An innovative surgical treatment method in persistent rectal prolapse: Ventral abdomino-rectosigmoidopexy through tube sigmoidostomy combined with Ekehorn's rectopexy.

OBJECTIVE: Herein, the results of the cases, who underwent surgical repair with or without ventral abdomino-rectosigmoidopexy through tube sigmoidostomy combined with Ekehorn's rectopexy due to recurrent rectal prolapse, were discussed. METHODS: The demographic characteristics, surgical technique, and results of children who were operated in the department of Pediatric Surgery for rectal prolapse between 2004 and 2022 were retrospectively analyzed. RESULTS: In 18 years, six pediatric cases (2 females [33%] and 4 males [67%]) were operated for persistent rectal prolapse. The mean operative age of the patients was 7.5 years (2.1-17), and all had severe rectal prolapse. Some of these patients were followed up in other centers and their rectal prolapse continued despite diet changes, toilet behavior training, and the treatment of sclerosing agents. Rectal trimming was applied to one of the first two patients who were operated for anal atresia and recurrence did not occur. In the second case who underwent laparoscopic colon pull-through, Ekehorn rectopexy was performed alone and no recurrence was observed also in this case. Considering that rectosigmoid colon adhesions formed on the anterior abdominal wall due to colostomy opening-closing may provide ventral sigmoidopexy, it was decided to offer the option of applying both methods together. Three of the next four cases were diagnosed with cystic fibrosis. All four underwent ventral abdomino-rectosigmoidopexy through tube sigmoidostomy combined with Ekehorn's rectopexy. Ekehorn's butterfly sutures were removed on 15th day and Foley catheters on 21st day. Three cases with cystic fibrosis were uneventful. However, a 14-year-old girl with a history of sexual abuse relapsed 6 months later. CONCLUSION: Ventral abdomino-rectosigmoidopexy through tube sigmoidostomy combined with Ekehorn's rectopexy is a successful and unique method in terms of providing intestinal fixation. It may be the primary option for definitive surgical treatment of persistent rectal prolapse.

An analysis of factors affecting survival in prenatally diagnosed omphalocele.

OBJECTIVES: To estimate factors affecting survival in prenatally diagnosed omphalocele, factors predicting genetic abnormalities, and association of omphalocele and specific groups of anomalies. METHODS: A retrospective observational study was performed, analyzing data of all omphalocele cases diagnosed prenatally in the perinatology clinic of a referral center. Demographic data, characteristics of the omphalocele (size, content, associated anomalies), results of genetic testing, pregnancy outcomes and postnatal outcomes were analyzed. RESULTS: Sixty-nine fetuses with omphalocele were included. The prevalence of omphalocele in livebirth was 0.007 %. Overall survival during the study period was 73.9 %. Twenty-eight (71.7 %) out of 39 cases with associated anomalies who were born live, survived, whereas survival was 85.7 % in the isolated cases. The most common anomaly associated with omphalocele were cardiac defects with 42 %; followed by placental or umbilical cord anomalies (28.9 %), skeletal defects (27.5), genitourinary anomalies (20.2 %), central nervous system (18.8 %) and facial anomalies (7.2 %), respectively. Eighty-five percent of the fetuses had at least one additional anomaly or ultrasound finding. Skeletal abnormalities and staged surgical repair of omphalocele were associated with survival. Associated skeletal anomalies and staged repair significantly increase the risk of postnatal death (OR: 4.6 95 % CI (1.1-19.5) and (OR: 10.3 95 % CI (1.6-63.9), respectively). CONCLUSIONS: Associated skeletal abnormalities and staged surgical repair are negatively associated with postnatal survival.

Twelve years of experience in the treatment of newborns with intrauterine gastrointestinal perforation.

BACKGROUND: Meconium peritonitis occurs when meconium leaks into the peritoneal cavity as a result of intrauterine gastrointestinal perforation. In this study, we aimed to evaluate the results of newborn patients who were followed and treated due to intrauterine gastrointestinal perforation in the pediatric surgery clinic. METHODS: All newborn patients who were followed up and treated for intrauterine gastrointestinal perforation in our clinic between December 2009-2021 were analyzed retrospectively. Newborns who had no congenital gastrointestinal perforation were not included in our study. The data were analyzed using NCSS (Number Cruncher Statistical System) 2020 Statistical Software. RESULTS: Within twelve years, intrauterine gastrointestinal perforation was detected in 41 newborns, including 26 (63.4%) males, and 15 (36.6%) patients who were operated on in our pediatric surgery clinic. Surgical findings of 41 patients diagnosed with intrauterine gastrointestinal perforation revealed the presence of volvulus (n=21), meconium pseudocyst (n=18), jejunoileal atresia (n=17), malrotation-malfixation anomaly (n=6), volvulus due to internal hernia (n=6), Meckel`s diverticulum (n=2), gastroschisis (n=2), perforated appendicitis (n=1), anal atresia (n=1), and gastric perforation (n=1). Eleven patients (26.8%) died. Total intubation time was significantly higher in deceased cases. Postoperatively, deceased cases passed their first stool significantly earlier than surviving newborns. Besides, ileal perforation was seen significantly more frequently in deceased cases. However, the frequency of jejunoileal atresia was significantly lower in the deceased patients. CONCLUSIONS: Although sepsis has been held primarily responsible for the deaths in these infants from past to present, insufficiency in lung capacity necessitating intubation negatively affects their survival. Early passage of stool is not always an indicator of good prognosis after the operation, and patients may die due to malnutrition and dehydration, even after they are discharged after feeding, defecating and having weight gain.

Fetal hydrometrocolpos: Challenges of prenatal differential diagnosis on ultrasound.

Hydrometrocolpos is a pelvic cystic mass representing the distension of the vagina and uterus due to a lower genital tract obstruction causing accumulation of utero-cervical-vaginal secretions or urine in the vagina and endometrial cavity. Prenatal diagnosis is uncommon and differential diagnosis of the underlying etiologies is quite challenging in the prenatal period. We present three cases of female fetuses with hydrometrocolpos and discuss the prenatal differential diagnoses in the light of ultrasound findings along with postnatal diagnoses and outcomes.

Can we predict the need for postnatal surgery in patients with prenatal fetal lung masses detected by CVR value?

AIM: The purpose of this study is to evaluate whether CPAM-volume ratio (CVR) can predict postnatal management (follow up for resolution and surgical treatment) in fetuses with fetal lung masses in the prenatal period. MATERIALS AND METHODS: 44 patients who presented at our center with prenatally diagnosed CPAM (Congenital Pulmonary Airway Malformation) and BPS (Bronchopulmonary Sequestration) were analyzed. Obstetric history and outcomes, karyotype results, CVR, additional sonographic findings, characteristics of masses were recorded. CVR was calculated for all cases. In the study we sought to identify a CVR threshold and did not use the thresholds classically used in the literature. RESULTS: 20 fetal BPS and 24 CPAM cases were analyzed. After excluding 5 patients, 46% of the patients were diagnosed with BPS and 54% with CPAM. In this study the cut off < 0,53 for CVR is taken, it predicts the no need for postnatal surgery with a sensitivity of 85% and a specificity of 88%. When we take the > 0,76 cut-off value for patients who will require emergency surgery within the first 10 days, it predicts the need for surgery with 90% sensitivity and 89% specificity. In addition, it was determined that all patients with mediastinal shift were operated. CONCLUSION: We believe that the CVR value and the presence of mediastinal shift should be evaluated in all cases of CPAM and BPS for prediction of the surgery. Proper counseling about the prognosis could be given to the family in cases with mediastinal shift and CVR value above 0,76.

Prenatal diagnosis and treatment of intrahepatic arteriovenous fistulas: case reports and the literature review.

BACKGROUND: Congenital arteriovenous intrahepatic fistulas, which are hepatic hemangiomas and arteriovenous malformations (AVMs) are rare and they confused with each other. Knowledge of prenatal medical treatment of AVMs is insufficient. OBJECTIVES: First is to emphasize the distinction between hepatic hemangioma and AVMs. Second is discussion of the first case of hepatic AVM that responded well to steroid-propranolol treatment in the prenatal period. METHODS: Color Doppler ultrasonography, fetal and postnatal MR were used for diagnosis. RESULTS: The first case is a giant hepatic hemangioma diagnosed and progressively growing in the prenatal period and gradually shrinking in the postnatal period. The second case was hepatic AVM with no signs of heart failure during the prenatal period and postnatal right extended hepatectomy was performed as the anastomosis was enlarged and intraportal collateral vessels were developed. The third case is the first hepatic AVM which reaches a term that was prenatally diagnosed and responded to treatment with marked reduction. CONCLUSION: Color flow and pulse Doppler imaging have a key role in the prenatal diagnosis of arteriovenous fistulas. Intrahepatic AVM are abnormal intrahepatic vascular network formation primarily fed by the hepatic artery or its branches and drained by the hepatic venous system. This vascular region looks like a mass, but it does not contain a solid area, which allows the separation of hepatic AVMs from hepatic hemangiomas. Steroid and propranolol therapy should be considered in management.

Natural history of fetal ovarian cysts in the prenatal and postnatal periods.

PURPOSE: To determine the natural history of fetal ovarian cysts and to investigate whether the prognosis can be predicted by prenatal ultrasonography (US). METHODS: This retrospective study includes cases of fetal ovarian cysts diagnosed by prenatal US over a 6-year period. Cases were divided into four subgroups of cysts (small and simple, small and complex, large and simple, large and complex) according to their size and echotexture. US examinations were repeated every 2 weeks from the time of diagnosis to treatment. RESULTS: A total of 37 cases were included in the study. 32.4% of the cases regressed spontaneously in the prenatal period and 32.4% did so in the infantile period. Prenatal resolution occurred more frequently with small cysts than with large cysts (p = 0.03). Neonates with complex cysts required surgical treatment more often than neonates with simple cysts (p = 0.009). 27.0% of the cases underwent surgery due to ovarian torsion. The torsion rate of fetal ovarian cysts that progressed in the prenatal period was significantly higher than in the case of stable cysts (p = 0.001). CONCLUSION: The size of the fetal ovarian cysts, their US appearance and the progression of the cysts during follow-up are the main determinants of the neonatal outcome.

Safety and Efficiency of Pyeloplasty in The First Six Weeks of Infants' Life.

PURPOSE: The aim of this study was to assess the safety and the efficiency of pyeloplasty in infants with ureteropelvic junction obstruction (UPJO) in the first six weeks of their life. MATERIALS AND METHODS: Clinical records of the patients who had surgery during first six weeks of life for UPJO between June 2009 and June 2014 were analysed retrospectively. RESULTS: In this period, twenty-six dismembered pyeloplasties were performed in twenty-four patients on mean operation age of 27.3 ± 10.2 days (range 8-42 days). On the first postnatal ultrasound all twenty-six renal units had SFU-4 hydronephrosis. Mean preoperative and postoperative anterior-posterior pelvic diameter and parenchymal thickness were 33.1 ± 8.9mm (range 14-49mm), 3.2 ± 1mm (range 1-4,6mm) and 14.7 ± 6.6mm (range 6-27mm) and 7.8 ± 1.9mm (range 3.0-10.4mm), respectively. The differences between preoperative and postoperative parenchymal thickness and anterior-posterior pelvic diameter were statistically significant (P ˂ 0.0001). Preoperative MAG3 dynamic renal scintigraphy showed obstructive pattern on the diuretic renogram in 26 units. Mean preoperative and postoperative differential renal function on dynamic renal scintigraphy of the affected renal units were 46 ± 15 and 44 ± 15, respectively. Postoperative drainage was normal on dynamic renal scintigraphy in 25 (96.2%) of the 26 units, redo-pyeloplasty was needed in only one unit (3.8%). CONCLUSION: In conclusion, patient selection and timing of surgery are very important in the protection of renal function in newborn with UPJO. In our opinion, if there is indication for surgery, early surgical intervention should not postpone in this period. Surgical treatment of UPJO during first six weeks of life is safe and effective.

Expression of androgen, estrogen, and progesterone hormone receptors in the penile tissues of children with different types of hypospadias.

OBJECTIVE: Androgen (AR), Estrogen (ER) and Progesterone (PR) hormones play an important role in the prenatal and postnatal development of urogenital tract and especially the penis. The expressions of AR, ER and PR receptors in penile tissues in children with hypospadiases had also been shown previously. In this leading study, to demonstrate of the sex hormone receptor expression in cases with different types of hypospadias were aimed. METHODS: This study was designed in children operated due to hypospadiases without DSD. Biopsy samples of 3 mm's were obtained from three different sytes as the lateral parameatal tissue and the anterior corner of the prepuce, and inner layer of posterior prepuce. The presence of AR, ER and PR receptors was investigated immunehistochemically. RESULTS: Mean age was 5.4 years in 18 children with hypospadiases; in totally 33 specimens were taken in 5 subcoronal as 5 specimens, and 7 penile as 15 specimens, and 6 penoscrotal as 13 specimens. According to sytes of samples; 13 samples were from lateral para-meatal tissues, and 13 were from anterior corners of prepuces, and 7 were from inner layers of posterior prepuces. In regard to receptor expression; ER and AR receptors were positive in 29 (87.8%) and 12 (36.4%) respectively; PR receptors were negative. CONCLUSION: This study emphasized the dominant expression of estrogen receptors in penile tissues of children with hypospadias. Although there was not a manifest correlation of androgen receptors absence in regard to the severity of hypospadias patients, there was a marked estrogen receptors presence in penile tissues. These findings suggest that the disrupted androgen and estrogen receptor interaction and/or balance could play a role during the development of external genitalia in hypospadias patients. Progesterone receptor was not present and therefore the active role in the postnatal development of hypospadias is still debatable.

Management of Transverse Testicular Ectopia with Persistent Mullerian Duct Syndrome.

According to additional anomalies, transverse testicular ectopia (TTE) is classified into three groups. Type-2 TTE, accompanied by persistent mullerian duct syndrome, constitutes approximately 20% of the patients. Surgical treatment should be planned after careful physical examination, ultrasonography, and genetic/endocrinologic evaluation. Herniorrhaphy, orchiopexy with testicular biopsy, and excision of the mullerian structures are the most appropriate surgical approaches in cases of TTE with persistent mullerian duct syndrome. We aimed to share our approach to the diagnosis and treatment of a patient with type-2 TTE. Possibility of TTE should be kept in mind in children with nonpalpable testis on one side and inguinal hernia on the other side.

Occurrence of paratubal cysts in childhood: An analysis of 26 cases.

A retrospective analysis was conducted on the case files of patients operated on for adnexal pathology between January 2004 and December 2015 at our institution. The data of the patients with a diagnosis of paratubal cysts (PTCs) was extracted. A total of eighty-seven children were operated on for adnexal pathology. Twenty-six (29.9%) patients had PTCs. The patients were predominantly grouped as adolescents (n=22) and newborns (n=3). One of the patients was a pre-pubertal girl. Of the 26 PTC cases, 18 of them occurred on the contralateral side to ovarian pathology (OP) or physiologic ovarian changes (POC) such as corpus luteum cyst or follicular cyst which their final diagnosis confirmed by histopathology examination; in one case, the PTCs were bilateral; and in three cases, PTCs were ipsilateral with OP or POC. There were only 4 solitary PTC cases identified among the 26 cases. Paratubal cysts can be observed at every age, even in newborns. Their association with OP or POC in childhood cases needs to be clarified to further understand the hormonal mechanisms that are influencing the occurrence of these cysts.

Prenatal diagnosis and outcomes of fetal teratomas.

PURPOSE: Our aim was to evaluate the diagnostic performance of ultrasonography (US) in the prenatal identification of teratomas and the perinatal outcome of the fetuses with those teratomas. METHODS: In this retrospective case series study, we searched the archives using the keywords "fetal mass" or "fetal tumor" or "fetal teratoma" and "sacrococcygeal teratoma," diagnosed between 2009 and 2014, within the US database of our center. RESULTS: One hundred seven fetuses were prenatally diagnosed as having a cystic or solid mass, tumor, or teratoma. Nineteen of those cases were diagnosed prenatally as having fetal teratoma, but that diagnosis could not be verified in three cases. In one fetus, the prenatal diagnosis could not be confirmed. The sensitivity of US in identifying fetal teratoma was 100% and the false-positive rate, 3.3%. Six pregnancies complicated by a fetal teratoma were terminated. A normal karyotype was identified in all fetuses that underwent karyotyping. Among the nine women who continued their pregnancy, polyhydramnios was identified in four fetuses; although high-output heart failure was also identified in two of those fetuses during prenatal follow-up, none developed hydrops. On delivery, nine infants were born alive, but three (33.3%) of them died within the early neonatal period. CONCLUSIONS: US has very high sensitivity and low false-positive rates in identifying fetal teratoma prenatally. The risk of chromosomal abnormalities is very low in fetuses with teratoma, and their prognosis depends on the location and size of the tumor and any associated perinatal complications.

The EXIT for Prenatally Diagnosed Cervical Cystic Teratoma: A Case Report.

The Ex-utero intrapartum treatment (EXIT) is a procedure performed during caesarean section while on fetal-placental circulation. We present a prenatally diagnosed cervical cystic mass causing tracheal compression which was managed successfully with the EXIT procedure.

Testicular cavernous hemangioma presenting as an ipsilateral lesion in a child with multicystic dysplastic kidney.

Childhood malignant testicle tumors are very rare. In the literature to date, it has been reported that there are only 24 cavernous hemangioma cases existing in English literature. In this study, we discuss a testicular mass case which was diagnosed as cavernous hemangioma. The patient, who followed for left multicystic dysplastic kidney since his birth, was admitted to our clinic with complaints of left testicular mass and pain at 1-year of age. Histopathological investigation revealed cavernous hemagioma. Even if it has its characteristic ultrasonographic findings, radiology is too far beyond to eliminate the malignancy, final diagnosis can only be made after orchiectomy.

Four-year retrospective look for acute scrotal pathologies.

OBJECTIVE: A group of diseases in the scrotum setting forth by the sudden swelling and redness and pain consist of acute scrotal pathologies. The most common causes of acute scrotum in children are epididymitis, epididymo-orchitis, orchitis, testicular torsion, torsion of the appendix testis, incarcerated inguinal hernia and traumatic hydro/hematocele. In this study; we aim to evaulate patients with acute scrotal pathologies who were interned in our department. METHODS: All hospital data of cases who were interned at our deparment due to acute scrotum in between June 2010-June 2014 were evaluated retrospectively. Cases with incarcerated inguinal herni were excluded in this study. RESULTS: In a 4-year-period 114 cases were interned in our department with acute scrotum. Mean age of the patients was 7.6±4.577 years (min: 1m-max: 18yrs). Doppler US was performed in 112 patients to evaluate the blood flow while in 2 patients applied after normal office hours were evaluated without Doppler US and operated under emergency conditions. The patients had received diagnosis of epididymitis/epididymo-orchitis/orchitis (n=83 cases; 72.8%), testicular torsion (n=24; 21.1%), torsion of the appendix testis (n=2; 1.8%) with and traumatic hydrocele/ hematocele (n=5; 4.4%). While detorsion was performed in 18 (75%) cases with testicular torsion and orchiectomy in 6 (25%) cases. Histopathological evaluation of orchiectomy specimens revealed hemorrhagic necrosis and hemorrhagic infarction or ischemic changes. Normal testicular size and vascularity were detected in 11 (61.1%) cases with detorsioned testis as detected by follow-up Doppler US. Late orchiectomy was performed in 7 cases (38.9%) with complete atrophy due to lack of blood supply. CONCLUSION: Although Doppler US is very helpful for differential diagnosis of patients with acute scrotum who applied early period, if Doppler US will lead to a waste of time, direct surgery without delay will reduce the risk of testicular loss.

Pure gonadal dysgenesis (Swyer syndrome) due to microdeletion in the SRY gene: a case report.

46,XY complete gonadal dysgenesis (Swyer syndrome) is a rare cause of disorder of sexual development. This syndrome is caused by a defect in the determination of sex during embryogenesis and is characterised with female external genitalia, normal or rudimentary uterus, and streak gonads, despite the presence of the 46,XY karyotype. Most of the studied cases presented with leak of secondary sex characteristics and primary amenorrhea during adolescence. Laboratory findings reveal hypergonadotropic hypogonadism. Herein we present the case of a female with a 46,XY karyotype who was admitted with delayed puberty and detected to have a microdeletion in the SRY gene and diagnosed to have Swyer syndrome. We highlight the importance of karyotype analysis in patients with delayed puberty and primary amenorrhea. Once the diagnosis of 46,XY complete gonadal dysgenesis is established, early laparoscopic removal of the dysgenetic gonads is crucial to prevent the development of gonadal malignancy.

Clinical features and long-term outcomes of idiopathic urethrorrhagia.

The aim of this study was to describe the clinical features and long-term outcome of the patients who were treated at our institution for idiopathic urethrorrhagia. The data of 10 male patients, who underwent cystoscopy between October 2010 and March 2013 due to urethrorrhagia, were evaluated retrospectively. Ten male patients aged between 8 and 16 years at first submission. Four patients (40%) had low voiding frequency (2-3 per day). Three of the four patients had abnormal uroflowmetry/EMG findings. Cystoscopy was done in all patients which revealed bulbar urethral inflammation and hemorrhage in all. Symptoms were not resolved on three of the patients who were under observation, having symptoms on average for 29.6±10.5 months. Complete resolution developed in the other seven patients. Six of the patients` symptoms were resolved soon after cystoscopy. In the patients' with or without normal uroflowmetry/EMG findings urethrorrhagia resolution rates were 86% and 33%, respectively. In the evaluation of urethrorrhagia; detailed history taking, basic laboratory investigation and cystoscopy are enough. The typical patients may be treated expectantly. In our opinion, it seems that dysfunctional voiding and infrequent voiding might cause delayed remission and/or recurrence of urethrorrhagia. Even though, it does not effect the treatment, in the persistent cases, confirmation of diagnosis by cystoscopy helps to lessen the anxiety of the family and might decrease the use of many unnecessary diagnostic tools in the long term follow ups.

Single center experience in patients with unilateral multicystic dysplastic kidney.

OBJECTIVE: To determine the clinical features, long-term outcomes, and additional urological anomalies of patients treated for multicystic dysplastic kidney (MDK). MATERIALS AND METHODS: Patients with MDK who were followed between January 2004 and October 2012 were reviewed retrospectively. Demographic, clinical, laboratory, and radiological data were evaluated. RESULTS: A total of 68 patients with MDK were followed for a mean period of 46.8 ± 32.4 months. MDK was detected by antenatal ultrasound in 64 (94.1%) of the patients. Ten patients had (14.7%) additional urological anomalies in contralateral kidney. Vesicoureteral reflux was found in five patients (7.3%). Other urological anomalies were megaureter (two), cortical renal cyst (two), ureteropelvic junction obstruction (one), and renal ectopy (one). Urinary tract infection was detected in 14 (20.5%) patients. Four (5.9%) patients had hypertension. Compensatory hypertrophy was detected in 29 (42.6%) patients. Eight (11.8%) of these 29 patients had glomerular hyperfiltration and three (4.4%) of these eight patients also had proteinuria. Follow-up ultrasound revealed complete involution in 19 (35.8%) patients. Nephrectomy was performed in 15 (22.0%) patients. Indications of nephrectomy were, recurrent urinary infection (four), hypertension (three), ureterocele (two), renal calculi (one), flank pain (one), hematuria (one), persistent large cystic kidney (three), and atypical US findings (two). CONCLUSION: In patients with MDK, depending on the clinical, laboratory, and radiological findings, appropriate timing of operation by multidisciplinary approach is important for early detection and treatment of any possible complications.

A case report: angiomatoid fibrous histiocytoma in a 6-year-old male and review of the literature.

Angiomatoid Fibrous Histiocytoma (AFH) is a distinctive tumor in children, adolescent and young adults which is slow growing with metastatic potential. The histogenesis of AFH is uncertain. Here, we present a case of AFH of 6-year-old on the trunk. In addition, the differential diagnosis for this lesion is also discussed.

Continuous-type splenogonadal fusion: report of a rare case.

Splenogonadal fusion is a rare congenital anomaly. It has two subtypes, discontinuous and continuous. Up to one-third of continuous-type fusion is associated with other congenital anomalies. We present a continuous-type splenogonadal fusion case that was found incidentally during indirect hernia repair; the testicle was preserved during excision. Laparoscopic exploration was helpful in identifying the isolated polysplenia as the origin of continuous-type splenogonadal fusion, and in excising the cord-like attachment proximally. The patient had no other associated anomaly.