Cervicofacial Rhabdomyosarcoma - Success of a Free Fibular Graft.

Cervicofacial rhabdomyosarcoma (RMS) presents surgical complexities in children due to its aggressive nature. This study presents a successful case of oral RMS treatment with a free fibular graft in a 7-year-old patient. Despite follow-up challenges, the efficacy of the approach is evident.

Sacrococcygeal Teratoma: Clinical Characteristics, Management, and Long-term Outcomes in a Prospective Study from a Tertiary Care Center.

INTRODUCTION: The study focuses on the clinical presentation, management, and outcomes (both short term and long term) in patients with sacrococcygeal teratoma managed over a decade in a tertiary care center. MATERIALS AND METHODS: This is a prospective study on children with sacrococcygeal teratoma over 12 years data collected included antenatal diagnosis, mode of delivery, age at diagnosis, clinical presentation, physical extent of mass (including Altman classification), levels of alpha-fetoprotein, surgical approach, histopathology, clinical outcome, recurrence and long-term results including bladder-bowel dysfunction and neurological impairment. Functional results were evaluated clinically and radiologically. RESULTS: During the study, 41 patients (male to female ratio of 1:3.1) with a median age of 36 days (1 day-11.6 years) with sacrococcygeal teratoma were managed at our center. The mean follow-up duration was 54 months (range 19-110 months). Nearly, two-thirds of the tumors were either Altman Type 1 or 2. Yolk sac tumor was present in 8 (19.5%) patients, while the rest has either mature or immature teratoma. Tumors were removed through a posterior sagittal or a chevron incision. In seven patients, abdominosacral approach was necessary. Eight patients with malignant disease received chemotherapy (neoadjuvant in 5). Overall survival was 95% at a mean follow-up of 54 months. Among the late complications, three patients had a local recurrence of tumor, and urinary dribbling was present in three patients. CONCLUSIONS: Teratomas are the most common germ cell tumors of the sacrococcygeal region. Most of the tumors are benign, and the incidence of malignancy increases with age. The evaluation of malignancy is, therefore, necessary in these children. Excellent survival of 95% was achieved in this series. Morbidity due to associated malformation, disease recurrence, and treatment may persist in these patients; hence, proper follow-up is needed.

Surgical Surprise during Posterior Sagittal Anorectoplasy: A Rectal Duplication.

An 8-month-old boy with anorectal malformation (ARM) was incidentally found to have double rectal pouches during posterior sagittal anorectoplasty. The distal blind-ending pouch was excised, and the larger proximal pouch was tapered and anorectoplasty performed. The excised pouch was confirmed as rectal duplication cyst. One must be aware of such uncommon associations with ARM.

Total Intestinal Atresia: Revisiting the Pathogenesis of Congenital Atresias.

Despite various theories to explain the pathogenesis of atresias, the exact mechanism is still controversial. Currently, atresias are believed to result from vascular accidents and less likely due to the failure of recanalization. We report a case which challenges this belief. A 1-day-old neonate was explored for suspected jejunal atresia. Apart from Type III jejunal atresia, 15 cm from DJ junction, there was surprisingly no distal lumen in the intestine from jejunum till rectum. Multiple enterotomies revealed the whole of the remaining jejunum, ileum, and large colon to be a solid cord-like structure. No distal luminal contents or histopathological evidence of ischemic damage was seen, thus suggesting the probable etiology to be a failure of recanalization of the gut cord rather than a late vascular accident. Such rare cases provide insights into possible embryogenetic mechanisms which can then aid in formulating preventive measures.

Evaluation of two-staged Fowler-Stephens laparoscopic orchidopexy (FSLO) for intra-abdominal testes (IAT).

INTRODUCTION: The best operative intervention for intrabdominal testis (IAT) has not been standardized as yet. The question of whether to bring down an IAT with a single-staged vessel-intact laparoscopic orchidopexy (VILO) or a two-staged laparoscopic Fowler-Stephens orchidopexy (FSLO) is still undergoing debate, with both the procedures being popular. The present study has been designed to evaluate the factors predicting the success or failure of two-staged FSLO for (IAT). METHODS: 43 boys with 49 non-palpable testes underwent diagnostic laparoscopy out of which 35 underwent two-staged FSLO. Size of the testis was measured with a graduated probe in both stages. Independent variables such as age, height, testis-to-internal ring distance (T-IR), neo internal ring-to-midscrotal distance (NIR-MS), and mobility-to-contralateral ring (MCIR) were analysed. Postoperatively 34 IATs were followed up clinically as well as ultrasonologically after 6 months, to see for the size, position, and vascularity. Based on this, the patients were divided into two groups, Group A (successful) and Group B (Failed). RESULTS: 24 IATs had a successful outcome (Group A) and 11 were failure (Group B). The overall success rate of the study was 68.6%. The difference in mean age of patients in both groups was insignificant (p = 0.89) (Fig. 1), and similarly, the difference in mean height was insignificant (p = 0.61). The difference in mean T-IR in both the groups was insignificant (1.85 versus 2.77 cm; p = 0.09) and mean NIR-MS was 5.41 cm in Group A and 5.10 cm in Group B, and the difference again was insignificant (p = 0.23). CONCLUSION: The success rate of FSLO was 68.6%. None of the above-described independent variables have any effect on the outcome of two-staged FSLO. While VILO remains the treatment of choice for IAT located at or near the ring, but IAT higher than this, two-staged FSLO gives a better chance for achieving intra-scrotal orchidopexy.

Pediatric Appendicitis Score or Ultrasonography? In Search of a Better Diagnostic Tool in Indian Children with Lower Abdominal Pain.

OBJECTIVES: To prospectively assess the performance of pediatric appendicitis score (PAS) in diagnosing acute appendicitis in the children with lower abdominal pain and correlated with ultrasound findings; and to assess the impact of the PAS on clinical outcome and its efficacy in differentiating between complicated and uncomplicated appendicitis. METHODS: A prospective study was done which included cases of lower abdominal pain. Appendectomy was done for PAS ≥ 6, and diagnosis was confirmed on histopathology. A receiver operator characteristic (ROC) curve was created to assess the PAS performance. The sensitivity, specificity, and accuracy of ultrasonography in diagnosing appendicitis were assessed, and analysis of agreement between ultrasonography and PAS score was done by kappa statistics. RESULTS: Of 260 cases with lower abdominal pain, 205 were suspected of having appendicitis. One hundred fifty-nine had PAS ≥ 6. There were 2/159 (1.26%) cases of negative appendectomies and 2/46 (4.34%) cases of missed appendicitis. The mean PAS was significantly higher in patients with appendicitis than in those without appendicitis. The area under the ROC curve was 0.9925. Sensitivity, specificity, and positive and negative predictive value of PAS were 98.74%, 95.65%, 95.7% and 95.65%, respectively. Complicated appendicitis had significantly more PAS, fever, and cough tenderness than uncomplicated appendicitis. The sensitivity and specificity of ultrasonography were 86.79% and 17.39%, respectively. Agreement between ultrasonography-proven appendicitis and PAS-dependent appendicitis was weak. CONCLUSION: PAS has high efficacy in diagnosing acute appendicitis. Clinical outcome was more favorable with the use of PAS. Ultrasonography should be used judiciously and in combination with clinical judgment.

Spectrum of congenital lung and foregut malformations: scope for awareness and prompt management-a case series.

Congenital lung and foregut malformations have been described in literature, but most articles are from the Western world. There are a separate set of problems which are faced in our country especially with the misdiagnosis of these problems which has not so much been addressed in writing. We retrospectively reviewed records of all patients with above thoracic lesions treated at a tertiary care hospital in Delhi from March 2017 to December 2019. Twenty patients were found. Eight of 20 patients were detected antenatally but none monitored serially. Age at presentation ranged from 5 days to 18 months. Eight patients presented with respiratory distress at birth. Three of these were congenital lobar emphysema wrongly diagnosed as pneumothorax and brought with intercostal drainage tube inserted. Eight suffered from pneumonia, 4 of which had history of previous hospital admission but undetected congenital pathology. All underwent surgery and had good outcome. There was radiological evidence of compensatory lung growth in all patients at 6 months follow-up. Thus, we conclude that the antenatal detection of congenital lung and foregut malformations may have increased but proper serial monitoring is still missing. There is scope of increasing index of suspicion for these lesions among pediatricians and surgeons. With modern-day safe anesthesia, proactive resection of congenital lung and foregut malformations is associated with good outcome. Delaying treatment predisposes the child to infective complications and makes surgery difficult.

Primary anastomosis in difficult cases of type "C" esophageal atresia: The atraumatic microvascular clamp technique of minimal tension with good outcome.

BACKGROUND: Surgical maneuvers for esophageal anastomosis in difficult cases of Gross type "C" esophageal atresia (EA) are challenging. The methods of early primary anastomosis are technically difficult and staged surgeries expose the child to repeated general anesthesia with problems of nursing care. We describe a simple method of partial disconnection of the lower esophagus from the fistula followed by approximation by an atraumatic microvascular clamp. The suitability of this method and its outcomes are discussed. METHODS: It was a prospective observational study that included 32 patients of type "C" EA between January 2014 and December 2016. Babies with birth weight more than 2 kg without cyanotic heart defects and requirement of intensive care were included. An early primary anastomosis using this technique was tried in all. A cervical esophagostomy with feeding gastrostomy was done where it was not possible. Analysis of the gap and post operative outcomes i.e. gastroesophageal reflux (GER), stricture, tracheomalacia, dysmotility, recurrence and survival were analyzed. RESULTS: The mean gap between esophageal ends was 4.3 cm. Primary anastomosis was possible in 26 (81.25%). Minor and major leak occurred in 3 (11.54%) and 1 (3.85%) patients respectively. Survival was 84.62% (22/26). All mortalities were early post operative. During mean follow up of 23.73 months (till December 2019), GER decreased from 63.64% (14/22) to 13.64% (3/22), partial stricture was seen in 18.18% (4/22), tracheomalacia in 36.36% (8/22) and dysmotility in 77.27% (17/22). There was no recurrence of fistula. Complications with this method did not show any significant difference as mentioned with other methods. CONCLUSION: This technique seems to be physiologically suitable as it enables anastomosis with minimal trauma to the esophageal ends. It is easy, reproducible and produces favorable outcomes comparable with other methods for difficult cases of type "C" esophageal atresia (EA).

Gastric pull-up by the retrosternal route for esophageal replacement: Feasibility in a limited-resource scenario.

OBJECTIVES: The authors herein report the results of esophageal replacement by gastric pull-up technique through the retrosternal route as an option for esophageal replacement in a resource-constrained setup. METHOD: Prospectively collected data upon twenty-two consecutive patients (male:female = 17:5) with mean age 24.9 months (7 months-12 years) and mean weight 7.9 kg (4.2-32 kg) who underwent retrosternal gastric pull-up for esophageal atresia (n = 18; 16 atresia with distal fistula & 2 pure atresia) and corrosive injuries to the esophagus (n = 4) over the past 8 years are presented. The management protocol and surgical technique have been described. Observations parameters included indication for esophageal replacement, age at surgery, sex of the child and other demographic details, clinical and operative findings, post-operative outcomes and follow-up details. RESULTS: Retrosternal gastric pull-up could be performed in all cases with no mortality or graft loss. Of 22, 20 cases were extubated on-table and 2 cases were extubated within 48 hours of surgery. Mean operative duration was 265 min (range: 175 min to 310 min) and blood loss was 115.3 ml (range: 80-400 ml). Dense vascular adhesions in the region of the esophageal hiatus were encountered in patients with abdominal esophagostomy (n = 4) which were probably related to the local dissection at the time of previous surgery. Minor anastomotic leak was observed in 8 of 22 patients which settled spontaneously over 21 days mean period (range: 18 to 31 days). Antegrade dilatation was required in 3 of 8 cases with minor leak. None of them required revision of anastomosis. Mean follow-up duration is 63 months (range: 11 months - 94 months). Weight gain after surgery was close to or beyond the 25th centile. Symptoms of dumping syndrome or GER were not observed in our cohort. CONCLUSION: Our data have demonstrated the safety and feasibility of esophageal replacement by gastric transposition through the retrosternal route in a resource-limited setup. No significant difference has been observed from the results and complications reported in literature for the same procedure. TYPE OF STUDY: Prospective observational study / treatment study. LEVEL OF EVIDENCE: Level III.

Outcomes of preputioplasty in patients undergoing TIP urethroplasty (tubularization of incised urethral plate) for distal and mid penile hypospadias.

BACKGROUND: Preputioplasty as a part of hypospadias repair restores the normal appearance of phallus, which is especially important in distal and mid penile hypospadias. However possibility of its inherent complications such as iatrogenic phimosis or preputial breakdown are the cause of controversy and reluctance regarding this procedure.This study evaluates the results of preputial reconstruction with TIP urethroplasty in distal and mid penile hypospadias repair and analyses if preputioplasty may be offered to these patients. MATERIALS & METHODS: In this prospective observational study, 48 cases of distal and mid penile hypospadias underwent TIP urethroplasty and preputioplasty and results were assessed at 2 weeks, 3 months and 6 months. Major complications included preputial dehiscence, tight prepuce (iatrogenic phimosis) and minor complications included ventral tethering, persistent dorsal whorls and redundant prepuce. Data was analysed with Microsoft Excel spreadsheet where descriptive statistics were obtained. RESULTS: Preputioplasty was performed in 48 children with a mean age of 5.1 years. Preputioplasty dehiscence was seen in three (6%) patients, which gave an appearance of irregular prepuce on 6 m follow up. Two patients (4%) were confirmed to have preputial tightness at 3 months but this resolved conservatively in one patient and only one patient (2%) required circumcision for a tight prepuce. Minor complications included ventral tethering, persistence of dorsal whorls and redundant prepuce. Ventral tethering was present in 3 patients (6.25%). Redundant prepuce was observed in 2 patients (4.16%). Additionally, unsightly dorsal whorls were found to be persistent in 2 children (4.16%). None of these patients opted for circumcision. The rest of the children had a cosmetically and functionally normal prepuce. Two patients (4%) developed urethrocutaneous fistula at 3 months' follow-up. CONCLUSION: Preputial reconstruction is feasible with a good cosmetic outcome and minimal complications in patients of distal and mid penile hypospadias undergoing TIP urethroplasty. Mild preputial tightness evolves over time and resolves with conservative measures. In patients with very prominent dorsal whorls and underlying bulky tissues the preputioplasty does not appear to be of satisfactory cosmesis. To help the patient and parents take a well informed decision, it would be useful to explain all possible major and minor foreskin complications, and their rectification.

Mesenteric Cyst in Association with Type-II Jejunoileal Atresia.

A rare case of type-II jejunoileal atresia with mesenteric cyst in a neonate is being reported here with a brief review of literature.

Transanal impalement of double J steel bar with colonic and jejunal injury: A unique pediatric case report.

Pediatric transanal impalement injuries are relatively uncommon and most are attributed to accidental fall on offending objects, sexual assault or blunt trauma. There may be difficulty in recognizing or properly treating such injuries because their severity may not be reflected externally. Evaluation of suspected rectal impalement injury involves careful history and physical examination and proper investigation. There are very few reports on pediatric perianal impalement with associated visceral injuries. We report a case of assault transanal impalement injury associated with mesenteric tear and jejunal perforation leading to devitalization of proximal jejunum in a 2 year male child and relevant literatures were reviewed. To the best of our knowledge, such dual proximal and distal gastrointestinal injury in such a small child has not been reported in any of the English literature so far.

Management of female anterior perineal injuries with urethrovaginal disruption by anterior sagittal transanorectal approach.

OBJECTIVE: Urethrovaginal disruption injury poses a considerable challenge in surgical correction, mainly due to difficult access to the receded urethrovaginal unit deep in the scarred anterior perineum. We assessed the feasibility, safety and efficacy of the anterior sagittal transanorectal approach (ASTRA) in the repair of anterior perineal injury with urethrovaginal disruption in female children. MATERIALS AND METHODS: Three girls with urethrovaginal disruption following perineal injury were operated by ASTRA between March 2008 and December 2010. All of them had severe scarring of the anterior perineum at the time of definitive repair by this approach. One underwent ASTRA repair without a covering colostomy. RESULTS: Total urethrovaginal mobilization and anchorage of urethral and vaginal orifices at the vestibule were successfully achieved in all patients. One patient has developed vaginal stenosis at 1.5 years follow up. Despite the absence of a colostomy cover in one case, there were no wound complications in the early postoperative period. CONCLUSION: The ASTRA is safe and efficacious in the repair of traumatic urethrovaginal disruption in children.

Portal teratoma causing obstructive jaundice in children: a rarity.

Teratoma in children is a common entity, usually occurring both in gonadal and extragonadal sites. Common extragonadal sites for teratoma in children are the sacrococcygeal region, retroperitoneum, and mediastinum. Various unusual extragonadal sites have been reported. However, teratoma in the hepatoduodenal ligament is a very rare occurrence. We herein report a case of a mature teratoma in the hepatoduodenal ligament in an 11-year-old child presenting with obstructive jaundice along with its surgical management and review of the literature.

Retrocaval ureter: a rare cause of hydronephrosis in children.

Retrocaval ureter is a relatively rare developmental anomaly of the inferior vena cava. It commonly manifests in the third to fourth decade of life; however, pediatric reports of this condition are very rare. Individuals with this anomaly are symptomatic because of ureteric obstruction. All symptomatic patients need surgery where the ureter is divided and anastomosed anterior to inferior vena cava. We report our experience of a child who presented with flank pain and, on evaluation, were found to have right hydronephrosis. During surgery, retrocaval anomaly was noticed and appropriately dealt with.