Should Pediatric Surgical Oncologists Receive Training in Vascular Surgery ? A Practice Survey Report From the International Society of Paediatric Surgical Oncology (IPSO).

BACKGROUND: Is vascular training in paediatric surgical oncology considered desirable ? METHODS: A voluntary survey of work practice was undertaken with the surgeon membership of The International Society Of Paediatric Surgical Oncology (IPSO) using a structured designed questionnaire. RESULTS: A total of 149 IPSO surgeon members completed the survey. 57% (N = 84) of surgeons surveyed had no specific training in vascular surgery. 43% surgeons (N = 63) stated they had acquired some skills in residency training and/or with transplantation surgery. 65% (N = 96) of respondent surgeons stated that vascular surgical training must be incorporated into pediatric surgical oncology training and 27% (N = 40) agreed that it was considered desirable. 89% (N = 133) of surgeon respondents had encountered major vascular injury during work practice while operating on pediatric solid tumors. Vascular injury repairs were undertaken and attempted by pediatric surgeons though expert assistance of vascular surgeons proved crucially essential in many instances. Emergent operations included patch repairs, vessel ligation techniques and insertion of vascular graft prostheses. Interventional radiology services to arrest life-threatening hemorrhage were also reportedly utilized by respondents. CONCLUSION: Vascular injuries have significant potential for devastating patient outcomes including never event 'mortality'. The IPSO surgeon survey highlights that there are visible 'gaps' in skills training. Training to be a pediatric oncology surgeon must incorporate acquisition of skill sets proficiency in vascular surgery.

Pulmonary Actinomycosis in Children: A Prompt Diagnosis Can Prevent Surgery.

Pulmonary actinomycosis is an extremely rare disease in children. The diagnosis is challenging as the clinical presentation and radiological investigations may be atypical. We report a case of a pulmonary lesion extending to the chest wall posing as a malignant lesion. Biopsy revealed colonies of Actinomyces. Antibiotic therapy resolved the mass. A rare diagnosis of pulmonary actinomycosis should be kept as a differential diagnosis of such a mass in children.

Transverse testicular ectopia with persistent mullerian duct syndrome.

Persistent mullerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism caused by defects in synthesis or actions of mullerian inhibiting factor characterised by persistence of mullerian duct structures in a normal karyotype male. Transverse testicular ectopia (TTE) is a rare disease in which both testes are located in the same inguinal canal. We report a case of PMDS with TTE in a 1-year-old child who presented with non-palpable testis on right side with hernia on left side. Left herniotomy with bilateral trans-septal orchidopexy was done in this patient.

Sacrococcygeal Teratoma in a Seven-Day-Old Child with Pulmonary Differentiation.

Sacrococcygeal Teratoma (SCT) showing pulmonary differentiation has been rarely reported in the literature. Till date, only eight cases have been reported, out of which six belong to teratomas arising from female genital tract. Spinal teratomas showing pulmonary differentiation are rarer and only two cases have been reported so far in the literature both of which had associated congenital spinal anomaly. We present a rare case of seven day old infant with sacrococcygeal teratoma showing mature lung differentiation as one of its components. The infant was otherwise asymptomatic and had no associated spinal anomaly. This case has been presented for its rarity. Extensive review of the literature along with the possible pathogenesis is also included for better understanding.

Type V Pouch Colon, Prune Belly Syndrome, and Congenital Anterior Urethrocutaneous Fistula.

Congenital pouch colon (CPC) or short colon syndrome is a rare type of anorectal malformation(ARM). Type V is the rarest form of CPC. We present a 1-day-old male child with type V CPC with prune belly syndrome and congenital anterior urethrocutaneous fistula (CAUF).

Role of Interferential Therapy in Children with Fecal Incontinence Postanorectal Malformation Surgeries.

BACKGROUND: Anorectal malformation (ARM) is one of the most common pediatric surgical problems dealt in day-to-day practice. Although the outcome of surgery has improved a great deal over the last three decades fecal incontinence (FI) is still a common long-term morbidity that affects the quality of life of these patients. Bowel wash (BW) program with pelvic floor exercise are standard care of management for these patients. This study was undertaken to assess the role of interferential therapy (IFT) along with BW compared to BW alone in the management of FI. METHODOLOGY: Twenty-four children more than 2-year-old age previously operated for malformation and having FI with Kelly score <4 were recruited and assigned to one of the two groups. One group was given standard BW regimen and the other BW with IFT for 3 months. Posttherapy Kelly scoring was done, and the results were compared. RESULTS: IFT with BW provided no added advantage over BW alone for the treatment of FI in patients of ARMs. CONCLUSION: The time-tested modality of BW is the cornerstone in the management of FI.

Congenital Syngnathia; Turmoils and Tragedy.

Congenital syngnathia is an extremely rare condition with no standardized treatment. We hereby report a case highlighting the difficulties faced in its management and the postoperative complications.

Metanephric Adenofibroma Masquerading as Wilms' Tumor.

Metanephric adenofibroma is a rare, biphasic, benign tumor containing both stromal and epithelial components and could be potentially mistaken as Wilms' tumor (WT). We present a 5-year-old girl who was suspected to have metastatic Wilms' tumor on radiological investigations/tru-cut biopsy and had received neoadjuvant chemotherapy, but postoperatively final histopathology revealed it as metanephric adenofibroma. No postoperative chemotherapy was given.

Symptomatic Mullerian Duct Cyst in a Male Infant.

Symptomatic Mullerian duct cyst is a rare entity in children. A 9-month-old male infant presented with bowel and urinary obstructive symptoms. Imaging investigations revealed a cystic mass in the rectovesical pouch compressing bladder neck and rectum. At laparotomy, a Mullerian duct cyst was found. Most of the cyst was excised and the remaining cyst mucosa was cauterized. The child improved thereafter.

Antero-posterior Duplicate Exstrophy with a Wet Bladder Plate: A Diagnostic Dilemma.

Variants of exstrophy are rare anomalies seen in the spectrum of bladder exstrophy-epispadias complex. We present a rare case of duplicate exstrophy with a wet bladder plate. This is a deviation from the classical description of antero-posterior duplicate exstrophy that is associated with a dry bladder plate.

Thoracoscopic excision of foregut duplication cyst: A case report and review of literature.

Thoracoscopy has major advantages over thoracotomy. We report a successful management of foregut duplication cyst thoracoscopically in a child with review of literature.

Primary Synovial Sarcoma of Lung: A Rare Tumor.

Synovial sarcoma of lung is a rare tumor with few case reports in literature. Though named synovial sarcoma due to its resemblance to synovium on light microscopy, it arises from mesenchymal tissue. Here, we present a case of synovial sarcoma of lung in a 7-year old boy, with main emphasis on difficulty faced in the management.

Recurrent Monophasic Wilms' Tumor in Pelvic Kidney - A Therapeutic Challenge.

Wilms' tumor in the current era of multimodality treatment has promising outcome, but approximately 10-15% of the patients with favorable-histology, experience tumor progression or relapse. We hereby present an unusual case of repeated loco-regional recurrences in a patient with stage I intermediate-risk monophasic (epithelial variant) Wilms' tumor (WT) of pelvic kidney requiring aggressive therapy over a decade and lay emphasis on the importance of initial completion of therapy and need for long term follow-up.

Segmental dilatation of ileum associated with anterior thoracolumbar meningomyelocele and bilateral undescended testes.

Segmental dilatation of ileum (SDI) is a rare clinical entity and so is anterior thoracic meningomyelocele (AMC). There has been no reported association between these two clinical entities. We hereby report a very rare presentation of these two in a 4 year old boy who presented with swelling in the right lower abdomen. Preoperative diagnoses were partial cecal volvulus and duplication cyst. At operation, SDI along with AMC was found.