Diagnosis and treatment of thyroid cancer in children in the multicenter analysis in Poland for PPGGL

Wstęp: Zróżnicowane raki tarczycy (DTC, differentiated thyroid carcinoma) występują u dzieci rzadko. Większość przypadków wykrywanych jest w wieku 11–17 lat. W odróżnieniu od dorosłych DTC u dzieci prezentują odmienne zachowanie biologiczne. Mała liczba przypadków DTC w poszczególnych ośrodkach oraz względnie łagodny ich przebieg utrudniają ocenę występowania i leczenia DTC u dzieci w Polsce, uzależniając ją od wysiłków włożonych w uzyskanie rzetelnych danych. Autorzy przedstawiają wstępne wyniki analizy wieloośrodkowej dotyczące występowania, diagnostyki i leczenia DTC u dzieci. Materiał i metody: Podjęte badania są retrospektywną analizą obejmującą lata 2000–2005, opartą na danych z historii chorób uzyskanych z ankiet rozesłanych do ośrodków dla dzieci i dorosłych podejmujących leczenie DTC. Do analizy zgłoszono 107 pacjentów z 14 ośrodków akademickich w Polsce. Analizie poddano wiek i płeć dzieci z DTC, wielkość i lokalizację zmian w tarczycy, sposoby rozpoznawania DTC, rodzaje i zakres wykonywanych zabiegów operacyjnych oraz leczenie uzupełniające izotopem J131. Wyniki: Raka brodawkowatego stwierdzono u 83 dzieci, pęcherzykowego u 10 dzieci, a rdzeniastego u 14 dzieci. Częstość występowania DTC u dzieci w Polsce wahała się między 18 a 23 przypadkami rocznie. W województwach: mazowieckim i połączonych wielkopolskim i lubuskim wykazano w okresie 2000–2005 wyższą (24 i 25) częstość występowania DTC, w pozostałych województwach wykazywano od 2 do 10 przypadków DTC. Największą grupę pacjentów stanowiły dzieci w wieku 11–15 lat, a stosunek dziewcząt do chłopców wynosił 3,3 : 1. Klinicznie DTC prezentowały się najczęściej jako pojedyncze guzki tarczycy. Limfadenopatię szyjną w badaniu klinicznym stwierdzono u 42% pacjentów, a śródoperacyjnie u 50% dzieci. U większości pacjentów dominowały niższe stopnie zaawansowania DTC (T1 u 36% i T2 u 26% dzieci). Operacje jednoetapowe wykonano u 65% dzieci, operacje dwuetapowe u 25% dzieci, a profilaktyczne tyreoidektomie u 79% dzieci z grupy pacjentów z rakiem rdzeniastym tarczycy (MTC, medullary thyroid cancinoma) i mutacją genu Ret. Leczenie izotopowe J131 podjęto u 80% dzieci. Przerzuty do płuc w scyntygrafii poterapeutycznej wykazano u 14% dzieci z DTC. Wnioski: We wnioskach podkreśla się konieczność wdrożenia na terenie całego kraju ujednoliconego i ocenianego na podstawie obiektywnych przesłanek sposobu postępowania z dziećmi z DTC.Introduction: Differentiated thyroid carcinoma (DTC) in children presents different biological behavior in comparison to adults. Authors presents preliminary results of multicenter analysis concerning incidence, diagnostics and treatment of DTC in children. Material and methods: The study is a retrospective analysis of 107 pediatric patients from 14 academic centers based on the data from 2000 to 2005 obtained by questionnaire in hospitals involved in the treatment of DTC in children. Results: Papillary thyroid cancer was diagnosed in 83 children, follicular thyroid cancer in 10 children and medullary thyroid cancer in 14 children. Incidence of DTC in children was estimated between 18 and 23 cases per year. The biggest group of patients consisted of children between 11 and 15 years of age, with girls to boys ratio 3.3 : 1. Clinically DTC in children presented most often as solitary thyroid nodule. Cervical lymphadenopathy was observed in 42% of patients. Intraoperative verification indicated metastatic nodes in 50% of children. Low stage DTC predominated (T1 in 36% and T2 in 26% of children). One step surgery was performed in 65% of children with DTC, two step surgery in 25% of patients. I131 therapy was undertaken in 80% of children. Lung metastases were indicated in post therapeutic studies in 14% of children with DTC. Prophylactic thyroidectomies were performed in 79% of children in the group of patients with MTC and RET gene mutations. Conclusions: The necessity of introduction of unified therapeutic standard in children with DTC in Poland is underlined

Chorzy trudni nietypowiTamponada osierdzia u noworodka z guzem serca

A case of a neonate with a life-threatening cardiac tamponade due to cardiac tumour is presented. Pericardiocentesis was performed and 300 ml of purulent fluid was evacuated. Pericardial drainage was undertaken. Six months later cardiac tamponade recurred and was again successfully treated with pericardial drainage. A subsequent 10-month follow-up was uneventful. Diagnosis and treatment of cardiac tumours associated with pericardial effusion are discussed

Ropnie i przetoki odbytu u dzieci – ocena skuteczności leczenia. Czy można uniknąć nawrotów?

Wprowadzenie: Ropnie i przetoki odbytu są częstym schorzeniem w populacji dziecięcej. U większości pacjentów ropień odbytu stanowi manifestację obecności przetoki odbytu. Doświadczenia naszego ośrodka wskazują na powszechny brak wiedzy w tym zakresie i postępowanie ograniczone wyłącznie do nacięcia ropnia, co w większości przypadków prowadzi do wielokrotnych wznów. Cel: Celem pracy jest przedstawienie możliwych sposobów postępowania oraz ocena ich skuteczności w wyeliminowaniu nawrotów ropni i przetok odbytu. Materiał i metoda: Przeanalizowano retrospektywnie dokumentację medyczną 24 niemowląt leczonych z powodu ropni i przetok odbytu w naszym ośrodku w latach 2013–2015. Połowa pacjentów (grupa I) była pierwotnie leczona w naszym ośrodku, druga połowa (grupa II) przed przyjęciem miała wykonywane interwencje chirurgiczne w innych ośrodkach. U wszystkich pacjentów z ropniami odbytu śródoperacyjnie zidentyfikowano kanał przetoki odbytu (100%) i wykonano fistulotomię. Wyniki: U żadnego pacjenta nie obserwowano nawrotu ropnia odbytu ani nietrzymania stolca. U pacjentów z grupy II obserwowano bardziej nasilony stan zapalny. Część z nich wymagała również dodatkowych procedur chirurgicznych w postaci drenażu rozległego ropnia pośladka; wymagali oni również dłuższej antybiotykoterapii i hospitalizacji. Wnioski: Procedury małoinwazyjne (nasiadówki, antybiotykoterapia, nakłucie lub nacięcie i drenaż ropnia) są kuszące z uwagi na swoją prostotę i brak konieczności znieczulenia pacjenta, ale wiążą się z powikłaniami i dużą liczbą nawrotów. Stosowanie procedur bardziej inwazyjnych (fistulotomia, fistulektomia) wiąże się z koniecznością znieczulenia pacjenta i leczenia operacyjnego, ale pozwala znacznie zredukować częstość nawrotów i innych powikłań

Perianal abscess and fistula-in-ano in children – evaluation of treatment efficacy. Is it possible to avoid recurrence?

Introduction: Perianal abscess and fistula-in-ano are common findings in infants and children. The perianal abscess is usually a manifestation of a fistula-in-ano. Experience of our center indicates general lack of knowledge of the origin of the abscess and therefore, it is usually treated by incision and drainage, which leads to repeated recurrences. Aim: We aimed to present the optimal management of fistula-in-ano and perianal abscess in order to reduce or even eliminate the risk of recurrence. Material and methods: The retrospective study included 24 infants with perianal abscess treated at our center between 2013 and 2015. Patients were divided into two groups: group I (50%) was primary treated in our center, while group II had undergone prior surgical interventions in other hospitals. Fistula-in-ano was intraoperatively identified in all patients (100%) and fistulotomy was performed. Results: No fecal incontinence or recurrence of perianal abscess were observed in any of our patients. In group II, the disorder was associated with severe inflammation, some patients underwent an additional surgical intervention, such as incision and drainage of an extensive buttock’s abscess; patients required longer antibiotic therapy and prolonged hospitalization. Conclusion: Minimally invasive approach (sitz baths, antibiotic therapy, puncture or incision and drainage of the abscess) appears tempting due to its simplicity and lack of need for general anesthesia, but it is associated with a high recurrence rate. Fistulotomy and fistulectomy, which are slightly more invasive procedures, significantly reduce the recurrence rate of fistulain- ano and perianal abscess

Liver Histopathology in Late Protocol Biopsies after Pediatric Liver Transplantation

Liver transplantation has become a routine treatment for children with end stage liver failure. Recently, the long term survival of pediatric patients after liver transplantation has improved, with a life expectancy much longer than that of adult recipients, but also with longer exposition of the graft to various injuries, including immunological, inflammatory and others. Biochemical tests, although important, do not always reflect graft injury. The aim of our study was to analyze the histopathology of the graft in late protocol biopsies and correlate it with the clinical and biochemical status of these patients. We analyzed 61 protocol liver biopsies taken from 61 patients. Biopsies were taken 9.03–17.09 years (mean 12.68, median 11.74 years) after transplantation. Liver specimens were examined particularly for the presence and stage of liver fibrosis, inflammation, steatosis, and acute or chronic cellular and humoral rejection. We did not find any abnormalities in 26 (42.6%) liver specimens. None of the patients had signs of cellular or antibody mediated rejection or chronic rejection. In 23 liver biopsies (37.7%), we found non-specific lymphoid infiltrates. Another problem was fibrosis (equal to or more than three on the Ishak scale)—we found it in 17 patients, including seven liver specimens (11.5%) with severe fibrosis (Ishak 5–6). Conclusions: Various pathomorphological abnormalities were found in more than half of patients with a median 11.74 years post-transplant follow-up. Most of them presented normal laboratory liver tests at the same time, suggesting a slow subclinical process leading to pathomorphological abnormalities. No single factor for the development of these abnormalities was found, but our study supports the need for protocol liver biopsies even in patients with normal/almost normal biochemical liver tests

Choledochal Cyst Excision in Infants—A Retrospective Study

A choledochal cyst is a rare malformation primarily diagnosed in children. The only effective therapy remains surgical cyst resection followed by Roux-en-Y hepaticojejunostomy. Treating asymptomatic neonates remains a point of discussion. Between 1984 and 2021, we performed choledochal cyst (CC) excision in 256 children at our center. Out of this group, we retrospectively reviewed the medical records of 59 patients who were operated on under one year of age. Follow-up ranged from 0.3 to 18 years (median 3.9 years). The preoperative course was asymptomatic in 22 (38%), while 37 patients (62%) had symptoms before surgery. The late postoperative course was uneventful in 45 patients (76%). In symptomatic patients, 16% had late complications, while in asymptomatic patients, only 4%. Late complications were observed in the laparotomy group in seven patients (17%). We did not observe late complications in the laparoscopy group. Early surgical intervention is not followed by a high risk of complications and may prevent the onset of preoperative complications, giving excellent early and long-term results, especially after minimally invasive laparoscopic surgery

Hand-assisted laparoscopic donor nephrectomy: a single centre experience

Introduction : The advantages of a minimally invasive nephrectomy are a faster recovery and better quality of life for the donors. Until recently, the majority of donor nephrectomies in Poland were done by open surgery. Aim : To present a single centre experience in hand-assisted laparoscopic donor nephrectomy (HALDN). Material and methods: The first videoscopic left donor nephrectomy in Poland was performed in our department in 2003 using a hand-assisted retroperitoneal approach. From 2011, we changed the method to a transperitoneal approach and started to harvest also right kidneys. Since then, it has become the method of choice for donor nephrectomy and has been performed in 59 cases. Preoperatively, kidneys were assessed by scintigraphy and by angio-Computed tomography. We harvested 32 left and 27 right kidneys. There were double renal arteries in 2 cases and triple renal arteries in 1 case. The warm ischaemia time (WIT) was 80–420 s (average 176.13 s); operative time was 85–210 min (average 140 min). Results: All procedures were uncomplicated, and all donors were discharged after 2–8 days with normal creatinine levels. The average follow-up period lasted 23 months (1–51 months). Out of all of the cases, 1 case had two minor complications, while all others were uneventful. None of the donors were lost to follow-up. All of the kidneys were transplanted. There were 2 cases of delayed graft function (DGF) and 2 cases of ureter necrosis. One of those kidneys was lost in the third postoperative week. Conclusions : Our limited experience shows that HALDN is a safe method and should be used routinely instead of open surgery

Impact of Graft Size Matching on the Early Post-Transplant Complications and Patients Survival in Children after Living Donor Liver Transplantations

We aimed to assess the impact of the graft-recipient weight ratio (GRWR) on early post-transplant complications and patient survival rates in children after living donor liver transplantation (LDLT). We retrospectively analyzed 321 patients who underwent LDLT from 2004 to 2019. The recipients were categorized into four groups: 37 patients had a GRWR ≤ 1.5% (Group A), 196 patients had a GRWR > 1.5% and ≤3.5% (Group B), 73 patients had a GRWR > 3.5% and <5% (Group C) and 15 patients had a GRWR ≥ 5% (Group D). Incidence of early surgical complications including vascular complications, biliary complications, postoperative bleedings, gastrointestinal perforations and graft loss were comparable among groups with a different GRWR. Delayed abdominal wound closure was more common in patients with a GRWR > 3.5%. Recipients with a GRWR < 5% had a significantly better prognosis concerning patients and graft survival. Using grafts with a GRWR < 5% allows us to expand the donor pool and decrease the risk of mortality while on the waiting list, when patients at the time of transplantation have less advanced liver disease. LDLT with a GRWR ≥ 5% is related to a higher risk of poor outcome, and thus should be an option for treating selected patients when the risk of a delayed transplantation is high and access to deceased donors is limited

Health Status in Long-Term Survivors of Hepatoblastoma

The aim of this study was to evaluate the health status of children cured from hepatoblastoma. Forty-five patients with hepatoblastoma treated between 1996–2014 were assessed. The recorded data included sex, age at diagnosis, disease stage, treatment methods, time since diagnosis, and the evaluation of health status domains which included performance status, growth development, hearing, cardiovascular, skeletal, gastrointestinal, genitourinary, neurological, and hematological function. There were 30 boys and 15 girls. The age at diagnosis ranged from one month to 14 years (median one year). At the time of the health status evaluation, the youngest patient was 5.5 years old and the oldest was 21 years of age (median—10 years). All patients were treated according to the Childhood Liver Tumors Strategy Group—SIOPEL recommendations, though they were not active participants of the studies. The median cumulative dose of cisplatin was 520 mg/m2 and 360 mg/m2 for doxorubicin. Thirty-six patients underwent partial hepatectomy, and nine total hepatectomy and liver transplantation. At a median of nine years from diagnosis, 68% of hepatoblastoma survivors had experienced at least one chronic health condition of any grade. The most frequent late complication was ototoxicity (28.8%), and the most serious were second malignancies (6.6%) and cardiomyopathy (4.4%). Conclusion: Survivors of hepatoblastoma are at risk for long-term complications. They require long-term monitoring for late effects