Report of Two Complicated Anorectal and Genital Malformation: Total Mobilization of Pelvic Organ and Prinea to Midline

We had two cases of female complicated anorectal and genital malformations which underwent total mobilization of all structures of perinea to midline.Case1:A 6 month old female with skin covered bladder, pubic diastasis, ectopic anus, vagina, and urethra to the right side of median cleft and lipoma on the left side. Closure of pubic diastasis with iliac osteotomy and double barrel sigmoid colostomy was done at 8 months age. In the second operation, right displaced anus, vagina and urethra and the entire muscle complex underwent total mobilization to the midline.Case2:An 8 Month old female with complicated anorectal malformation who underwent double barrel colostomy on day 8 of birth. Median cleft lipoma was excised and total mobilization of anus, urethra, right hemivagina and muscle complex to the midline was done and the two hemivaginas were sutured together.Unusual and bizarre anatomic arrangements can be seen in this group. Each case represents a unique challenge for the surgeon, with a different prognosis and therapeutic implications. No general guidelines can be drawn for the management of these patients. Each case must be cared for individually

Outcome of tubularized incised plate (TIP) urethroplasty: A single-center experience with 307 cases

Introduction: In order to assess our skill in the tubularized incised plate (TIP) urethroplasty technique in children with hypospadias.Material andMethods: Of 307 children (mean age 38 month) who received a TIP urethroplasty, all had primary hypospadias. Patients who were referred with complication were eliminated from our study. The hypospadias defects were subcoronal in 25 (8%), distal penile in 236 (76%), midpenile in 25 (8%), proximal in 14(4%) and unknown in 7(2%) cases. Chordee was present in 53 (17.2%) patients. Presence of complications requiring reoperation and overall general appearance was recorded.Results: The mean follow-up was 36 (19-72) months. Overall success rate was 70 % (215). Re-operation was required in 92 patients (30%): for urethrocutaneous fistula in 79 (26%), complete disruption of the repair in 2(0.6%) cases and meatal stenosis requiring meatoplasty in 12 (3%). Complete glans dehiscence occurred in 3 patients, which was repaired using the MAGPI technique. Partial breakdown of the glans occurred in 10 cases which did not require further surgery. One case had a huge urethral diverticulum.Conclusions: This technique is relatively common compared to other accessible operations, but attention to details is necessary in order to achieve good results. It appears that complications, such as fistulas and meatal stenosis are more common in this method than other techniques, which require more research in the future

An Assessment of Preoperative and Postoperative Nutritional Status in Children with Cleft Lip and Palate

Background: Children with cleft lip and palate generally suffer from inappropriate and inadequate feeding, resulting in insufficient weight gain leading to malnutrition and poor growth. We aimed to evaluate the nutritional and growth status of cleft lip and palate children before and after reconstructive cleft lip and palate surgery.Methods: All cleft lip and palate children aging from birth up to 36 months who were admitted to a referral Children's Hospital were investigated by a nutritionist. Data were collected using a questionnaire. About 5-6 months after surgery, data regarding their height, weight, feeding status and feeding difficulties were collected by telephone. Statistical analysis was performed using SPSS 18 software. For qualitative variables we used frequency and percentage and for quantitative variables we measured mean and standard deviation. We used Paired Sample T-test for comparing weight differences before and after surgery.Results: In this study, formula was the most common type of feeding before and after surgery. Nasal regurgitation and insufficient sucking was the most common complaint of mother's before surgery, and also nasal regurgitation was reported as the most common difficulty after surgery, probably due to the need for reoperation. Bottle feeding was the most common method of feeding before and after surgery. After surgery only one mother claimed that her child spent more than 30 minutes for feeding. Weight for age of 18 children before surgery was lower than 5th percentile while after surgery this was reduced to 13 children. Also, there were significant differences between mean age before and after surgery which could implicate increase of weight after surgery.Conclusions: Our study demonstrates the results of surgical repair of cleft lip and palate on nutritional status in children.

Chronic nonpigmented Villonodular synovitis in a 13 years old girl: A case report

Nonpigmented villonodular synovitis is a very uncommon and benign proliferative disorder involving the synovium. It is mostly seen in the knee. Here we present a 13-year old girl with a 2 year history of left wrist mass without any pain, tenderness or limitation in range of motion. We believe it is necessary to considere villonodular synovitis in a child with chronic joint effusion as a differential diagnosis

A Case of Single System Ectopic Ureter and Dysplastic Kidney and Contralateral Refluxing Duplex Kidney

We report a six-year-old female child who  presented with continued incontinence and a normal physical examination. Urodynamic study was normal and uroflowmetry showed a prolonged low peak flow rate (PFR) in voiding. VCUG revealed left grade ш VUR. Her symptom continued in spite of medical treatment. Finally, MRU showed a single system ectopic ureter with a dysplastic kidney on the right side and a refluxing duplex kidney on the contralateral side. She underwent surgical correction and recovered.   Keywords:  Ectopic Ureter;  Ureter Abnormalities;  Magnetic Resonance Imaging; Urography; child; Renal Dysplasia

The role of routine transanastomotic T tube ostomy in jejunoilealatresia:A prospective, randomized study

Introduction: Jejunoileal atresia (JIA) is a common cause of neonatal intestinal obstruction and is a common surgical emergency. The aim of this study was to compare primary anastomosis without resection of the dilated segment with transanastomotic T tube jejunostomy (TATTO) versus resection of the dilated segment without T tube as the control group for treatment of patients with JIA.Material and Methods: During 2008 to 2013, 105 patients were diagnosed with JIA at the time of surgery and were recruited for this study. Data relating to efficacy and procedural complications were compared among patients. The criteria for exclusion were duodenal obstruction, colonic atresia, intestinal perforation, malrotation, and also JIA patients with associated other anomalies like meconium ileous, gastroschisis and anorectal malformation.Results: A total of 125 patients were diagnosed and 16 patients were excluded. Our patients were divided into a control group (n=52) and a TATTO group (n=57). The TATTO group had a significantly shorter postoperative hospital stay (12 vs. 23 days, p=0.001) and time to start feeding (9 vs. 13 days, p=0.003) compared with the control group. Sepsis (12.0% vs. 32.7% p=0.004), other complications (3.6% vs. 15.4% p=0.001) and the associated morbidity were significantly lower in the TATTO group.Conclusion: The findings suggest that the TATTO technique was a better method for the treatment of children with JIA, as study showed lesser morbidity and mortality rates.

Results of Onlay Flap Versus Durham Smith in Proximal Hypospadias

Introduction: Urethroplasty was originally used for the repair of hypospadias of the mid and distal portion of the penis but since complications of the two-stage Durham Smith technique such as mega urethra and proximal anastomotic strictures became apparent; the Onlay island flap technique has been increasingly used in more severe cases of hypospadias. The aim of our study was to compare the outcome of these two techniques in the surgical treatment of hypospadias.Material and Methods: In this retrospective study, thirty -three patients underwent surgery for the treatment of hypospadias. The two- stage Durham smith repair was used for 17 cases and the onlay island flap technique was carried out on 16 patients. Data was analyzed using SPSS software version 21 and complication rates of the two procedures were compared.Results: Altogether, frequency of complications was higher in the smith technique than the onlay flap procedure without any significant difference between the two techniques (47% for Smith technique and 19% for onlay repair; P=0.141). Fistula was the most frequent complication in the two techniques (41% for Smith technique and 13% for onlay repair; P=0.117). Meatoplasty was done for meatal stricture in the smith group. Moreover, the smith technique failed completely in one case who then underwent Tabularized Inside-Plate (TIP). There was a case of chordee recurrence in the smith technique; for which a dorsal Nesbit plication was carried out. The mean days of hospitalization was more with the smith technique (7.1 days vs 5.0, P=0.016). In addition, the mean cases which required reoperation was higher in the smith group than the onlay flap (1.0 vs 0.2, P=0.025).Conclusions: The results of this study showed that the onlay flap technique had lower complications and reoperation rates than the two-stage smith technique. In addition, the onlay flap technique has fewer days of hospitalization than the smith technique, resulting in lower costs to the health system and patients

Postoperative apnea among premature or anemic infants undergoing inguinal hernia repair

Introduction: Premature or anemic newborns undergoing hernia repair are prone to more postoperative complications than full-term infants. The incidence of respiratory complications among these patients is more than 30%, being postoperative apnea the most common. Some investigators found that gestational and postconceptional age, the presence of continuing apneic episodes and anemia are the main determinants of postoperative apnea. It seems that infants who do not receive intravenous anesthetics experience less respiratory complications. Intravenous anesthetics have hepatic metabolism for elimination and the immature liver of the premature has not sufficient elimination capacity. Materials and Methods: Study participants were 1047 neonates with post conceptual age (PCA) lower than 60 weeks undergoing inguinal hernia operation. Sevoflurane gas mask was used for anesthesia induction and during deep anesthesia, caudal block was administered using 1cc/kg bupivacaine 2%. The neonates were managed with spontaneous breathing with Sevoflurane gas mask or endotracheal tube until the end of the operation. Results: In the present study, 916 (87.5%) male and 131 (12.5%) female neonates were included. Mean gestational age and PCA were 36.62 ± 38.0 and 46.80 ± 45.60 weeks respectively. Postoperative apnea did not occur in any patient. Conclusion: Many studies confirm our findings that inhaled sedative drugs without hepatic or renal metabolism are safe for sedation of premature or anemic infants

A bizarre presentation of Peutz – jegher’s syndrome in a 2 year old

Peutz - jegher’s syndrome (PJS) is a rare autosomal dominant disorder with gastro intestinal and mucosal pigmentations. We present a 2 y/o boy with a lesion prolapsing from his rectum that could not be reducted. We found him to have PJS and a familial history of PJS in his parents

Teratoma in Infants and Children

Background: Teratomas arise from three germ cell layers, the ectoderm, endoderm and mesoderm1, 2, and have several degrees of differentiation. We report our experience with teratomas at a tertiary pediatric surgery center.Patients and Methods: The hospital records of all patients with the pathological diagnosis of teratoma treated during 10 years between 2004 and 2014 were reviewed and the following information was obtained: Sex, site of tumor, treatment and outcome.Results: Sixty seven patients consisting of 49 girls (73%) and 18 boys (27%) were treated with teratomas at various sites of the body. These included: sacrococcygeal (SC) 32 patients (27 females& 5 males), ovarian 12 cases, cervical 4 patients (1 females & 3 males), retroperitoneal 9 (5 females & 4 males), Nasopharyngeal 2 patients both of which were females, mediastinal 2 cases (1 female & 1 male) and 5 testicular teratoma patients. All patients underwent surgery, and the most common procedure was total resection in 63(94%) of patients. Twenty eight (42%) received chemotherapy. In follow-up 52(77%) patients were in complete remission, 8(12%) had died, and 4 cases did not attend follow-up visits.Conclusions: Teratomas are a group of tumors with similar histological picture but different behaviors. Sacrococcygeal teratomas are the commonest and the majorities are benign but the risk of malignant transformation increases with age. Management of teratomas is a combination of surgery and chemotherapy which may lead patients to a better prognosis