A dual H-type tracheoesophageal fistula; why not being repaired simultaneously? A case report and review of literature.

BACKGROUND: H-type Tracheoesophageal Fistula (TEF) is a particular type of congenital esophageal anomalies, in which patients present with non-specific symptoms that can result in delayed diagnosis. Here, we report two pediatric cases with a rarer variant called ?dual H-type TEFË®. CASE PRESENTATION: We present two cases of H-type TEF. The first was a 45-day-old boy with feeding problem and cyanosis while feeding, and the second was a three-month-old girl with cough and choking after feeding from the first day of birth. In both cases, two separate TEFs were detected during diagnostic evaluation by flexible bronchoscopy. Both were repaired simultaneously through a cervical incision. The first patient deteriorated 13 days after the surgery, disturbancing in acid-base balance and expired unfortunately. CONCLUSION: Hence, it is necessary to consider the possibility of double TEF in any newly diagnosed H-type TEF.

Pediatric Surgery Workforce Around the World: A Need to Revise Residency Programs?

Objectives:The study aimed to address the shortage of pediatric surgery specialists globally by investigating the discrepancies in training programs and population metrics across different countries and regions. Methods: An international survey of pediatric surgeons gathered data on training duration, examination procedures, certification, and population metrics like mortality rate and surgeon-to-population ratio. Results: The study included 44 countries. The average length of pediatric surgery training was 5.7 years, with no significant difference between different regions. The pediatric mortality rate and surgeons count per 100 000 people were inversely correlated, while training duration was associated with GDP and life expectancy, but not pediatric mortality rate or surgeons' count. Conclusion: Many countries' pediatric surgery training programs do not align with their actual need for pediatric surgeons. Nations with limited economic resources may opt to shorten residency programs or offer pediatric surgery as a direct specialty after medical school to mitigate the shortage effectively.

The outcome and complications of modern staged repair surgery in newborns with classic bladder exstrophy in different genders: A retrospective study.

INTRODUCTION: Classic bladder exstrophy (CBE) is a rare anterior midline birth defect that remains a challenge for pediatric surgeons. Despite multiple reconstructive methods, outcomes vary widely in various reports. This study aims to compare the success rate and complications of modern staged repair of exstrophy (MSRE) in each gender and compare together. METHODS: This retrospective cross-sectional study included cases of CBE between 2010 and 2020 that underwent MSRE. Short-term follow-up results, including incontinence rate, vesicoureteral reflux (VUR), urinary infections, deformed genitalia, and so on, were measured in each gender, and their differences were reported. RESULTS: Among the 40 newborns with CBE who underwent MSRE, 25 (62.5%) were boys, while the others had non-male genitalia. The rates of incontinence, VUR, dehiscence, and fistulas did not differ significantly between genders. However, chronic urinary tract infections (UTIs) were more frequent in girls, and boys were more likely to have malformed genitalia (p < .05). CONCLUSION: Our findings indicate a similar rate of complications in each gender. However, chronic UTIs and external genitalia deformities were significantly more common in girls and boys, respectively. Further large-sized controlled trials may be needed to corroborate these findings.

The usage of a magnet-powered instrument in the extraction of metallic or magnetic aspirated foreign bodies: A case report.

INTRODUCTION AND IMPORTANCE: The unintended passage of foreign bodies into the airway, is a common problem among children which can lead to death. Prompt diagnosis and extraction of the entrapped object is the key of success in these cases. Unfortunately, in addition to various extraction methods, in some cases the bronchoscopy may not be successful, and exploratory thoracotomy is inevitable. CASE PRESENTATION: We describe the story of a 7-year-old preschooler boy who was taken to the emergency department complaining the sudden onset of cough and dyspnea. Initial investigations, showed an entrapped oval-shaped object in the right main bronchus. Several conventional bronchoscopic attempts were failed. We used a handmade magnet-powered instrument to extract the object. CONCLUSION: Using the magnet-powered grasping forceps may be beneficial after repetitive failed attempts in the extraction process of airway foreign bodies. Although there is no specific evidence-based guideline for choosing the best removal technique, we recommend using this technique in round-shaped high weight metallic objects as the first removal technique.

Efficacy of bupivacaine-neostigmine and bupivacaine-tramadol in caudal block in pediatric inguinal herniorrhaphy.

BACKGROUND: Limited duration of analgesia is among the limitations of single caudal injection with local anesthetics. Therefore, the purpose of this study was to evaluate the effectiveness and safety of bupivacaine in combination with either neostigmine or tramadol for caudal block in children undergoing inguinal herniorrhaphy. METHODS: In a double-blinded randomized trial, sixty children undergoing inguinal herniorrhaphy were enrolled to receive a caudal block with either 0.25% bupivacaine (1 ml x kg(-1)) with neostigmine (2 microg x kg(-1)) (group BN) or tramadol (1 mg x kg(-1)) (group BT). Hemodynamic variables, pain and sedation scores, additional analgesic requirements, and side effects were compared between two groups. RESULTS: Duration of analgesia was longer in group BT (17.30 +/- 8.24 h) compared with group BN (13.98 +/- 10.03 h) (P = 0.03). Total consumption of rescue analgesic was significantly lower in group BT compared with group BN (P = 0.04). There were no significant differences in heart rate, mean arterial pressure, and oxygen saturation between groups. Adverse effects excluding the vomiting were not observed in any patients. CONCLUSION: In conclusion, tramadol (1 mg x kg(-1)) compared with neostigmine (2 microg x kg(-1)) might provide both prolonged duration of analgesia and extended time to first analgesic in caudal block.

Abdominoscrotal hydrocele: A case report.

Abdomino scrotal hydrocele (ASH) is a very rare condition in which the hydrocele sac extends beyond the scrotum to the abdomen via the inguinal canal. Although various ideas have been proposed regarding this disease, there is still controversy over its etiology. We report a case of abdominoscrotal hydrocele in a one year old boy (Mofid Children's hospital, Tehran, Iran) with history of right sided herniorrhaphy one month ago in other center. Slow growing mass in lower abdomen was noted by parents. For better diagnose, ultrasound and CT scan was performed. In operation missed large abdominalscrotal hydrocele was confirmed.

Early pyeloplasty versus conservative management of severe ureteropelvic junction obstruction in asymptomatic infants.

BACKGROUND: Ureteric-pelvic junction obstruction (UPJO) is the most common cause of antenatal and neonatal hydronephrosis and its management remains controversial. While conservative management is advocated for all, this strategy puts a quarter of these patients at risk for possibly irreversible renal damage. AIM: In this study, we compare functional and anatomic outcomes in newborns and infants less than 1 year of age with high-grade unilateral UPJO, following early surgical pyeloplasty (ESP) versus conservative management (CM). MATERIALS AND METHODS: This was a single center prospective interventional study. Infants referred to our tertiary care pediatric surgery clinic between September 2016 and September 2018 with UPJO were considered. To be included patients must have been less than 1 year old, lack of clinical symptoms, suffer from severe hydronephrosis as defined by Society for Fetal Urology (SFU) grades 3 or 4, and have affected kidney Split Renal Function (SRF) above 40%. Patients with bilateral disease, structural anomalies, or an abnormal voiding cystourethrogram (VCUG) were excluded. Anatomical and functional outcomes were measured and compared at 6 and 12 months. RESULTS: Fifty-six patients were assigned to receive either ESP (n = 28) or CM (n = 28). At 6 months Cortical thickness, polar length, and SFU indices were significantly lower in the ESP group, while none of the outcomes were significantly different between the two groups at 12 months. Despite the two groups not being different at 12 months regarding differential renal function (DRF), there was a significant decrease of function in the CM group compared to baseline. CONCLUSION: When considering treatment options for infants with high-grade UPJO, it appears that ESP hastens improvement of anatomic and functional indices, while CM may lead to a significant deterioration in renal function.

Neonatal neuroblastoma presented with respiratory distress, a case report.

BACKGROUND: The most common neonatal tumor is neuroblastoma and adrenal gland is the most common site of involvement. 11-26% of this tumor is seen in the thorax of neonates. Due to a similar appearance of the mediastinal tumor with pneumonia, a high index of suspicion is necessary for early diagnosis of this disorder. CASE REPORT: A 17-day -old female and term neonate delivered by caesarian section was reported in the study. The mother had a normal pregnancy without any reported complications. The newborn admitted to a neonatal intensive care unit (NICU) in her hometown, because of respiratory distress, stridor, and tachypnea which was started from 7 days of life and due to lack of recovery and persistent respiratory symptoms, the patient referred to our hospital. In chest X-ray, opacity in the right upper lobe of the lung was seen. In barium study, a mass like lesion in the size of 35.34 mm adjacent to upper intra-thoracic esophagus with mild mass effect was observed and in CTS a posterior mediastinal mass with severe compression on the trachea and extension to the spinal canal was reported. Due to severe obstruction on the airway, the baby underwent emergency surgery and a mediastinal mass with adhesion and involvement of the ribs and spinal cord was resected. The final diagnosis of poorly differentiated neuroblastoma was confirmed by biopsy. CONCLUSION: In every neonate with persistent respiratory distress, stridor and abnormal chest X-ray, diagnosis of neonatal neuroblastoma should be considered.

Evaluation of Onlay Island Flap Technique in Shallow Urethral Plate Hypospadiasis.

BACKGROUND: Hypospadias is one of the most common congenital genital anomalies in males that necessitates to be operated early in infancy (when 6 to 9 months old). On the other hand, hypospadias is a challenging field of pediatric urology with multiple reconstruction techniques. A perfect hypospadias repair is supposed to return urethral continuity with sufficient caliber, eradicate phallus curvature, and supply an acceptable appearance with low complications. OBJECTIVES: This study aimed to evaluate the outcomes of using onlay island flap technique in the repair of hypospadias with shallow urethral plate. PATIENTS AND METHODS: In this prospective study within June 2012 to December 2013, we performed onlay island flap procedure to repair hypospadias with shallow urethral plate measuring less than 6 millimeter. This technique was selected for all types of hypospadiasis except subcoronal type. Nesbit's dorsal plication procedure was established for chordee. In cases with very small glans, urethroplasty was performed without glansplasty. RESULTS: Twenty three patients with mean age of 30 (range 10 - 60) months underwent onlay island flap repair; all had a shallow urethral plate < 6 mm, 3 had a very small glans, and 18 had chordee. Meatus was located in distal shaft in 5 cases, mid shaft in 8, proximal in 6 and penoscrotal type in 4 patients. Chordee was corrected with Nesbit's dorsal plication in 16 cases. Complications were: meatal stenosis in 2 cases and urethrocutaneous fistula in 2 patients, all of which were repaired surgically. Mean follow up time was 13 (3 - 20) months. All cases that had glansplasty have excellent esthetic appearance. CONCLUSIONS: This technique offers acceptable results regarding meatal stenosis, urethrocutaneous fistula and esthetic outcome.

Soft palatine mass with diagnosis of mature teratoma.

Teratoma is a true neoplasm that consists of tissues from all 3 embryonic germ layers: ectoderm, mesoderm, and endoderm. Nasopharyngeal teratoma is very rare. We present one case of nasopharyngeal teratoma attached to the soft palatal wall in a newborn.

Outcomes of Second Look Exploration in Testicular Torsion of Children.

BACKGROUND: Testicular torsion (TT), or twisting of the testicle resulting in a strangulation of the blood supply, occurs in men whose tissue surrounding the testicle is not well attached to the scrotum. It is important to emphasize that testicular torsion is a medical emergency. OBJECTIVES: The aim of this study is to evaluate the second look exploration and outcomes in TT. PATIENTS AND METHODS: Seventy boys out of 124 patients underwent early exploration and 48 hours later second look exploration due to TT. All patients were checked with preoperative color-doppler ultrasonography (CDU) and intraoperative bleeding test. Data included age at admission, side of pathology, relation of TT with season of year, duration of preoperative history, degree of testicular torsion, CDU findings, and degree of bleeding; results of second look exploration, follow-up, and outcomes were analyzed. RESULTS: Totally 70 patients were included in this study within five years, of which mean age was 28.6 ± 32.9 months (range 1 to 144), 48% of our patients had nausea and vomiting. Preoperative CDU showed absent/weak flow in 50 (71%) cases. Winter showed most frequently (44%) referred cases of testicular torsion. Orchidopexy was performed in 44 (63%) and orchidectomy in 26 (37%) cases after second look exploration. Mean follow-up duration was 3.1 ± 1.4 years. 4 (9%) cases in orchidopexy group developed testicular atrophy during follow-up, all four cases had a history of longer than 12 hours and grade II testicular bleeding test intra-operatively. Other orchidopexy patients salvaged. 26 patients, who were in grade III, underwent orchidectomy in second look exploration. CONCLUSIONS: TT requires emergency attention. The ischemia time of the testis is traditionally after 6 hours, and imaging or other diagnostic modality should not be a cause of delay. Early surgical exploration is modality of choice, and second look exploration after 48 hours can be more effective and salvageable in these patients.

Pediatric Germ Cell Tumors; A 10-year Experience.

OBJECTIVE: 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. METHODS: 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 version16.0. Findings : 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). CONCLUSION: 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.

OBJECTIVE: 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). METHODS: 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 version18 and analyzed by descriptive statistical. Findings : 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. CONCLUSION: 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.

New single nucleotide variation in the promoter region of androgen receptor (AR) gene in hypospadic patients.

BACKGROUND: Hypospadias is one of the most common congenital abnormalities in the male which is characterized by altered development of urethra, foreskin and ventral surface of the penis. Androgen receptor gene plays a critical role in the development of the male genital system by mediating the androgens effects. OBJECTIVE: In present study, we looked for new variations in androgen receptor promoter and screened its exon 1 for five single nucleotide polymorphisms (SNP) in healthy and hypospadias Iranian men. MATERIALS AND METHODS: In our study, at first DNA was extracted from patients (n=100) and controls (n=100) blood samples. Desired fragments of promoter and exon 1 were amplified using polymerase chain reaction. The promoter region was sequenced for the new variation and exone 1 screened for five SNPs (rs139767835, rs78686797, rs62636528, rs62636529, rs145326748) using restriction fragment length polymorphism technique. RESULTS: The results showed a new single nucleotide variation (C→T) at -480 of two patients' promoter region (2%). None of the mentioned SNPs were detected in patients and controls groups (0%). CONCLUSION: This finding indicates that new single nucleotide polymorphism in androgen receptor promoter may have role in etiology of hypospadias and development of this anomaly. This article extracted from Ph.D. thesis. (Nasim Borhani).

Protective temporary vesicostomy for upper urinary tract problems in children: a five-year experience.

OBJECTIVE: Temporary vesicostomy is a urinary diversion procedure for patients with upper urinary tract (UUT) dilatation, secondary to bladder outlet obstruction or dysfunction. The aim of this study was to evaluate our experience in children undergoing such diversion, analyzing its efficacy to prevent urinary tract infection (UTI), improve or resolve hydronephrosis, stabilize or improve kidney function and restore the health of UUT. METHODS: In this retrospective study, patients who had vesicostomy by Blocksom technique due to bladder outlet obstruction or dysfunction were evaluated in Mofid Children's Hospital (in Tehran) from March 2007 to March 2012. The reason for applying this procedure was failure in clinical treatment. Data regarding gender, age, diagnosis, time of any surgical intervention, associated anomalies, primary/secondary complications and mortality were collected using a questionnaire, and evaluated by giving a grade that ranged from 0 (worst) to 10 (best) based on Lickert's scale. FINDINGS: From a total number of 53 patients, (88.7% male and 11.3% female) with a mean age of 225 days, 66% had posterior urethral valve and 16 (30%) neurogenic bladder. UTI was present in all cases, hydronephrosis in 52 (98.1%), and vesico-ureteral reflux only in 45 (84.9%) patients. Valve ablation was performed in 17 cases, and clean intermittent catheterization in14 patients which were unsuccessful. We performed vesicostomy in all patients. Mortality rate was 7.5%. Vesicostomy was closed in 35 patients. Cure rate was 85% in UTI, 82.7% in hydronephrosis, 80% in VUR, and 86.5% in kidney function. CONCLUSION: Vesicostomy is a simple procedure that protects upper urinary tract, decreases hydronephrosis, and improves kidney function. The procedure is well tolerated and reversible, with less complication and should be considered in children in whom conservative and medical treatment has failed.

Posterior Urethral Valves; A single Center Experience.

OBJECTIVE: 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. METHODS: 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 version18. FINDINGS: 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 diversion was performed in 42 cases. The most common symptom in our group was dribbling poor stream 51% and urinary tract infection (UTI) 40.8%. There was vesico-ureteral-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). CONCLUSION: 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.

Acute mediastinitis in children: a nine-year experience.

BACKGROUND: Acute mediastinitis is a serious medical condition with a mortality rate of 30 to 40% or even higher. Early diagnosis with prompt and aggressive treatment is essential to prevent its rapid progression. We evaluated acute mediastinitis cases and analyzed the outcomes. MATERIALS AND METHODS: A retrospective chart review was conducted on patients diagnosed with acute mediastinitis who were admitted to Mofid Children's Hospital from January 2001 to January 2010. RESULTS: Seventeen patients aged 1 to 10 yrs. (mean =3.8 yrs) were evaluated including 12 (70%) boys and 5 (30%) girls. The most common symptoms were fever, dyspnea, cyanosis, tachycardia and tachypnea. The etiology of mediastinitis was iatrogenic esophageal perforation (EP), and related to manipulation in 13(77%), and leakage of esophageal anastomosis in 4 cases (33%). The underlying diseases were esophageal atresia in 2(12%), corrosive injury of the esophagus in 13(76%), congenital esophageal stenosis in one (6%), and gastroesophageal reflux esophagitis also in one (6%) patient. Patients with clinical symptoms were evaluated by immediate chest radiography, and gastrografin swallow. After early diagnosis, the patients received wide spectrum antibiotics and immediate mediastinal or thoracic drainage, followed by esophagostomy and gastrostomy. Only one case of endoscopic perforation was managed by NG tube. Fifteen patients (88%) survived successfully. We had 2(12%) cases of mortality in our study (one patient after esophageal substitution, mediastinal abscess and septicemia, and the other one developed esophageal perforation 6 months after early management and died of cardiac arrest during endoscopic dilation). CONCLUSION: Prevention of acute mediastinitis is still a difficult challenge. As the prognosis is not good and patients have high mortality, rapid management is mandatory.

Primary intrarenal neuroblastoma with hypertension and disseminated intravascular coagulation.

The primary intrarenal neuroblastoma (IRNB) is a rare condition. Intrarenal neuroblastoma typically results from direct renal invasion from an adrenal neuroblastoma, but true intrarenal neuroblastoma originates either sequestered adrenal rests during the fetal life or intrarenal sympathetic ganglia. Clinical, radiological, and pathological correlation is very essential for diagnosis and appropriate management of this type of unusual cases. The distinction of this rare tumor from Wilms' tumor is an important challenge since both tumors have major differences in prognostic and therapeutic response. We present a 3-year-old boy of primary intrarenal neuroblastoma with extensive abdominal and mediastinal mass, persistent hypertension, and disseminated intravascular coagulation (DIC).

Evaluation of one hundred pediatric muscle biopsies during a 2-year period in mofid children and toos hospitals.

OBJECTIVE: Muscle biopsy is a very important diagnostic test in the investigation of a child with suspected neuromuscular disorder. The goal of this study was to review and evaluate pediatric muscle biopsies during a 2-year period with focus on histopathology diagnosis and correlations with other paraclinic studies. MATERIALS & METHODS: We investigated 100 muscle biopsies belonging to patients with clinical impression of neuromuscular disorder. These patients have been visited consecutively by pediatric neurologists during 2010 to 2012. Samples were investigated by standard enzyme histochemical and immunohistochemical techniques. RESULT: Sixty-nine (69%) males and 39 (39%) females with a mean age of 5.7 years were evaluated. Major pathologic diagnoses were Muscular dystrophy (48 cases), Neurogenic atrophy (18 cases), nonspecific myopathic atrophy (12cases), congenital myopathy (6 cases), storage myopathies (4 cases) and in 6 cases there was no specific histochemical pathologic finding. EMG was abnormal in 79 cases. Degree of correlation between EMG and biopsy result was significant in children ≥ 2 years of age. CONCLUSION: This study confirms the high diagnostic yields of muscle biopsy especially only if standard and new techniques such as enzyme study and immunohistochemistry are implemented. Also, we report 11 cases of Merosin negative congenital muscular dystrophy. This is the largest documented case series of Merosin deficient congenital muscular dystrophy reported from Iran.

Clinical evaluation of disc battery ingestion in children.

BACKGROUND The purpose of this study was to evaluate the characteristics, management, and outcomes of disc battery ingestion in children. METHODS 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. RESULTS 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. CONCLUSION 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.