Pediatric germ cell tumors; a 10-year experience

The aim of this study was to evaluate the outcome of germ cell tumors in patients admitted to our center during a ten year period. In a retrospective descriptive study, patients with the pathological diagnosis of germ cell tumor [GCT] were included. All records were evaluated and patients followed by personal visit in clinic or phone call. Data regarding age, sex, tumor site, bio-chemical assay, pathology, treatment and outcomes were gathered. For qualitative variables we computed frequency and percentage and for quantitative variables, mean and standard deviation. Survival analysis was performed using Kaplan-Meier. All statistical analyses were performed by SPSS version 6.0. Forty four patients consisted of 32 girls [72.7%] and 12 boys [27.3%]. Their median age was 23 months. The most common pathological tumor types were 18 [40.9%] mature teratomas and 14 [31.8%] yolk sac tumors. Extra gonadal tumors were more prevalent [32 cases] and consisted of 21 [47.7%] sacrcoccygeal, 7 [15.9%] retroperitoneal, 2 [4.4%] mediastinal and 2 [4.4%]cervical tumors. In gonadal tumors 9 patients had ovarian and 3 patients testicular involvement. Staging at the time of diagnosis revealed stage one in 23 [52.3%] cases. All patients were treated surgically and the most common procedure was total resection in 41 [93.2%] patients. Fifteen [34.1%] patients received chemotherapy. In follow-up 31 [77.5%] patients were in complete remission, 9 [22.5%] had died, and 4 cases did not appear to follow-up visits. The median survival was 16 months [IQR 4-49 months]. The highest mortality rate was found in patients with yolk sac tumors [8 of 13 cases]. The patients with extra-gonadal GCT and a high AFP level have the worst prognosis and lower survival rate. Combination of surgery and chemotherapy can lead to a better prognosis

Treatment outcome of neurogenic bladder dysfunction in children; a five-year experience

A neurogenic bladder is one which functions abnormally due to disorders of sacral nerves that control the bladder's ability to fill, store and empty urine. Abnormal bladder function can cause the bladder to be underactive or overactive. This study was planned to evaluate the treatment outcome of our patients with neurogenic bladder dysfunction [NGBD]. Thirty three patients who have been treated for NGBD were evaluated. Diagnosis was confirmed by voiding-cysto-urethrography [VCUG] and urodynamic study. The patients were treated medically and all had clean intermittent catheterization [CIC]. Data regarding age, sex, clinical and paraclinical findings, sonography, imagings, renal scan, associated anomalies, treatment and outcomes were collected and entered in SPSS software version 18 and analyzed by descriptive statistical. Totally 33 patients aged three days to four years [mean 6.8 months] were included in this study. There were 20 [61%] males and 13 [39%] females. Mean follow-up period was 3.4 +/- 1.2 years [1.5 months to 5 years]. Eighty two precent cases had bilatral and 18% unilatral hydronephrosis and bilatral vesicouretral reflux [VUR] existed in 67% and unilatral in 33% of the patients. Treatment consisted of antibiotherapy and CIC in all patients, which was only in 33% of the cases succesful. The most common associated anomaly was meningomyelocle in 8 patients. Vesicostomy was performed in 22 [67%] cases. Kidney scan showed scar in 10 patients at follow-up study. Complete continence on follow-up was achieved in 24 [71%] patients, and it was improved in 6 [18%] cases. Mortality rate was 9% [3 cases]. Cure rate was 85% in urinary tract infection, 82.7% in hydronephrosis, 80% in VUR and 86.5% in kidney function. Anticholinergic medications was not effective in all our patients. We believe that permanent vesicostomy is an effective and acceptable surgical intervention for protection of upper urinary tract decompression, especially in those who do not respond to medical treatment and have high risk position

Posterior urethral valves; a single center experience

Posterior urethral valves [PUV] are the most common cause of bladder outlet obstruction in infancy that impair renal and bladder function. This study was planned to evaluate and record the various clinical presentations and management, complications, and surgical management and long-term outcome of PUV. In a retrospective study, 98 patients who have been treated for PUV are evaluated in Mofid Children's Hospital from January 2007 to December 2012. Detailed history taken and paraclinical examinations were performed in each patient and diagnosis was confirmed by voiding-cysto-urethrography [VCUG]. PUV had been ablated in 62 patients by electric hook, and diversion was performed in 42 [42.85%] cases. Data were analyzed by SPSS software versionlS. Totally 98 patients with mean age at diagnosis 62 [ +/- 13] days were included in this study. Fifty seven cases had been catheterized within one to 6 days of life [mean age one day], PUV was ablated in 62 patients by electric hook, and dive rsion was performed in 42 cases. The most common symptom in our group was dribbling poor stream 51% and urinary tract infection [UT1] 40.8%. There was vesicoureteral-reflux [VUR] in 61.2%, and hydronephrosis in 82.6%. Most common associated anomaly was kidney anomalies [multicystic kidney disease and renal agenesis/dysplasia] in 8 [8.2%] patients. Twenty patients had prenatal diagnosis of PUV. Complication occurred in three [3.1%] patients. Mortality occurred in 5 [5.1%] patients. Mean follow-up period was 3.4 +/- 1.2 years [1.5 months to 5 years]. Urinary drainage by feeding tube in early days of infancy, followed by valve ablation is the best treatment in PUV, and urinary diversion improves the outcome. VCUG is still the gold-standard imaging modality for documenting PUVs. The factors like renal dysplasia and UTI have their role in final outcome

Survey on childhood solid malignant tumors in cases admitted to Mofid pediatric hospital from 1996-2010: a single-center study

As children comprise a considerable proportion of our population, the importance of local epidemiologic research and geographic and racial differences can't be disputed on childhood malignancies. In this descriptive retrospective study, we extensively reviewed the medical records of patients younger than 15 years of age, diagnosed with solid malignant tumors, from 1996 to 2010, using the last version of International Classification of Childhood Cancers. In our study the order of incidence of solid malignancies was relatively similar to the other national studies, with lymphomas and Central Nervous System [CNS] tumors as the most common, followed by Sympathetic Nervous System [SNS] tumors, soft tissue sarcomas and renal tumors. The peak age of diagnosis was between 1 and 4 years old. In our study, the overall male to female ratio was 1.38, with a trend towards male dominance in the older age groups. We also observed a disturbing trend of childhood solid malignancies. The total number of cases almost doubled from 2009[54[6.9%]] to 2010[96[12.2%]] .This trend was particularly detected in CNS and SNS tumors. Further analysis showed that malignant CNS tumors had played a more pronounced role in this change. Changes in trends of some tumor categories have illustrated a desperate need to further research in regional and national levels. Also the gathered data can be used to make more accurate programs for a better control of cancer and to help policymakers to allocate more evidence-based resource for hospitals

Clinical evaluation of disc battery ingestion in children

The purpose of this study was to evaluate the characteristics, management, and outcomes of disc battery ingestion in children. We reviewed the medical records of children admitted to Mofid Children's Hospital due to disc battery ingestion from January 2006 to January 2010. Clear history, clinical symptoms and results of imaging studies revealed diagnosis of disc battery ingestion in suspected patients. The clinical data reviewed included age, gender, clinical manifestation, radiologic findings, location of disc battery, duration of ingestion, endoscopic results and surgical treatment. We found 22 cases [11 males and 11 females] of disc battery ingestion with a mean age of 4.3 years [range: 9 months to 12 years]. Common symptoms were vomiting, cough, dysphagia, and dyspnea. The mean duration of ingestion was 2.7 days [4 hours to 1.5 months]. A total of 19 patients had histories of disc battery ingestion, but three cases referred with the above symptoms, and the batteries were accidentally found by x-ray. Only three cases had batteries impacted in the esophagus. Twelve batteries were removed endoscopically, 6 batteries spontaneously passed through the gastrointestinal [GI] tract within 5 to 7 days, and 4 patients underwent surgery due to complications: 3 due to tracheo-esophageal fistula [TEF] and 1 due to intestinal perforation. There was no mortality in our study. Most cases of disc battery ingestion run uneventful courses, but some may be complicated. If the battery lodges in the esophagus, emergency endoscopic management is necessary. However, once in the stomach, it will usually pass through the GI tract

Treatment of bilateral macrostomia [lateral lip cleft]: case report

Macrostomia as a rare facial deformity is classified among facial clefts. It originates from failure in union of maxillary and mandibular prominences of first brachial arch during 7[th] embryonic week. We report a case of bilateral macrostomia [bilateral lip cleft] in a female newborn as a sole entity without other skeletal and facial deformities. The cleft was repaired by a simple linear triangular flap using extra oral landmarks to locate lip commissures. Patient was followed through a six-month period. Acceptable results were gained in mouth appearance as well functional aspects. Commissural repair through a linear flap can result in minimal visible scar with satisfying results in both esthetics and functional aspects

Congenital rhabdomyosarcoma of shoulder

A 16-day-old female was referred with congenital swelling on her right shoulder. On examination, there was a hard, round, ecchymotic, nontender, slightly movable, warm and shiny 10x15 cm mass on the right axillary pits which was extended to the right side of neck and chest wall. The mass separated the shoulder from the chest wall causing paralysis of right hand. Chest X-ray, ultrasound and MRI with contrast demonstrated a soft tissue mass suspected to be a hemangioma. The mass rapidly increased in size despite aggressive steroid therapy with rupture and bleeding. On the 45th post natal day the baby was taken to operating room to control the bleeding and if possible total excision of the mass. The mass was separated easily from the surrounding tissue and was excised along with right upper extremity. At the end of surgery the baby had cardiac arrest, and apparently died of Disseminated Intravascular Coagulation [DIC]. The final pathology report was Rhabdomyosarcoma [RMS]

Abdominal inflammatory myofibroblastic tumor: report on four cases and review of literature

The Abdominal Inflammatory Myofibroblastic Tumor [AIMT] is a rare tumor with unknown etiology which usually occurs in children and adolescents. It is composed of myofibroblastic spindle cells intermixed with inflammatory cells. We present four cases of AIMT. We herein present four cases of AIMT in different ages [range: 3.5 to 13 years] and in different organs [stomach, periduodenal, mesenteric, and colon]. There were two females and two males. The main symptoms were abdominal pain/mass/obstruction, vomiting, and weight loss. In all four patients, diagnosis was made by laparatomy and pathologic examination of excised mass lesion. Three patients underwent complete excision and no residual disease was present, one patient received chemotherapy due to tumor recurrences. The patients were followed up in average for four years. As the imaging and laboratory tests are non-specific, the diagnosis of AIMT is rarely made before surgery. AIMT should, therefore, be considered when a mass arises in an unusual location in the pediatric age group. Complete surgical resection should be performed whenever possible and the child should be kept on long-term follow-up

Congenital nasopharyngeal teratoma in a neonate

Congenital germ cell tumors are uncommon. The most common site of teratoma is in the sacrococcygeal region. Teratoma arising from the head and neck comprises less than 10% of reported cases and of these, nasopharyngeal lesions are rare. Teratomas are generally benign, and have a well recognized clinical and histopathological entity. We present a case of nasopharyngeal teratoma [NPT] associated with a wide cleft palate. A 20 day old female neonate with a teratoma of the nasopharyngeal area, and wide cleft palate was referred to our center. The protruded mass which measured 6x4x3cm, was of soft consistency, blocked the airway, and prevented oral feeding. Preoperative evaluation and imaging was performed and mass was excised 2 days after admission. Pathology revealed a well-differentiated mature solid teratoma [hairy polyp]. The patient had no complication in the post-operative period. Cleft palate was surgically repaired when 2 years old. She is now a six year old girl with normal development. Congenital nasopharyngeal teratomas are usually benign. Surgery is the treatment of choice, and should be undertaken on an urgent basis, especially in a patient who presents with signs and symptoms of airway obstruction

Can we rely on frozen sections of a rectal biopsy for one-stage trans-anal pull-through operation in Hirschsprung's disease?

Successful results after one-stage trans-anal pull-through [OSTAPT] operation for Hirschsprung's disease [HD] depend on accurate identification of the aganglionic segment in intra-operative frozen section [FS]. Misinterpretation of the findings of the rectal biopsy is an anxiety-evoking pitfall for the surgeon. This study aims to describe our experiences in comparing results of FS and permanent Section [PS] rectal biopsies in children with HD who were candidates for OSTAPT in a single-step operation. Subjects under the age of 14 years, admitted from March 2000 to July 2008 in a university-affiliated children's hospital for open rectal biopsy to diagnose HD were included in the study. All biopsies were taken 2-3 cm above the dentate line. 210 specimens of full-thickness rectal biopsy were obtained for both frozen section and permanent biopsy from all patients, examined by two well experienced pediatric pathologists for ascertaining the presence of ganglion cells, and the results were compared. Analysis was performed by SPSS Software version 11.5. Two-hundred one infants and children underwent FS rectal biopsy to exclude HD. Positive results were seen in 63.8% of the specimens examined as PS and in 58.3% of FS samples. 93.9% of positive results in FS studies were confirmed by PS studies. 6.1% of FS reports were false positive and 21.7% were false negative [P<0.001]. The sensitivity of FS was 85.8% and specificity 90.2%. Positive predictive value [PPV] was 93.9% and negative predictive value [NPV] was 78.3% in FS studies [P<0.001]. The accuracy of FS was 80.4%. Although FS of the rectal biopsy is useful in defining the aganglionic segment during operation, according to this study, it cannot be used as the sole base for performing primary pull-through operation before the results of the permanent section are on hand

Liver hydatid cyst in children [a 14-year review]

Hydatid disease is still an important health hazard in the world. This disease is a parasitic infestation which is endemic in many sheep and cattle raising areas such as in Iran. The aim of this study was to evaluate the clinical appearance, diagnosis, and treatment of liver hydatid cyst in children. This retrospective study evaluated 100 patients who were referred to Mofid Children's Hospital with liver hydatid cyst from March 1996 to March 2010. Medical records of 1 to 14 year old patients who had definitive liver hydatid cyst were included and analysis of variables such as age, gender, symptoms, diagnostic investigation, operative technique, hospital stay, mortality, morbidity and outcome of treatment were evaluated. The patients consisted of 54 boys [54%] and 46 [46%] girls with an age range of 1-14 years [mean 11.8 +/- 4.6]. The incidence rate increased by age. The patients had totally 110 cysts, right pulmonary lobe 81 [73%] cysts and left side had 29 [27%]. Abdominal mass was the most common [50%] symptom. Abdominal sonography gave correct diagnosis in 94 [94%] patients. Conservative surgical treatment was carried out in 98 children. Two patients were treated medically as the cysts were small and calcified. The most common complication was wound infection in 3 cases. Mean length of hospitalization was 9 days. In 100% of our patients the type of parasite was Echinoccocus granulosus. The morbidity rate was 12% [prolonging external catheter drainage in 12 patients]. There was only one [1%] mortality and 2 [2%] recurrences. Due to the less invasive and high accuracy of liver sonography in diagnosis of hydatid cyst, we recommend it as the method of choice for the diagnosis in endemic regions. Surgery is the method of choice for treatment

Early exploration in the management of acute scortum in children

Acute scrotal conditions are a common clinical setting that present with pain and swelling of the hemiscrotum. The aim of our study has been to evaluate the findings in boys operated on acute scrotum. A descriptive study was conducted on 100 patients with acute scrotum admitted to Mofid Children's Hospital from March 1993 to March 2007. Data included history, age, primary symptoms, definite diagnosis, side involvement, paraclinical tests, imaging modalities, medical or surgical management and type of the surgery. Diagnosis was made mainly by clinical signs and symptoms and surgical exploration. Torsion of testis [n=31] was the most common cause of acute scrotum followed by incarcerated inguinal hernia [n=30], torsion of testicular appendage [n=27], epididymo-orchitis [n=7], idiopathic scrotal edema [n=4] and hematocele [n=1]. Most [34%] of the patients were in the first year of life and the mean age was 5.4 years. The commonest signs were pain and swelling [62%] followed by pain, swelling and redness [21%] and pain alone [16%]. 83 patients consisting of 31 with torsion of testis, 14 with torsion of testicular appendage, 30 with incarcerated hernia and 7 with epididymo-orchitis underwent surgical exploration after careful physical examination. 10 of 31 patients with torsion of testis had orchiectomy and orchiopexy of contrea-lateral testis and the rest had detorsion and bilateral orchiopexy. 80% of patients were referred to the hospital after 12 hours of clinical onset of symptoms. Early exploration of scrotum based on careful physical examination excludes the risk of misdiagnosis by diagnostic procedures and unnecessary delay by diagnostic techniques. Exploration of scrotum is a relatively safe and simple procedure with good cosmetic results, it also allows an accurate diagnosis to be made

Thyroid nodules in childhood: a single institute experience

Thyroid nodules are rare in children. Multiple diagnostic modalities are used to evaluate the thyroid mass. The aim of this study was to determine results of management of thyroid nodules in children with special attention to the role of fine needle aspiration biopsy, [FNAB] in diagnosis. Thirty-two children who underwent surgery for thyroid nodules in Mofid Children's Hospital within 10 years [1996 to 2005] were retrospectively studied. From clinical records we [obtained data about demographic characteristics, clinical manifestations, ultrasonography [USG] findings, and FNAB results, pathological reports, surgical therapy and complications. Data was analyzed statistically for association with thyroid cancer. Twenty-five patients [78.1%] were girls, and 7 [21.9%] boys. Mean age was 10.9 [range 8 to 14] years. 24 [75%] patients had benign and 8 [25%] malignant tumors. 18 [56.25%] nodules were located in the right lobe. Statistical analysis revealed sensitivity, specificity, accuracy, and positive and negative predictive values as follows: 80%, 65%, 25%, and 86% for USG; 35%, 41%, 40%, 18%, and 66% for RNS; 91%, 94%, 90%, 74%, and 96% for FNAB respectively. Clinical judgment as determined by serial physical findings with USG continues to be the most important factor in the management of thyroid nodules in children. FNAB is the most accurate method of investigation and its accuracy is improved by USG guidance

Penile agenesis: report on 8 cases and review of literature

Penile agenesis [PA] is an extremely rare anomaly with profound urological and psychological consequences. The opening of the urethra could be either over the pubis or at any point on perineum or most frequently in anterior wall of the rectum. The aim of treatment is an early female gender assignment and feminizing reconstruction of the perineum. We report 8 cases of penile agenesis with urination and defecation through the rectum, apparently normal scrotum, bilateral descended testis, normally located anus, urethral opening in anus, 46xy karyotype and associated anomalies. In 2 cases parents refused any surgical interventions, but in 6 cases we did perform different operations [transforming five cases to females and one case to male gender]. We recommend feminizing operations in newborns or infants, but in older patients, regarding the child's psychology, it is advised to perform masculinizing operations, and finally, no surgical intervention should be undertaken before counseling the parents

results of posterior sagittal anorectoplasty in anorectal malformations

Posterior sagittal anorectoplasty [PSARP] is the newest and most-widely-used technique for correction of anorectal malformations [ARMs]. In the present investigation, the functional outcomes of PSARP and also the relationship between the complications and the sacral ratio [SR] have been studied. The study group included 49 patients [25 males and 24 females] with ARMs, operated on by the PSARP technique during 1995 - 1999. The patients had been followed up for at least 4 years. Those with mental retardation were not included in this retrospective study. For objective evaluation of the sacrum, the SR was calculated in all of the patients. In the female patients, rectovestibular fistula [46%] and in the males, perineal fistula and imperforate anus without fistula, each with equal frequency [28%] were the most common anomalies. The mean SR in the study group was 0.72 + 0.04. SR was 0.67 + 0.03 among patients who had associated urogenital anomalies, 0.69 + 0.04 in patients suffering from soiling, and 0.67 + 0.02 in patients who had postoperative fecal incontinence. The most common complication following PSARP was soiling [44.9%] and then constipation and fecal incontinence in order of frequency. None of the cases developed urinary incontinence or other urinary complications after PSARP. Seventy-three percent of the patients had voluntary bowel movements [VBMs] and 51% were totally continent. Although the PSARP has a negligible complication rate, the success and outcome of the surgical correction in view of the bowel function depend on the development of the sacral nerves. SR reflects the sacral bone development and can be easily calculated by a pelvic AP film. Considering the lower SR in patients suffering from postoperative soling and fecal incontinence, in comparison to the normal group [0.77], SR could be used as a prognostic index to predict the probability of achieving total continence following PSARP

Perinatal testicular torsion: a case report

Torsion of the testicles is typically seen in children of prepubertal age. This condition is extremely rare in perinatal period. The perinatal diagnosis of spermatic cord torsion is often difficult. Here, we report a case of a male newborn baby who was born with swollen and erythematous scrotum. Ultrasonography showed an enlarged left testis, with a nonhomogeneous texture and a regular border, bilateral hydrocele, and septate fluid collection on the left side. Following the diagnosis of acute scrotal condition, surgical exploration was performed. The left testis was gangrened with a blue coloration because of complete extravaginal torsion. Left orchidectomy, along with a fixation of right testis were carried out. Perinatal extravaginal testicular torsion, although a rare condition, is a true immediate emergency that needs early and quick medical attention, and surgical intervention to save testicular tissue