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Fetal Megacystis: a New Morphologic, Immunohistological and Embriogenetic Approach

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Fetal Megacystis: a New Morphologic, Immunohistological and Embriogenetic Approach applied sciences Article Fetal Megacystis: A New Morphologic, Immunohistological and Embriogenetic Approach Lidia Puzzo 1,*, Giuliana Giunta 2, Rosario Caltabiano 1 , Antonio Cianci 2 and Lucia Salvatorelli 1 1 Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, Azienda Ospedaliero-Universitaria “Policlinico-Vittorio Emanuele”, Anatomic Pathology Section, School of Medicine, University of Catania, 95123 Catania, Italy; [email protected] (R.C.); [email protected] (L.S.) 2 Department of General Surgery and Medical Surgical Specialties, Department of Obstetrics and Gynecology-Policlinico Universitario G. Rodolico, University of Catania, 95123 Catania, Italy; [email protected] (G.G.); [email protected] (A.C.) * Correspondence: [email protected]; Tel.: +39-095-3782026; Fax: +39-095-3782023 Received: 23 September 2019; Accepted: 26 October 2019; Published: 28 November 2019 Abstract: Congenital anomalies of the kidney and urinary tract (CAKUT) include isolated kidney malformations and urinary tract malformations. They have also been reported in Prune-Belly syndrome (PBS) and associated genetic syndromes, mainly 13, 18 and 21 trisomy. The AA focuses on bladder and urethral malformations, evaluating the structural and histological differences between two different cases of megacystis. Both bladders were examined by routine prenatal ultrasound screening and immunohistochemistry, comparing the different expression of smooth muscular actin (SMA), S100 protein and WT1c in megacystis and bladders of normal control from fetuses of XXI gestational age. Considering the relationship between the enteric nervous system and urinary tract development, the AA evaluated S100 and WT1c expression both in bladder and bowel muscular layers. Both markers were not expressed in the bladder and bowel of PBS associated with anencephaly. In conclusion, megacystis could be considered only a macroscopic definition, concerning the size of the fetal bladder rather than the embryologic origin; it may be a single or multiple malformation; the possible association with the bowel and/or encephalic malformations will decide the outcome and prognosis in fetal megacystis. Keywords: immunohistochemistry; urinary tract malformations; megacystis; enteric nervous system; outcome and prognosis 1. Introduction Congenital anomalies of the kidney and urinary tract (CAKUT) are the most common congenital malformations, with a frequency of 3–6 per 1000 live births. They include: isolated kidney malformations (agenesis, hypo-dysplasia, multicystic renal disease, ureteropelvic junction obstruction) and urinary tract malformations (megaureters, megacystis, posterior urethral valve (PUV), urethral atresia/obstruction, urogenital sinus and cloacal malformations, obstructive ureterocele). CAKUT have also been reported in Prune-Belly syndrome (PBS) and associated genetic syndromes, mainly 13, 18 and 21 trisomy [1]. The fetal bladder may be viewed and evaluated by ultrasound from the 12th gestational week, as a pelvic, oval, anechogen structure less than 6 mm of sagittal diameter. An increased sagittal diameter of the fetal bladder has been considered as megacystis, regardless of etiologic factors and macroscopic features. Therefore, prenatal megacystis may be considered mainly an ultrasound diagnosis [1,2]. Appl. Sci. 2019, 9, 5155; doi:10.3390/app9235155 www.mdpi.com/journal/applsci Appl. Sci. 2019, 9, 5155 2 of 9 Appl. Sci. 2019, 9, x 2 of 10 We aimed to evaluate the structural and histological differences between two megacystis diagnosed by routineWe prenatalaimed to ultrasoundevaluate the screening structural in and the Obstetricalhistological anddifferences Gynecological between Clinic, two megacystis University of diagnosed by routine prenatal ultrasound screening in the Obstetrical and Gynecological Clinic, Catania, Italy, and examined in the Pathologic Anatomy Section of G.F. Ingrassia Department, University University of Catania, Italy, and examined in the Pathologic Anatomy Section of G.F. Ingrassia of Catania. Department, University of Catania. 2. Materials and Methods 2. Materials and Methods Two fetuses with urinary tract malformations were examined in the Pathologic Anatomy Section Two fetuses with urinary tract malformations were examined in the Pathologic Anatomy of the G.F. Ingrassia Department, University of Catania, Italy. The study was conducted in accordance Section of the G.F. Ingrassia Department, University of Catania, Italy. The study was conducted in with with the Declaration of Helsinki, and the protocol was approved by the Ethics Committee of accordance with with the Declaration of Helsinki, and the protocol was approved by the Ethics CataniaCommittee 1 (48102 of Catania of 7 November, 1 (48102 accordingof November to national 7, according legislation to national about legislation osservational about studies, osservational 20 March 2008,studies, AIFA). March 20, 2008, AIFA). Case 1: 19th week termination of pregnancy (TOP); male, 330 gr; total length 24 cm; crown rump Case 1: 19th week termination of pregnancy (TOP); male, 330 gr; total length 24 −cm; (CR)crown length−rump 15.3 (CR) cm; mediallength length15.3 cm; of footmedial 3 cm; length cranial of circumferencefoot 3 cm; cranial 15.5 cm;circumference thoracic circumference 15.5 cm; 13thoracic cm; abdominal circumference circumference 13 cm; abdominal 11 cm. Ruby circumference red skin; 11 perforated cm. Ruby orifices. red skin; Umbilical perforated cord orifices. stump 10Umbilical0.7 cm; cord edema stump of penis. 10 × 0.7 The cm; anatomic edema of relationship penis. The anatomic of thoracic relationsh organsip was of thoracic normal: organs heart inwas situs × solitusnormal: with heart atrium in situsventricular solitus andwith ventricular atrium−ventricularvascular and concordance ventricular and−vascular lung development concordance coherent and − − withlung gestational development age. coherent Examination with gestational of the abdominal age. Examination cavity showed: of the abdominal megacystis cavity (25 mm showed: sagittal diameter)megacystis with (25 thickened mm sagittal wall diameter) (6 mm), megaureter with thickened with stenosiswall (6 ofmm), ureteral megaureter orifices with in the stenosis bladder, of and bilateralureteral hydronephrotic orifices in the kidneysbladder, (both and 18bilateral mm maximum hydronephrotic diameter). kidneys (both 18 mm maximum diameter).Ecographic diagnosis: urinary obstruction syndrome due to posterior urethral valve (PUV). The US (ultrasonographic)Ecographic diagnosis: examination urinary showed obstruction a megacystis syndrome with due a maximum to posterior diameter urethral of 44valve mm (PUV). and mild hydroureteronephrosisThe US (ultrasonographic) with examination bright hyperechogenic showed a megac kidneys.ystis The with bladder a maximum had the diameter so-called of “key44 mm hole sign”,and suggestingmild hydroureteronephrosis a possible PUV. Fetal with biometry bright hyperechogenic and amniotic fluidkidneys. were The appropriate bladder had for gestationalthe so- called “key hole sign”, suggesting a possible PUV. Fetal biometry and amniotic fluid were age (GA). No other major abnormalities were detected (Figure1). appropriate for gestational age (GA). No other major abnormalities were detected (Figure 1). FigureFigure 1. 1. CaseCase 1,1, US (ultrasonographic) (ultrasonographic).. (A) ( ASagittal) Sagittal view view of the of entire the entire fetus fetuswith megac with megacystis.ystis. (B) Coronal view of the bladder with the keyhole sign. (C) Coronal view of the hyperechogenic kidneys. (B) Coronal view of the bladder with the keyhole sign. (C) Coronal view of the hyperechogenic kidneys. Courtesy of Dr. G. Giunta. Courtesy of Dr. G. Giunta. CaseCase 2: 2: 15th 15th weekweek TOP; XXY XXY karyotype karyotype;; 40 40 gr; gr; total total length length 11 cm; 11 cm;crown crown−rumprump (CR) length (CR) length 8.5 cm; medial length of foot 1.5 cm; cranial circumference 7 cm; thoracic circumference− 6 cm; 8.5 cm; medial length of foot 1.5 cm; cranial circumference 7 cm; thoracic circumference 6 cm; abdominal circumference 7 cm. Ruby red skin; imperforated orifices. Turricephal and anencephal abdominal circumference 7 cm. Ruby red skin; imperforated orifices. Turricephal and anencephal skull, prognathism, prominent ocular bulbs and low implant of the ears. The abdominal wall was skull, prognathism, prominent ocular bulbs and low implant of the ears. The abdominal wall was flaccid due to the incomplete development of the diaphragm and ribs. The abdominal organs were flaccid due to the incomplete development of the diaphragm and ribs. The abdominal organs were herniated in the thoracic cavity and in the neck across the skin. Megacystis (65 mm sagittal herniated in the thoracic cavity and in the neck across the skin. Megacystis (65 mm sagittal diameter) diameter) with a thinned wall (2 mm) occupied the abdominal and thoracic cavities; both kidneys withand a surrenal thinned glands wall (2 were mm) underdeveloped occupied the abdominal (both with and a maximum thoracic cavities;diameter both of 10 kidneys mm). and surrenal glandsEcographic were underdeveloped diagnosis: Prune (both- withBelly asyndrome, maximum PBS. diameter The ofultrasonographic 10 mm). (US) examination wasEcographic very difficult diagnosis: due to maternal Prune-Belly BMI syndrome,> 35 and the PBS. anhydramnios The ultrasonographic—absence of (US) amniotic examination fluid. The was veryfetus di ffishowedcult due a megacystis
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