Wyniki wyszukiwania - Biblioteka Nauki

1

Clinical Spectrum of Malrotation in Children

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Bagłaj M. , Patkowski D. , Czernik J. , Hilger T.

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2007

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tom 79

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nr 4

286-296

EN

The aim of the study. Retrospective analysis of diagnosis, surgical management and final outcome of malrotation in children.Material and methods. Clinical charts, operative notes and imaging studies of all children operated on for malrotation in years 1985-2005 were studied. Children in whom malrotation was diagnosed incidentally during an operation performed because of other indications were excluded. A detailed analysis of the presentation, signs and symptoms, clinical course, and diagnostic modalities used in affected patients was carried out. Anatomical variants of malrotation and their operative management were evaluated as well.Results. In the period from 1985 to 2005 forty children with malrotation were treated. There were 25 neonates, 10 infants and 5 children older than 1 year of age. Thirty-five children were referred directly as inpatients from other hospitals. An acute or urgent clinical presentation was noted in 32 children, while in 8 patients as the presentation was subacute or chronic. In all children bile-stained vomiting was the predominant sign. In 21 children, ileus was the referral diagnosis. Only 5 children were admitted with an established diagnosis of malrotation. In six cases duodenal atresia was suspected. Delay in diagnosis of congential intestinal anomaly was identified in 15 children. In five newborns, the initial diagnosis was enterocolitis. Four other patients, including one infant and three older children, were admitted to the pediatric departments several times before receiving the final diagnosis. In 18 children, the only pre-operative imaging was abdominal plain X-ray. In 22 patients, contrast studies were performed as well. All children underwent operative management. Ladd's syndrome was diagnosed in 16 patients, and in another 7 children was associated with small bowel torsion. Isolated small bowel torsion was noted in 7 patients. In 6 patients, midgut volvulus with strangulation was found intraoperatively. Two children with typical anatomical features of non-rotation presented with recurrent episodes of ileus. In single cases, mesocolic internal hernia and sigmoid torsion were noted. 5 children died postoperatively, four of whom had midgut volvulus with massive small bowel necrosis.Conclusions. Malrotation is a rare congenital anomaly that can present with a wide anatomical and clinical spectrum but should be considered in the differential diagnosis for all children, regardless of age, manifesting with bile-stained vomiting. Suspicion of malrotation seems to be an obvious indication for referral to a tertiary pediatric surgical department where necessary imaging should be undertaken with the participation of an experienced radiologist. Regardless of its clinical presentation, malrotation should be considered an unequivocal indication for surgical treatment due to risk of midgut volvulus with its serious sequelae.

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Late-Presenting Congenital Diaphragmatic Hernia in Children

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Bagłaj M. , Ładogórska J. , Czernik J.

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2007

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tom 79

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nr 4

305-311

EN

Congenital diaphragmatic hernia (CDH) is a typical neonatal emergency. There is, however, a subset of patients who manifest this congenital anomaly in infancy and early childhood. Late-presenting diaphragmatic hernia is characterized by a variety of gastrointestinal and respiratory symptoms with acute or chronic modes of manifestation.The aim of the study. Analysis of the clinical course, diagnosis, and surgical management of latepresenting CDH in children.Material and methods. A retrospective study of medical charts and diagnostic studies was performed for children older than 1 month of age with CDH, treated in the University Department of Pediatric Surgery and Urology in Wroclaw between 1975 and 2005.Results. In a 30-year period, 23 children with late-presenting CDH were treated. In one case, paraoesophageal hernia was diagnosed; Morgagni hernia was diagnosed in 2 cases. Among 20 children with postero-lateral CDH, 12 were younger than 1 year of age at diagnosis. In this group, left-sided CHD was noted in 17 children, while a right-sided defect was noted in 3. Nine infants with left CDH presented mainly respiratory symptoms; in 3 cases gastrointestinal symptoms prevailed. In the group of children beyond 1 year of age, 6 manifested chronic respiratory symptoms and 2 had mainly acute gastrointestinal complaints. In all children, CDH was diagnosed preoperatively. The most accurate diagnostic modality in 17 cases was a contrast upper GI study. Intraoperative findings revealed the presence of a hernial sac in only 3 patients. A complicated course of CHD with torsion of the stomach was noted in 2 children; intestinal necrosis was seen in one patient. Small and large bowel were the organs most commonly displaced into the thoracic cavity (noted in 12 and 11 children, respectively). In all children, surgical management of CDH was successful.Conclusions. Lack of typical clinical signs and symptoms of late-presenting CDH seems to be a factor responsible for delayed diagnosis of this anomaly in older children. Late presentation of CDH should be taken into differential diagnosis in all children presenting with gastrointestinal or respiratory symptoms. Late-presenting diaphragmatic hernia is an unequivocal indication for surgical management as soon as its diagnosis has been established.

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Diagnostyka prenatalna wad wrodzonych

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Wojtyłko-Gołowkin A. , Bagłaj M. , Wojtyłko A.

Puls Uczelni

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2014

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nr 1

26-29

PL

Diagnostyka prenatalna oferuje szerokie spektrum badań możliwych do przeprowadzenia na różnych etapach zaawansowania ciąży w celu wczesnego rozpoznania wad wrodzonych. Celem niniejszej publikacji jest omówienie metod badań prenatalnych, określenie ich przydatności i dostępności. Wykrycie nieprawidłowości anatomicznych in utero i postnatalna weryfikacja rozpoznania w sposób istotny zmniejszają u dziecka ryzyko związane z ewentualną wadą rozwojową. Szczególnie ważne jest prenatalne wykrycie wady, wymagającej interwencji chirurgicznej w pierwszych dniach życia. Wewnątrzmaciczne zdiagnozowanie wady pozwala wdrożyć postępowanie diagnostyczne i lecznicze u noworodka bezpośrednio po urodzeniu. Transport in uterojest najbezpieczniejszym sposobem przekazania dziecka do ośrodka wielospecjalistycznego. Zespół konsultacyjny opiekujący się matką i noworodkiem obarczonym wadą wrodzoną powinien składać się ze specjalistów z zakresu położnictwa, neonatologii, pediatrii, anestezjologii, chirurgii dziecięcej i genetyki. W przypadku prenatalnego podejrzenia lub wykrycia wady wymagającej interwencji chirurgicznej, właściwe wydaje się włączenie do zespołu terapeutycznego chirurga dziecięcego w momencie rozpoznania wady w okresie prenatalnym. Zgodnie z rekomendacją PTG zaleca się, aby każda ciężarna miała wykonane USG płodu przynajmniej 3-krotnie podczas trwania ciąży. Minimalnie inwazyjny screening zarezerwowany jest dla wszystkich ciężarnych kobiet w Polsce, bez względu na wiek. Badania inwazyjne proponowane są w przypadku dodatnich badań przesiewowych (obecność sonograficznego markeru zespołu genetycznego lub nieprawidłowego wyniku testu biochemicznego) lub u ciężarnych z obciążonym wywiadem i w wieku powyżej 35 r. ż.

EN

Prenatal diagnosis offers a wide range of tests that can be carried out at various stages of pregnancy in order to conduct early diagnosis of congenital malformations. The purpose of this publication is to discuss the methodology, suitability and availability of prenatal testing. The detection of anatomical abnormalities in utero and postnatal verification of the diagnosis decreases the risk associated with malformation in a significant way. Prenatal detection of the defect requiring the surgical intervention on the first days of the life is particularly important. Intrauterine diagnosing of the defect allows to implement the diagnostic and healing progression at the newborn directly after the birth. Transport in utero is the safest way of transmitting the child to the high- specialistic centre . The consultative team looking after the mother and the newborn with an inborn defect should be composed of specialists from obstetrics, neonatology, pediatrics, anesthesiology, pediatric surgery and genetics. In the case of prenatal suspicion or detection of the defects that are possible to surgically repair, it seems appropriate to incorporate a pediatric surgeon to the therapeutic team in the moment of detection in the prenatal period. According to the Polish Gynecological Association it is recommended for every pregnant woman to have an ultrasound scan of the foetus at least 3 times during pregnancy. Minimally invasive screening is destined to all pregnant women in Poland, irrespective of the age. Invading examinations are proposed in case of the positive screening (presence of genetic sonographic marker or wrong biochemical test results) or for pregnant women with the past medical history and at the age above 35.