Chronic intussusception in 16-year-old boy caused by enterogenic cyst of caecum

Chronic intussusceptions are usually observed in older children. They create a clinical problem due to the occurrance in the course of disease, non-characteristic abdominal pain associated with vomiting and sometimes diarrhea without macroscopic blood in the stool. They are caused in the majority of cases by the presence of a lead point and require surgical treatment. Visualization of the lead point by imaging techniques is difficult and laboratory examinations are usually normal. The authors present a case of chronic ileocecal intussusception in which lead point was an enterogenic cyst of the caecum. A changing clinical manifestation and diagnostic difficulties caused a delay in the decision to operate for 23 days. The definitive diagnosis was determined only intraoperatively, during a reconnaissance laparotomy. Luckily for the patient this delay did not cause complications (necrosis and perforation of the wall of the intestine) and did not influence the final result of the treatment.Wgłobienia przewlekłe zazwyczaj obserwuje się u starszych dzieci. Stanowią trudny problem diagnostyczny ze względu na występujące w ich przebiegu niecharakterystyczne bóle brzucha z towarzyszącymi wymiotami i czasami biegunką, bez makroskopowo widocznej krwi w stolcu. W większości przypadków są one spowodowane obecnością punktu prowadzącego i wymagają leczenia operacyjnego. Uwidocznienie punktu wiodącego w badaniach obrazowych jest trudne, a badania laboratoryjne z reguły nie wykazują odchyleń od normy. Autorzy przedstawiają przypadek przewlekłego wgłobienia krętniczo-kątniczego, którego punktem wyjścia była torbiel enterogenna kątnicy. Zmienny obraz kliniczny i trudności diagnostyczne spowodowały opóźnienie decyzji o leczeniu operacyjnym o 23 dni. Ostateczne rozpoznanie postawiono dopiero śródoperacyjnie, w czasie laparotomii zwiadowczej. Szczęśliwie dla pacjenta opóźnienie to nie spowodowało wystąpienia powikłań (martwicy i przedziurawienia ściany jelita) i nie wpłynęło na końcowy wynik leczenia

Features of impaired seminiferous tubule differentiation are associated with germ cell neoplasia in adult men surgically treated in childhood because of cryptorchidism.

Seminiferous tubule differentiation was related to the occurrence of germ cell neoplasia in 38 men, aged 17-47, treated surgically in childhood for cryptorchidism. Tissues from 46 testes obtained from biopsies taken as a neoplastic preventive procedure or whole testes removed because of GCT were evaluated quantitatively. Paraffin sections were treated with antibodies against placental like alkaline phosphatase (PLAP), a marker of germ cell neoplasia, and cytokeratin 18 (CK-18), a marker of immature Sertoli cells. Quality of spermatogenesis and number Leydig cells were assessed with a score count. Seminiferous tubules diameter, thickness of basal membrane and size of intertubular spaces were measured with image analysis software. In 17.4% of testes spermatogenesis was normal (9.9 points) (N) and neoplasia was not found there. In the other 38 specimens (83%) spermatogenesis was abnormal (A). When spermatogenesis was arrested or when germ cells were absent (3.7+/-1.8 points), neoplastic lesions were found in 13.1% of the specimens. In A group 5.1+/-7.1% of tubules contained immature Sertoli cells, while in N they were not found. Tubular diameter was significantly lower in A (161.5+/-31.8 microm) than in N (184.6+/-24.3 microm) and the percentage of seminiferous tubules with the thickening of tubular basal membrane was also greater in A. Intertubular spaces were significantly larger in A (49.9+/-18.6%) in comparison to N group (32.6+/-12.5%). Mean number of Leydig cells was similar in both groups. To conclude, in most of the formerly cryptorchid testes, despite surgical treatment, impaired seminiferous tubules differentiation is predominant. Germ cell neoplasia is present in testes with retarded seminiferous tubules differentiation. Retardation of seminiferous tubule differentiation consists of inhibited spermatogenesis, presence of tubules with immature Sertoli cells, decreased tubular diameter, increased thickness of basal membrane and enlarged intertubular spaces. Examination of testicular biopsy with respect to the state of seminiferous tubule differentiation may be helpful to predict the appearance of germ cell neoplasia in adult men with cryptorchidism in anamnesis. Orchiopexy of cryptorchid testes may not prevent the occurrence of features of testicular dysgenesis and the associated germ cell neoplasia

The risk of neoplasm associated with dysgenetic testes in prepubertal and pubertal/adult patients

Introduction. In patients with Y-chromosome in the karyotype, partial gonadal dysgenesis and disorders of male reproductive sex organs development are usually resected in childhood because of the high risk of germ cell tumours (GCT). In patients with Y-chromosome, complete gonadal dysgenesis and female genitalia gonadectomy is performed markedly later. However, due to the relatively low number of adult patients with preserved dysgenetic gonads, the true risk of neoplasm is unknown. The aim of the study was to evaluate the prevalence of neoplasia in dysgenetic gonads of children and adults with Y-chromosome in a retrospective study. Material and methods. A review of medical documentation of 94 patients with disorders of sex development (DSD), Y-chromosome and gonadal dysgenesis (GD), aged 1.2–32 years (47 prepubertal, 1.2–10 years; 47 pubertal/adult, 13–32 years), was conducted. Serum levels of follicle-stimulating hormone (FSH), luteinizing hormone (LH) and testosterone were determined. Bilateral gonadectomy was performed in 73.4% of patients, and unilateral gonadectomy with biopsy of the contralateral gonad in 26.4%. All gonadal tissues were subjected to immunohistochemical evaluation with antibodies against PLAP and OCT3/4 (markers of malignant germ cells, but also foetal multipotent germ cells), while gonads of prepubertal patients were examined by c-KIT, as well. Results. Streak gonads were identified on both sides (complete GD) in 30.8%, a streak gonad on one side and an underdeveloped testis on the other (asymmetric GD) in 38.3%, and underdeveloped testicular structure on both sides (partial GD) in 30.8% of cases. Germ cell neoplasia was found in 53.2% of patients (51.1% in children, 55.3% in pubertal/adults). Invasive GCT were identified in 11.7% of cases, of which 90.9% were in pubertal/adult patients. Other neoplastic lesions included gonadoblastoma (16% prevalence) and testicular carcinoma in situ (25.5%). In younger patients FSH serum levels were increased in 81% of cases (mean 2.82 ± 2.18 IU/L), while LH in 58% (mean 1.82 ± 1.69 IU/L). Hypergonadotropic hypogonadism was diagnosed in most of the pubertal/ /adult patients (mean FSH 54.2 ± 23.3 IU/L, mean LH 21.7 ± 12.1 IU/L, mean testosterone 5.5 ± 4.5 nmol/L). Conclusions. Dysgenetic gonads in patients with Y chromosome have a high risk of germ cell neoplasia (ca. 50%). If they are preserved until puberty/early adulthood, they may develop overt, invasive GCT. The gonads also have poor hormonal activity (hypergonadotropic hypogonadism) in most of the pubertal/adult patients. Each of these cases must be considered individually and a decision to remove the gonad or not should be based on the comprehensive analysis of the phenotype by a multidisciplinary team of specialists in consultation with the patient and the parents. If dysgenetic gonads are not resected in childhood, these patients need careful ongoing follow-up examination, including biopsy and histopathological evaluation.

Anorectal malformations in children – diagnostics, classification and management

Author presented anorectal malformations (ARM): their incidence, diagnostics, classification and associated anomalies. Author compared operative methods used in treatment of ARM and results of treatment in regards to the type of ARM. He discussed technical considerations of the posterior sagittal approach, a new operative method of author’s preference, as well as the use of anorectal manometry in assessment of postoperative function of the anorectal canal. The first description of ARM was presented by Paulus Aegineta in VII, however functionally effective operative methods appeared in the 70’s of last century: sacroperineal operation by Stephens and sacroabdominoperineal procedure by Kiesewetter. Though postoperative results were unsatisfactory till 1982 when de Vries and Peña introduced posterior sagittal anorectoplasty. The concept of approaching the defect through a wide posterior sagittal incision with division of all muscle structures exactly in the midline was based on the assumption that such an incision cannot damage the symmetrical, bilateral nerve and blood supply. The idea of the new operation is based on some elements of importance for the postoperative function of the rectoanal canal, i.e. use of electrostimulation, traction sutures and “tapering” - tailoring of the rectum to match the size of muscle structures. All previously used classifications of ARM were prepared mostly due to the necessity of choosing the proper corrective operations different for low and different for high ARM. Posterior sagittal approach allows correction of defect independent on type of ARM. It can also be used successfully in reoperations in incontinent children after correction of ARM with use of other operative technique.Autor omówił w pracy wady wrodzone odbytu i odbytnicy (WWOO): ich występowanie, rozpoznawanie, typy wad oraz wady towarzyszące. Autor porównał metody leczenia chirurgicznego WWOO oraz wyniki leczenia w poszczególnych typach wad. Następnie omówił techniczne aspekty dostępu tylnego strzałkowego, metody operacyjnej preferowanej przez autora, a także sposób pooperacyjnej oceny czynności kanału odbytniczo-odbytowego przy użyciu manometrii odbytniczej. Pierwszy opis WWOO przedstawił już w VII w.n.e. grecki chirurg Paulus Aegineta, ale skuteczne, w sensie czynnościowym, metody operacyjne pojawiły się dopiero w latach 70-tych ubiegłego wieku: operacja krzyżowo-kroczowa Stephens’a i operacja krzyżowobrzuszno- kroczowa Kiesewetter’a. Wyniki leczenia WWOO były jednak niezadowalające, aż do momentu wprowadzenia w 1982 roku przez de Vries i Peña operacji z dostępu strzałkowego tylnego (ang. posterior sagittal approach). Operacja polega na dotarciu do niedrożnego jelita od strony krocza poprzez rozdzielenie wszystkich struktur mięśniowych idealnie w linii pośrodkowej, opierając się na założeniu, że takie cięcie nie uszkadza symetrycznego, bilateralnego unerwienia i unaczynienia tej okolicy. Nowa technika operacyjna zawiera kilka elementów mających znaczenie w aspekcie pooperacyjnej czynności kanału odbytniczo-odbytowego, m.in.: użycie elektrostymulacji, zastosowanie szwów trakcyjnych i „tapering” czyli dopasowanie jelita rozmiarem do istniejących struktur mięśniowych. Poprzednio obowiązujące podziały WWOO uzasadnione były przede wszystkim koniecznością doboru odpowiedniej metody operacyjnej, innej w wadach typu niskiego i innej dla wad typu wysokiego. Dostęp strzałkowy tylny umożliwia korekcję każdej wady odbytu niezależnie od jej typu. Metoda może być użyta również w reoperacjach u pacjentów z niezadowalającym wynikiem czynnościowym po operacji innymi metodami

Czy przetoka odbytniczo-kroczowa w wadach odbytu i odbytnicy jest ektopowym kanałem odbytu ze zwieraczem wewnętrznym odbytu?

Authors tried to determine the role of an internal anal sphincter (IAS) in patients with rectoperineal fistula undergoing PSARP, with or without IAS-saving procedure. Materials and methods: 20 girls and 6 boys with rectoperineal fistula undergoing PSARP between 1993-2008 were included in the study. An IAS-saving operation was performed in 19 children (73.1%), 17 girls and 2 boys, while 7 children underwent regular PSARP with resection of narrowed distal portion of fistula. Results: Functional postoperative result according to the „10” score was good (8-10 pts.) in all 26 children. There was no significant difference in RAP (16.3 vs. 16.9 vs. 17.2 cmH2O) and ACL (1.7 vs. 1.7 vs. 1.9 cm) values between children with and without preserved IAS and the reference group. Positive RAIR was observed in 13 of 19 children with preserved IAS, and in 2 of 7 patients after resection of IAS, but this incidence did not differ statistically (p = 0.095). The major functional disorder in the examined children was constipation, observed in 9 children after sphincter-saving procedure and in 2 patients after regular PSARP, but the difference was not significant (p = 0.658). Conclusions: An IAS is important, but not a decisive factor in fecal continence and IAS-saving procedures are associated with high incidence of constipation. Authors suggest that IAS can be spared, wherever it was possible, i.e. the fistula is wide (at least 8 mm) and it will not impair function of the neoanus.Autorzy podjęli próbę oceny roli zwieracza wewnętrznego odbytu (ZWO) u pacjentów z przetoką odbytniczo-kroczową po operacji wady odbytu metodą PSARP, z wycięciem lub oszczędzeniem ZWO. Materiały i metody: Badaniami objęto grupę 20 dziewczynek i 6 chłopców z przetoką odbytniczo-kroczową operowanych metodą PSARP w latach 1993-2008. Operację oszczędzającą ZWO przeprowadzono u 19 dzieci (73,1%), 17 dziewczynek i 2 chłopców, natomiast u 7 pacjentów wykonano operację PSARP z wycięciem zwężonego, dystalnego odcinka przetoki. Wyniki: Pooperacyjny wynik czynnościowy wg skali „10” był dobry (8-10 pkt.) u wszystkich 26 dzieci. Nie stwierdzono istotnej różnicy w wartościach RAP (16,3 vs. 16,9 vs. 17,2 cmH2O) i ACL (1,7 vs. 1,7 vs. 1,9 cm) u dzieci z i bez ZWO oraz grupą referencyjną. RAIR wywołano u 13 spośród 19 dzieci z zachowanym ZWO oraz u 2 spośród 7 pacjentów bez ZWO, bez różnicy statystycznej (p = 0,095). Najpoważniejszym zaburzeniem czynnościowym u dzieci były zaparcia obserwowane u 9 pacjentów z zachowanym ZWO i u 2 dzieci po klasycznej operacji PSARP, bez różnicy statystycznej (p = 0,658). Wnioski: ZWO jest ważnym, ale nie decydującym czynnikiem w mechanizmie trzymania stolca, a operacjom oszczędzającym ZWO towarzyszy wysoki odsetek zaparć w okresie pooperacyjnym. Autorzy sugerują oszczędzenie ZWO, jeżeli jest to możliwe, tzn. ujście przetoki jest szerokie (co najmniej 8 mm) i nie upośledza to czynności nowego odbytu

Speciation analysis of arsenic, antimony and selenium in Lednica lake water

The paper describes the results of determination of different forms arsenic, antimony and selenium present in lakes water (As, Sb (III), As, Sb (V), Se (IV) and their organic forms). The studies of speciation analysis of ultratraces metal concentrations determined by hydride generation atomic absorption spectrometry give the imagination of processes are proceeding in water environment

Skręt wyrostka robaczkowego jako przyczyna „ostrego brzucha” u 6-letniego chłopca

The authors present a rare case of acute abdomen in a 6-year-old boy caused by torsion of the vermiform appendix. An analysis of the clinical manifestation, mechanism, usefulness of imaging techniques and treatment of torsion based on the review of literature and personal experience was performed.Autorzy prezentują rzadki przypadek „ostrego brzucha” u 6-letniego chłopca, wywołanego skrętem wyrostka robaczkowego. Na podstawie przeglądu piśmiennictwa i własnych doświadczeń przeanalizowano obraz kliniczny, przyczyny, przydatność badań obrazowych i leczenie skrętu wyrostka

Epididymal Cysts in Childhood - Conservative or Surgical Approach?

The aim of the study. To decide on the accurate way of treatment and to establish criteria for operation in boys with pubertal epididymal cysts (ECs).Material and methods. Results of scrotal ultrasound of 363 boys and adolescents, aged 2 months to 18 years, were reviewed retrospectively.Results. Of all 363 patients with scrotal ultrasound 59 (16.2%) at mean age of 14.03 yrs had ECs. The EC incidence increased with age and 42 out of 124 boys (33.8%) older than 14 yrs had cysts (chi2=27.627, p=0.000). Out of 59 patients, in 30 (50.8%) cysts were diagnosed incidentally at the time of scrotal US, 29 boys (49.2%) presented with scrotal mass and/or pain. 31 patients with ECs (52.5%) underwent elective surgery and the remaining 28 boys (47.5%) received conservative treatment. The age of boys with ECs who underwent surgery ranged from 8 to 18 years (mean 14.32). The age range of patients treated conservatively was 7-18 (mean 13.71). There was no statistical difference in age between boys treated surgically and conservatively (t=0.924, p=0.36). ECs resolved in 17 patients out of 28 boys treated conservatively, in remaining 11 boys the size of cysts was stabile and they remain asymptomatic. Clinical and ultrasonographic follow-up were carried out from 11 months to 5 years.Conclusions. ECs are more common in older boys (over 14 years). Management of ECs smaller than 10 mm should be conservative with clinical and ultrasound controls, leaving surgery for cysts increasing in size over 10 mm which did not involute with time