerlendirildi.
Bulgular: Çalýþmaya alýnan 542 hastanýn 124’ünde (%22,9) hipoparatiroidizm, 120’sinde (%22,1) hipokalsemi saptandý. 6. Ay takiplerine göre 110 (%20,3) hastada geçici, 7 hastada kalýcý (%1,3) olup, 7 hastanýn (%1,3) 6 aylýk takiplerine ulaþýlamadý. Grup 1’de hipokalsemi oraný daha yüksekti (%39,3 vs %14,3, p<0,0001), postoperatif 1. Gün Ca deðerleri daha düþüktü (8,2+0,7 mg/dl vs 8,5+0,6 mg/dl; p=0,000). Grup 1’de grup 2’ye göre sýrasý ile paratiroid ototransplantasyonu (%15,8 vs %8; p=0,006), piyeste paratiroid saptanma oraný (%20 vs %10,6; p=0,003), santral diseksiyon yapýlmasý (%16,4 vs %5,3, p<0,0001) anlamlý olarak daha yüksekti. Yerinde kalan paratiroid sayýsý açýsýndan 2 grup arasýnda fark anlamlý olup, grup 2’de yerinde 4 paratiroid kalan hasta sayýsý oraný grup 1’den daha yüsekti (%84,1 vs %67,9, p=0,000).
Lojistik regresyon analizinde sadece santral diseksiyon yapýlmasý hipoparatiroidism geliþimini etkileyen baðýmsýz risk faktörü olup, santral diseksiyon yapýlmasý hipoparatiroidizm riskini yaklaþýk 2,3 kat arttýrmaktadýr (p=0,014; OR: 2,336). Diðer faktörler baðýmsýz risk faktörü olarak saptanmadý
Sonuç: Tiroidektomi ile birlikte santral boyun diseksiyonu yapýlmasý hipoparatiroidizm riskini arttýrabilir. Santral diseksiyonda endikasyonlar ve diseksiyon geniþliði deðerlendirilirken hipoparatiroidizm riski göz önünde bulundurulmalýdýr. Santral diseksiyon sýrasýnda paratiroid bezleri ve vaskülarizasyonlarýnýn korunabilmesi için maksimum çaba harcanmalý, eðer piyeste çýkarýlmýþ paratiroid bezi varsa ototransplante edilmelidir. (SETB-2022-05-128)

Objectives: One of the most common complications of thyroidectomy is hypoparathyroidism and that complication has a multifactorial etiology. The etiology of post-operative hypoparathyroidism is multifactorial, some factors affecting hypoparathyroidism have been revealed in the literature, and there are some conflicting results about this complication. In the present study, we aimed to evaluate pre-operative and intraoperative factors affecting development of hypoparathyroidism.
Methods: Data of 542 patients underwent thyroidectomy±central dissection (±lateral dissection) and whose post-operative parathormone values could be obtained, between 2012 and 2020 were collected prospectively and evaluated retrospectively. A parathyroid hormone (PTH) value of <15 pg/mL at the post-operative 4th h was defined as hypoparathyroidism, and a calcium (Ca) value of <8 mg/dl on the 1st post-operative day was defined as biochemical hypocalcemia. Patients were divided into two groups as post-operative hypoparathyroidism (Group 1) and non-hypoparathyroidism (Group 2). In addition, PTH value below the reference value at the post-operative 6th month and/or still needing calcium treatment was defined as permanent hypoparathyroidism. Demographic data of the patients, pre-operative biochemical values, surgical indications, intraoperative findings, post-operative 4th h PTH values, post-operative 1st day calcium values, and pathological examination of the specimen whether there was an unintenionally resected parathyroid gland or not were evaluated as risk factors for hypoparathyroidism. A logistic regression model was used to determine independent risk factors for the development of hypoparathyroidism.
Results: Hypoparathyroidism was determined in 124 (22.9%) and hypocalcemia was determined in 120 (22.1%) patients. According to 6-month follow-up period; 110 (20.3%) patients were transient, 7 (1.3%) patients were permanent, and 7 (1.3%) patients data could not be obtained. The hypocalcemia rate was higher in Group 1 (39.3% vs. 14.3%, p<0.0001), also the post-operative 1st day calcium values were lower (8.2+0.7 mg/dl vs. 8.5+0.6 mg/dl; p=0.000). The rate of parathyroid autotransplantation, the rate of parathyroid gland in pathological specimen, and the rate of central dissection were significantly higher in Group 1 compared to group 2 (15.8% vs. 8%; p=0.006; 20% vs. 10.6%; p=0.003; 16.4% vs. 5.3%, p<0.0001, respectively). The difference between the two groups was significant in terms of the number of remaining parathyroids, and the rate of the number of patients with four remaining parathyroids in place was higher in Group 2 than in Group 1 (84.1% vs. 67.9; p=0.000). In the logistic regression analysis, only central dissection is an independent risk factor affecting the development of hypoparathyroidism, and central dissection increases the risk of hypoparathyroidism approximately 2.3 times (p=0.014; OR: 2.336). The other factors were not determined as independent risk factor.
Conclusion: Performing central neck dissection with total thyroidectomy may increase the risk of hypoparathyroidism development. The risk of hypoparathyroidism should be considered when evaluating the indications and dissection extent in the central dissection. Maximum effort should be made to preserve the parathyroid glands and their vascularization during central dissection, and if there is a removed parathyroid gland, it should be autotransplanted.

Abstract | English Full Text

Amaç: Tiroid nodülleri yaygýn görülür. Bu nodüllerin oluþumunda, genetik ve çevresel faktörlerin yaný sýra kronik inflamasyon da rol oynamaktadýr. Tanýsal deðerlendirmede anahtar nokta, maligniteyi dýþlamaktýr. Malignite olasýlýðýný gösterebilecek biyobelirteçler araþtýrýlmaya devam etmektedir. Çalýþmamýzda eritrosit sedimentasyon hýzý, lökosit, fibrinojen, C reaktif protein ve pentraxin-3 enflamatuvar biyobelirteçleriyle tiroid nodül özellikleri ve sitolojileri arasýndaki iliþkiyi deðerlendirdik.
Yöntemler: Bu çalýþmada 55 hastadan oluþan nodüler guatr grubu ve 58 saðlýklý bireylerden oluþan kontrol grubu oluþturuldu. Katýlýmcýlarýn cinsiyeti, yaþý, aile öyküsü, tiroid fonksiyon testleri, sedimentasyon, lökosit, fibrinojen, CRP ve pentraksin-3 serum düzeyleri kaydedildi. Ultrasonografide nodül sayýsý, en büyük nodül çapý, nodüllerin yapýlarý incelendi ve tiroid biyopsisi yapýldý.
Bulgular: Hastalarda tiroid nodülü sayýsý 1 ile 4 arasýnda idi. Ýki tiroid nodülü olan hasta sayýsý daha fazlaydý (%47,3, n: 26). Nodül çaplarý 3-62 (ortalama 21) mm arasýnda deðiþmekteydi. Tiroid biyopsisinde hastalarýn %25.5'inde (n: 14) papiller tiroid kanseri saptandý. CRP deðerleri arttýkça ultrasonografide nodül sayýsý arttý (p=0.013). Ayrýca fibrinojen deðerleri arttýkça ultrasonografide nodül sayýsý azaldý (p=0,003). Sedimantasyon, lökosit ve pentraksin-3 deðerleri açýsýndan gruplar arasýnda anlamlý fark bulunmadý.
Sonuç: Nodül sayýsý CRP ile pozitif, fibrinojen seviyeleri ile negatif korelasyon gösterdi. Bu klinik takipte önemli olabilir. Ancak benign ve malign ayrýmý ile biyobelirteçler arasýnda fark yoktu. Nodül sayýsýnýn artýþýyla korelasyon gösteren CRP deðerleri, prognozda ve klinik takipte kullanýlabilir. (SETB-2021-03-063)

Objectives: Thyroid nodules (TN) are common. Genetic and environmental factors as well as chronic inflammation play a role in occurrence of these nodules. The key point in diagnostic assessment is to rule out malignancy. Biomarkers that can show the possibility of malignancy continue to be investigated. We evaluated the relationship between sedimentation rate, leukocyte, fibrinogen, C-reactive protein (CRP), and pentraxin 3 (PTX3) inflammatory markers and characteristics and cytology of TN.
Methods: This study included a nodular goiter group with 55 persons and control group with 58 persons. Participants’ gender, age, family history, thyroid function tests, sedimentation, leukocyte, fibrinogen, CRP, and PTX3 serum levels were recorded. The number of nodules, the largest nodule diameter, nodular echogenicity, and nodule structures were examined on ultrasonography (US) and thyroid biopsy was performed.
Results: The number of TN in patients was between 1 and 4. The number of patients with two TN was higher (47.3%, n=26). Nodule diameters differed between 3 and 62 (mean 21) mm. In thyroid biopsy, papillary thyroid cancer was detected in 25.5% (n=14) of the patients. The number of nodules on US increased as CRP values increased (p=0.013). In addition, the number of nodules on US decreased as fibrinogen values increased (p=0.003). No significant difference was found between the groups in terms of sedimentation, leukocyte, and PTX3 values.
Conclusion: The number of TN was positively correlated with CRP and negatively correlated with fibrinogen levels. However, there was no difference between benign and malignant differentiation and biomarkers. CRP values that correlate with the increase in the number of nodules can be used in prognosis and clinical follow-up.

Abstract | English Full Text

Amaç: Ýnterstisyel Cajal hücrelerinden kaynaklanan gastrointestinal stromal tümörlerde (GÝST) malignite potansiyeli, mitoz, tümör çapý ve yerleþim yerine dayanan prognostik hastalýk risk sýnýflamasýyla belirlenir. Fosfohiston H3 (FHH3) mitotik aktivite içindeki hücre nükleuslarýný gösteren bir immunhistokimyasal belirleyicidir. Çalýþmamýzda GÝST’lerde mitoz sayýsýný FHH3 belirleyicisiyle deðerlendirmek, hematoksilen&eozin (HE) boyalý preparatlarda sayýlan mitozla arasýnda uyum olup olmadýðýný karþýlaþtýrmak ve diðer prognostik verilerle iliþkisini irdelemek amaçlanmýþtýr.
Yöntem: Çalýþmamýzda 2006-2017 yýllarý arasýndaki GÝST tanýlý olgularýn klinikopatolojik bulgularý ve takip süreleri belirlenmiþtir. Her olgunun HE boyalý kesitinde mitoz sayýlan bloða FHH3 immunhistokimyasal belirleyicisi uygulanarak HE ve FHH3 bazlý mitoz sayýsýna göre prognostik risk skoru saptanmýþtýr. Olgular HE ve FHH3 boyanma uyumuna göre gruplandýrýlmýþtýr: Grup I: HE+ ve FHH3 + ve Grup II: HE+ ve FHH3 –. Olgular, ayrýca, 2012 öncesinde ve 2012 ile sonrasýnda taný alanlar olarak gruplandýrýlmýþ ve HE ile FHH3 arasýndaki boyanma uyumu tekrar analiz edilmiþtir.
Bulgular: Çalýþmada 98 olgu yer almýþtýr. Olgularýn %63.3’ünde (62/98 olgu) HE ve FHH3 ile mitoz görülmüþtür (Grup I). Ancak, olgularýn %36.7’sinde HE’de saptanan mitozlar FHH3 ile gözlenememiþtir (Grup II). Sadece iki olguda FHH3 ile risk skoru deðiþmiþtir (çok düþük→intermedier grade). 2012 ve sonrasýnda HE+ ve FHH3 + olgularýn oraný, HE+ ve FHH3 – olgulara göre anlamlý derecede daha yüksektir. HE ve FHH3’e dayalý risk skorlarý arasýnda istatistiksel olarak anlamlý uyum saptanmýþtýr (p<0.05). HE bazlý risk skoru gruplarý arasýnda toplam yaþam süreleri bakýmýndan anlamlý farklýlýk bulunmuþtur (p<0.05). Buna göre, risk skoru çok düþük ya da düþük olanlarýn toplam yaþam süresi risk skoru yüksek olanlara göre daha uzundur. FHH3 bazlý risk skoru gruplarý arasýnda ise, fark mevcut deðildir (p>0.05).
Sonuç: GÝST olgularýnda FHH3, laboratuvarýn teknik koþullarý dikkate alýnarak ve daha yeni bloklarda mitozu belirlemekte kullanýlabilir, ancak HE ile saptanan mitoza üstün görünmemektedir. Ayrýca, GÝST’te, saðkalýmý öngören yeni belirleyiciler üzerinde araþtýrmalara devam edilmelidir. (SETB-2021-07-215)

Objectives: In gastrointestinal stromal tumors (GIST), malignancy potential is determined by the prognostic disease risk stratification based on mitosis, tumor size, and location. Phosphohistone H3 (PHH3) is an immunohistochemical marker showing mitotic activity in cells. In this study, we aimed to evaluate mitosis in GIST with PHH3, compare the results with hematoxylin and eosin (HE) stained slides, and examine its relationship with other prognostic data.
Methods: Clinicopathological findings and survival were determined in GIST cases diagnosed between 2006 and 2017. The prognostic risk score was calculated according HE- and PHH3-based mitosis. The cases were classified as Group I: HE + and PHH3 + and Group II: HE + and PHH3–. They were also grouped as those diagnosed before and after 2012 and the staining results of HE and PHH3 were re-analyzed.
Results: Ninety-eight cases were included in the study. Mitosis was detected with both HE and PHH3 in 63.3% of the cases (62/98 cases) (Group I) while in 36.7% of cases, it was detected with HE but not with PHH3 (Group II). In only two cases, the risk score changed with PHH3 (very low → intermedier grade). The ratio of HE + and PHH3 + cases in 2012 and after was significantly higher than HE + and PHH3 – cases. A statistically significant relation was found between HE- and PHH3-based risk scores (p<0.05). There was a significant difference between HE-based risk score groups in terms of survival (p<0.05), while no difference was observed between the PHH3-based risk score groups (p>0.05).
Conclusion: In GIST cases, PHH3 can be used to determine mitosis in more recent blocks, taking into account the technical conditions of the laboratory, but it does not seem to be superior to mitosis detected by HE. Research should continue on new survival determinants for GIST.

Abstract | English Full Text

Amaç: Varyant histolojinin üst üriner sistem ürotelyal karsinomu nedeni ile ameliyat olan hastalarýn patolojik ve sað kalým sonuçlarýn üzerindeki etkisini saptamak.
Yöntem: 2001-2019 yýllarý arasýnda tek merkezde üst üriner sistem ürotelyal karsinomu nedeni ile ameliyat olan 128 hastanýn verileri geriye dönük olarak incelendi. Saf ürotelyal karsinom hastalarý ve varyant histoloji olanlar demografik özellikler, patolojik sonuçlar ve sað kalým parametreleri açýsýndan birbiri ile karþýlaþtýrýldý.
Bulgular: Ortalama hasta yaþý 65±11yýl olarak saptandý. Kadýn erkek oraný 30/98 olarak, medyan takip süresi ise 26,5 (1-176) ay olarak belirlendi. Hatalarýn 14,8% inde varyant histoloji saptandý. Varyant histoloji cerrahi sýnýr pozitifliði, lenf nod metastazý, lenfovasküler invazyon varlýðý, yüksek tümör evre ve derecesi ile iliþkili bulundu (p=0.001, p=0.012, p=0.001, p=0.002, p=0.009, sýrasýyla). 3 yýllýk kanser spesifik ve genel sað kalým oraný sýrasýyla 79,6% ve 77,3% olarak saptandý. Varyant histoloji ile kanser spesifik sað kalým ve genel sað kalým arasýnda istatistiksel anlamlý bir iliþki belirlenemedi. (p=0.514, p=0.515, sýrasýyla).
Sonuç: Üst üriner sistem ürotelyal karsinomunda varyant histoloji lokal ileri hastalýk ile iliþki iken, sað kalým üzerinde bir etkisi gösterilemedi. (SETB-2021-06-175)

Objectives: The objective of the study was to determine the effect of variant histology on pathological outcomes and survival in patients operated for the upper urinary tract urothelial carcinoma (UTUC).
Methods: Data of 128 patients who were operated for UTUC between 2001 and 2019 were retrospectively analyzed. Patients with pure urothelial carcinoma and patients with variant histology were compared in terms of demographics, pathological outcomes, and survival.
Results: The mean age of the patients was 65±11 years, female to male ratio was 30/98 and median follow-up period was 26.5 (1–176) months. Variant histology was detected in 14.8% of patients. Variant histology was found to be associated with surgical margin positivity, lymph node metastasis, presence of lymphovascular invasion, high tumor stage and grade (p=0.001, p=0.012, p=0.001, p=0.002, and p=0.009, respectively). Three-year cancer-specific and overall survival rates were 79.6% and 77.3%, respectively. There was no statistically significant relationship between variant histology with cancer-specific and overall survival (p=0.514 and p=0.515, respectively).
Conclusion: Variant histology of UTUC was found to be associated with locally advanced disease, but its effect on survival could not be demonstrated.

Abstract | English Full Text

Giriþ: Konjenital adrenal hiperplazi (KAH), tuz kaybý ve virilizasyon ile karakterize, otozomal resesif kalýtýlan bir hastalýktýr. CYP21A2 genindeki mutasyonlardan kaynaklanan 21 hidroksilaz eksikliði KAH’lý hastalarýn %90-95’ini oluþturur. Diðer daha nadir formlar ise 3-β-hidroksisteroid dehidrogenaz eksikliði (3β-HSD), 11-β-hidroksilaz eksikliði (11β-OHD) (%5-8), 17-α-hidroksilaz eksikliði (17α-OHD) ve STAR defektleridir (Konjenital lipoid adrenal hiperplazi).
Bu çalýþmamaýzda, kliniðimizde izlenen 64 KAH hastasýnýn klinik ve laboratuar verileri sunulmuþtur.
Material-metod: Ýzmir Dr. Behçet Uz Çocuk Hastanesi Çocuk Endokrinoloji kliniðinde izlenmekte olan 64 KAH tanýlý hastanýn klinik, laboratuar ve genetik özellikleri retrospektif olarak incelenmiþtir.
Bulgular: Hastalarýn ellialtýsý (%87,5) 21-OHD, 4’ü (%6,3) 17α-OHD, 3’ü (%4,7) 11β-OHD ve biri (%1,5) STAR defekti tanýsý almýþtý. 21-OHD tanýlý olgularýn en sýk baþvuru nedeni belirsiz dýþ genital yapý idi. 21-OHD tanýlý olgular diðer nadir KAH nedenli olgulardan daha erken taný almýþlardý. 21-OHD tanýlý olgularýn 46’sýnda CYP21A2 geninde mutasyon tanýmlandý; en sýk mutasyonlar IVS2, Q318X, I172N ve büyük delesyonlar idi. 11β-OHD tanýlý üç olgu makropenis ve erken pubik kýllanma ile baþvurmuþtu (median baþvuru yaþý: 26 ay idi). 17α-OHD tanýlý dört olgu mevcuttu ve bu olgularda CYP17A1 geninde iki farklý mutasyon tanýmlandý; bu mutasyonlardan biri daha önce literatürde tanýmlanmamýþtý. STAR defekti tanýlý olgu, yaþamýnýn 44. Gününde dehidratasyon ve kolestaz tablosunda baþvurmuþtu. Genetik analizinde STAR geninde daha önce tanýmlanmamýþ novel bir varyant saptandý.
Sonuç: Sonuç olarak, çalýþmamýzda 21-OHD’li KAH olgularýnda literatür ile uyumlu fenotip-genotip korelasyonu izlendi. Ayný zamanda çalýþmamýzda KAH’ýn nadir tiplerine neden olan STAR ve CYP17A1 genlerinde daha önce tanýmlanmamýþ novel mutasyonlar saptandý. (SETB-2021-06-177)

Objectives: Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive diseases characterized by salt wasting or virilization. 21 hydroxylase deficiency (21-OHD) accounts for 90–95% of all cases of CAH and caused by the genetic defects of CYP21A2. Other forms include 3-β-hydroxysteroid dehydrogenase deficiency, 11-β-hydroxylase deficiency (11β-OHD) (%5-8), 17-α-hydroxylase deficiency (17α-OHD), and steroidogenic acute regulatory protein (STAR) defects (congenital lipoid adrenal hyperplasia) with mutations in HSD3B2, CYP11B1, CYP17A1, and STAR, respectively. Objectives: Herein, we aimed to present the clinical and genetic features of 64 patients with various types of CAH.
Methods: Sixty-four patients with CAH, monitored in the Izmir Dr. Behcet Uz Children Hospital Division of Pediatric Endocrinology, were retrospectively analyzed for the clinical, laboratory, and genetic data.
Results: Fifty-six patients (87.5%) had 21-OHD and four patients (6.3%) had 17α-OHD, three patients (4.7%) had 11β-OHD, and one patient (1.5%) had STAR defect. The most common presenting features in 21-OHD were ambiguous genitalia. Patients with 21-OHD were diagnosed earlier than the rare groups. Disease-causing variants of CYP21A2 were identified in 46 patients. The most common mutations were IVS2, Q318X, I172N, and large deletions. Three patients with 11β-OHD were presented with enlargement of penis and early pubic hair at the median presenting age of 26 months. 17α-OHD deficiency was detected in 4 cases. Genetic analysis revealed two different homozygous CYP17A1 variants. The patient with STAR defect was presented with dehydration and cholestasis in 44 days of the life. Genetic analysis of patient with STAR deficiency revealed a novel homozygous variant.
Conclusion: The current study reported a genotype-phenotype correlation consistent with literature data in CAH cases with 21-OHD. This study also reported novel homozygous variants in STAR and CYP17A1 genes that lead to rare types of CAH.

Abstract | English Full Text

Papiller tiroid kanseri genellikle boyun lenf bezlerine metastaz yapabilir. Uzak metastazlarý genellikle solid organ metastazý olarak görülmesine raðmen, çok nadiren cilt metastazý yapabilir. Bu çalýþmada uzak organ metastazýnýn ilk bulgusu olarak cilt metastazý ile baþvuran papiller tiroid kanserli olguyu sunmayý amaçladýk.
63 yaþýnda erkek hasta boyun sol lateralinde cilt lezyonu ile baþvurdu. 14 yýl önce subtotal tiroidektomi yapýlmýþ ve patolojisinde malignite saptanmamýþ olan hastanýn, yeni geliþen boyun sol lateralindeki lezyondan yapýlan eksizyonel biyopside foliküler varyant papiller kanser saptandý. Yapýlan USG’de sol lobta 12x10x8 mm hiperekoik nodülün ÝÝAB’si Bethesta 3 olarak deðerlendirildi. Toraks BT’de bilateral multipl akciðer metastazý ile uyumlu lezyon saptandý ve en büyük nodülden yapýlan trucut biyopsi metastaz ile uyumlu geldi. Total tiroidektomi yapýldý ve patolojide kanser saptanmadý. Hastaya postoperatif multipl RAÝ tedavisi uygulandý.
Tiroid kanseri öyküsü olmayan hastalar çok nadiren cilt metastazý ile prezente olabilirler ve ayýrýcý tanýda tiroid kanseri gözardý edilmemelidir. Cilt metastazýndan sonra yapýlan tiroidektomi spesimesinin dikkatli deðerlendirilmesine raðmen tiroidde tümör saptanmayabilir. Papiller tiroid kanserinde cilt metastazý uzak metastaz olarak deðerlendirilmeli ve diðer uzak metastazlar açýsýndan araþtýrýlmalý, tedavisi multidisipliner olarak planlanmalýdýr. (SETB-2021-05-150)

Papillary thyroid cancer can usually metastasize to neck lymph nodes. Distant metastases are generally seen as solid organ metastases, in contrast, skin metastasis can rarely be seen. We present a case with papillary thyroid cancer diagnosed with skin metastasis as first clinical sign of distant organ metastasis.
A 63-year-old male patient admitted with a skin lesion in the left lateral neck. He had undergone subtotal thyroidectomy 14 years ago without relevant history of malignancy. Follicular variant papillary thyroid cancer was detected in the excisional biopsy performed from the cutaneous lesion. A 12 × 10 × 8 mm hyperechoic nodule in the left lobe was detected in USG and evaluated as Bethesta-III in fine needle aspiration biopsy. Bilateral multiple lung metastases were detected on thorax CT, trucut-biopsied from largest nodule and confirmed as metastasis. Carcinoma was not detected in total thyroidectomy, and post-operative multiple RAI therapies were applied.
Patients without history of thyroid cancer rarely present with skin metastases and thyroid cancer should not be overlooked in differential diagnosis. Despite meticulous evaluation of the thyroidectomy specimen, tumor may not be detected in the gland. Skin metastasis in papillary thyroid cancer should be evaluated as distant metastasis and investigations for other metastases should be done, and the treatment should be planned in a multidisciplinary manner.

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