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Anorectal malformations (ARM) in females are identified by abnormal location of the anal opening. Management is guided by clinical examination to find the number of perineal openings. Two openings in the perineum of a female may be seen in cases of imperforate anus without fistula, vaginal agenesis with vestibular fistula or imperforate anus with recto-vaginal fistula (RVF). We present a case series of ARM with RVF and discuss their diagnosis and management.

Introduction: Posterior urethral valve (PUV) is life threatening congenital anomaly of urinary tract. Aim of the study was to correlate urethral ratio (UR) and bladder wall thickness (BWT) with cystoscopic findings in PUV patients to assess residual valves and to validate UR as a diagnostic tool for residual valves. It also aimed to assess the utility of bladder wall thickness in diagnosis of residual valves. Materials and Methods: A total of 31 patients were included in the prospective study done from 2017 to 2019. Calculation of UR was done in oblique VCUG films by dividing maximum posterior and anterior urethral diameter without the catheter insitu. Measurement of BWT was done at dome and bladder neck at full distension with feeding tube insitu and was done at same volume in follow up. The procedure was repeated at 3 months follow up. The findings were compared with cystoscopic findings for the status of residual valves as gold standard. Each patient served as control for self in the study. Results: Median age of presentation was 1 years with range of 1day to 10 years. The most common complains at the time of presentation in our study was straining (35.48%) followed by antenatally diagnosed patients (25.81%) and recurrent UTI (19.36%). Pre fulguration median UR was 2.45. Post Fulguration median UR was 1.20. It showed a statistically significant reduction (p < 0.001) after fulguration. Pre fulguration median BWT was 4 mm. Post fulguration median BWT was 2.5 mm. BWT showed a statistically significant reduction (p < 0.001) after fulguration as well. ROC curve was plotted for UR and BWT. BWT more than 1.95 mm (sensitivity-80%) and UR more than 1.2 (sensitivity-70%) indicates residual valves. Conclusion: A step ladder approach including BWT, UR and check cystoscopy can serve as a new diagnostic algorithm for the assessment of residual valves thereby avoiding extra radiation and general anesthesia exposure.

BACKGROUND Hyponatremic-hypertensive syndrome (HHS) is characterized by combination of polyuria, polydipsia, hypertension, hyponatremia and hypokalemia in association with unilateral renal artery stenosis. CASE CHARACTERISTICS A 10-year- old girl presented with polyuria, polydipsia, hypertension, hyponatremia, hypokalemia and proteinuria. Ultrasonography with doppler study revealed bilateral normal renal arteries. Completed tomography of abdomen detected a left adnexal mass, which was later confirmed as ovarian paraganglioma on histopathology. OUTCOME After tumor excision, polyuria subsided and blood pressure normalized. MESSAGE Hyponatremic-Hypertensive Syndrome does not always result from unilateral renal artery stenosis. High index of clinical suspicion with appropriate imaging technique may clinch rare endocrine causes of hypertension, like paraganglioma.

Gorham–Stout disease (GSD) is a very rare disorder of unknown etiology, characterized by the abnormal proliferation of lymphatic endothelial cells, and also called “vanishing bone disease,” “phantom bone disease,” and “massive osteolysis,” as osteolysis is a characteristic feature. Patients present with a variety of symptoms such as swelling, pain, physical disability and deformity, shortness of breath, and neurological symptoms. It is a progressive disease, and owing to its rarity, the research and treatment options are limited. Our patient is a 15-year-old male, who presented with large mass over abdomen and chest, chylothorax, and osteolysis. This patient was initially treated with chest drains, subcutaneous drains, and sclerotherapy. Eventually, symptoms were worsened. After literature search, the patient was diagnosed with GSD, and sirolimus therapy was started. After 2 months of sirolimus therapy, the lesions subsided and well controlled.

Spontaneous or idiopathic biliary perforations are an infrequently encountered but an important cause of surgical jaundice in paediatric patients and one of the most common causes of surgical jaundice in infancy. A pre-operative diagnosis with a clinical history and physical findings may not be possible in most of the cases. The exact cause of the perforation remains unclear and the diagnosis is made at the time of laparotomy for an acute abdomen. An early, efficient and an effective surgical management is associated with a good prognosis; however, a delay in the correct diagnosis or an inappropriate management may result in bacterial contamination of the biliary ascites, with an unfavourable outcome. The relative rarity of this condition is reflected by the very few case reports, limited case studies and scarcity of published literature.

Lumbocostovertebral syndrome (LCVS) is a rare type of congenital lumbar hernia. Its features include lumbar hernia associated with genitourinary, vertebral, and rib anomalies. Less than 25 cases have been reported to date. We describe the clinical manifestations and associated anomalies in 6 cases of LCVS managed by us. The patients with suspected LCVS syndrome should be evaluated by detailed clinical history, examination, conventional radiography, magnetic resonance imaging spine, ultrasound abdomen, and echocardiography. The defect can be repaired using local tissues in infancy. There is a need for long-term follow-up for possible late recurrence and scoliosis.

OBJECTIVES: To determine how often neuroimaging confirms, clarifies, or contradicts initial diagnoses of late-life cognitive disorders. DESIGN: Retrospective case review. SETTING: Outpatient clinic specializing in memory disorders. PARTICIPANTS: One hundred ninety-three consecutively referred cognitively impaired patients. MEASUREMENTS: Diagnoses using research criteria were developed for each patient at the first visit and ranged from cognitive impairment without dementia to dementias of single, complex, or indeterminate etiology. Structural (noncontrast magnetic resonance imaging) and perfusion (technetium-99m ethyl cysteine dimer single photon emission computed tomography) images were categorized together as normal, suggestive of specific diseases, or abnormal/not diagnostic. RESULTS: When a single neurodegenerative disease was suspected clinically (n=94), imaging confirmed the diagnosis in 50, contradicted the diagnosis in 32, and was abnormal/not diagnostic in 12. When more than one neurodegenerative etiology was clinically suspected (n=21), imaging assigned a single diagnosis in 13 and only cerebrovascular disease in one and was abnormal/not diagnostic in seven. In dementia not otherwise specified (NOS) (n=33), imaging suggested a specific etiology in 23 and was abnormal/not diagnostic in 10. Abnormal/not diagnostic images were more common in cognitive disorder NOS (n=25, 68%) than in other clinical groups (22%, chi-square=22.8 P

Adult PET/CT acquisition protocols need to be modified for pediatric imaging to minimize the radiation dose while maintaining diagnostic utility. We developed pediatric PET/CT acquisition protocols customized to patient weight and estimated the dosimetry and cancer risk of these low-dose protocols to communicate basic imaging risks. Methods: Protocols were developed for whole-body 18F-FDG imaging of patients in PET mode with a weight-based injected activity (5.3 MBq/kg) and acquisition times (3–5 min/field of view) and for CT for attenuation correction and localization with a weight-based tube current ranging from 10 to 40 mAs. Patients were categorized on the basis of the Broselow–Luten color-coded weight scale. Dosimetry and radiation-induced cancer risk for the PET and CT acquisition in each category were derived from mean patient sizes and the interpolation of factors from accepted patient models. Results: Whole-body pediatric PET/CT protocols require the customization of PET-acquisition settings and task-specific selection of CT technique. The proposed weight-based protocols result in an approximate effective dose ranging from 8.0 mSv for a 9-kg patient up to 13.5 mSv for a 63-kg patient. The radiation dose from the proposed protocols is 20%−50% (depending on patient weight), the dose from PET/CT protocols that use a fixed CT technique of 120 mAs and 120 kVp. The approximate, conservative estimate of additional lifetime attributable risk (LAR) of cancer incidence for females using the proposed protocols was approximately 3 in 1,000, with a variation of 18% across patient categories. For males, the additional LAR of cancer incidence was approximately 2 in 1,000, with a variation of 16% across categories. Conclusion: Low-dose PET/CT protocols for 11 patient weight categories were developed. The proposed protocols offer an initial set of acquisition parameters for pediatric PET/CT. The use of multiple categories allows for the continued refinement of dose-reduction parameters to minimize dose while maintaining image quality across the range of pediatric patient sizes.

Management of pyothorax and pneumothorax requires aspiration and/or intercostal drainage.We present two cases which were complicated by instrument-related events resulting in breakage of needle or intercostal drainage tube resulting in pleural foreign body.The patients were stabilized and the foreign bodies retrieved using thoracoscopic approach.Thoracoscopy provides a minimally invasive approach to deal with pleural foreign bodies.

We report a four-yr-old girl who was successfully treated for a large gastro-duodenal duplication that communicated with extra-lobar pulmonary sequestration on one end and the main pancreatic duct on the other. Such an association has not been reported hitherto.

Bronchogenic cysts occur due to the anomalous development of the primitive tracheobronchial tree early in fetal life. They are usually present in middle mediastinum. Rarely, they have been found in other locations. We describe two patients with subcutaneous bronchogenic cysts located over manubrium sterni with special emphasis on the difficulties in pre-operative diagnosis. The two boys were managed by complete excision of the cysts. The children are well on follow-up.

Urothelial polyps arising from the anterior urethra are rarely encountered in the pediatric age group and are even rarer in the females. Scarcely reported in English literature, their exact incidence is not known. They may be asymptomatic for years or present with features of urinary obstruction, mass or "vaginal" bleeding. They are important in the differential diagnosis of interlabial masses in female children. Anterior urethral polyps are benign fibroepithelial lesions. Surgery is indicated for relief of symptoms and differentiation from malignant lesions such as a sarcoma or a papilloma. No recurrences have been reported. We report a case of solitary anterior urethral polyp in an 8-year old girl managed successfully at our centre.

Tuberculosis (TB) is widely prevalent in developing nations and has recently made a comeback in industrialized countries, with the rise in immunocompromized patients. Musculoskeletal TB in children presents a diagnostic challenge because it is difficult to recognize in the early stages of the disease, and imaging features mimic other entities. The clinical onset is insidious, with an indolent course and a resultant late presentation. It leads to significant morbidity; a delay in diagnosis can cause potentially serious neurological complications and bone and joint destruction. Conventional radiographs are the initial imaging modality and US, CT and MRI are used in conjunction to better delineate the disease extent and morphology. Radiologists should be familiar with the spectrum of imaging features of TB, including plain radiographs and MRI, and aid the clinician in making an early diagnosis. Aspiration or biopsy with examination for acid-fast bacillus and histological evaluation is required to confirm the diagnosis.

Background: To assess the prognosis of surgical neonates at admission and the factors responsible for mortality in neonates. Material and Methods: A prospective study was conducted in a tertiary level hospital over 15 months and various clinical and biochemical parameters were collected and analyzed using STATA® and SPSS®. Results: On multivariate analysis of 165 neonates, early gestational age, respiratory distress and shock at presentation were the factors of poor prognosis in neonates. The factors could be related to poor antenatal care and sepsis acquired before transfer of the baby to the nursery. Conclusion: The improvement in antenatal care and asepsis during transfer and handling the babies is of utmost importance to improve the prognosis of surgical neonates.

Pediatric 18F-FDG dosing and acquisition durations are generally based on coarse extrapolation from adult guidelines. This study sought to determine whether shorter acquisition durations or a lower 18F-FDG injected activity could be used for pediatric 18F-FDG PET/CT examinations while maintaining diagnostic utility. Reduction of overall scan time potentially reduces motion artifacts, improves patient comfort, and decreases length of sedation. Alternatively, decreased 18F-FDG dose minimizes radiation risk. Methods: Fourteen whole-body 18F-FDG PET/CT examinations were performed on 13 patients (weight, 13–109 kg; age range, 1–23 y) with a weight-based injected activity (5.3 MBq/kg [0.144 mCi/kg]), fixed acquisition durations (3 min/field of view [FOV] if 22 kg), and list-mode acquisition. For each examination, the list-mode data were truncated to form multiple datasets with shorter acquisition durations down to a minimum of 1 min/FOV (i.e., 1, 2, 3, 4, and 5 min/FOV data were formed from single 5 min/FOV acquisition). Fifty-six image volumes were generated, randomized, and reviewed in a masked manner with corresponding CT image volumes by 5 radiologists. Overall, subjective adequacy and objective lesion detection accuracy by body region were evaluated. Results: All examinations with maximum acquisition duration were graded as adequate and were used as the reference standard for detection accuracy. For patients less than 22 kg, 1 of the 3 PET/CT examinations was graded as inadequate for clinical tasks when acquisition duration was reduced to 2 min/FOV, and all examinations were graded as inadequate when reduced to 1 min/FOV. For patients more than 22 kg, all 3–5 min/FOV studies were graded as adequate, and 2 of the 9 studies were graded as inadequate for 2 min/FOV studies. Lesion detection accuracy was perfect for acquisition times between 3 min/FOV and 5 min/FOV for all regions of the body. However, lesion detection became less accurate when imaging acquisition time was reduced more than 40%. Conclusion: Evaluation of image volumes generated from simulated shorter acquisition durations suggests that imaging times for larger patients (>22 kg) can be reduced from 5 min/FOV to 3 min/FOV without a loss of diagnostic utility. Using decreased acquisition times as a surrogate for 18F-FDG dose, 18F-FDG dose can be reduced by approximately 40% when all patients were scanned for 5 min/FOV.

To determine how often neuroimaging confirms, clarifies, or contradicts initial diagnoses of late-life cognitive disorders.Retrospective case review.Outpatient clinic specializing in memory disorders.One hundred ninety-three consecutively referred cognitively impaired patients.Diagnoses using research criteria were developed for each patient at the first visit and ranged from cognitive impairment without dementia to dementias of single, complex, or indeterminate etiology. Structural (noncontrast magnetic resonance imaging) and perfusion (technetium-99m ethyl cysteine dimer single photon emission computed tomography) images were categorized together as normal, suggestive of specific diseases, or abnormal/not diagnostic.When a single neurodegenerative disease was suspected clinically (n=94), imaging confirmed the diagnosis in 50, contradicted the diagnosis in 32, and was abnormal/not diagnostic in 12. When more than one neurodegenerative etiology was clinically suspected (n=21), imaging assigned a single diagnosis in 13 and only cerebrovascular disease in one and was abnormal/not diagnostic in seven. In dementia not otherwise specified (NOS) (n=33), imaging suggested a specific etiology in 23 and was abnormal/not diagnostic in 10. Abnormal/not diagnostic images were more common in cognitive disorder NOS (n=25, 68%) than in other clinical groups (22%, chi-square=22.8 P.001). Neuroimaging indicators of cerebrovascular disease were common (60% prevalence) but not predicted by the presence of vascular risk factors alone.Overall, neuroimaging confirmed, clarified, or contradicted the initial clinical diagnosis in more than 80% of patients, whereas fewer than 20% had abnormal/not diagnostic patterns. Imaging suggested a complex dementia etiology in 21% of cases clinically thought to be caused by a single process, whereas 46% of complex clinical differential diagnoses appeared to reflect a single causal pattern. Further work is needed to determine whether refinement of clinical diagnoses using specialized neuroimaging improves clinical decision-making and patient outcomes.

Recurrent respiratory papillomatosis (RRP) is an indolent and primary disease of the larynx and the trachea and rarely extends to smaller airways. F-18 fluoro-2-deoxy-d-glucose positron emission tomography and computed tomography (FDG-PET/CT) evaluation in this condition has not been reported earlier. We report FDG-PET/CT findings in a case of RRP that included a large papillomatous lung mass resembling cancer.

Gastrointestinal duplications are a common type of congenital anomaly usually restricted to one part of the gut. Triplication of colon is a rare variation of duplication of the hindgut. Hindgut duplications are commonly associated with anomalies of other derivatives of the cloaca, namely, the lower genitourinary system. Here, we describe a child with triplication of colon with associated duplication of bladder and urethra and diphallus. The child described here is the only reported survivor with triplication.

Labial synechiae, also referred to as labial adhesion or labial agglutination, is a disorder of the female genitalia characterized by thin, membranous adherence of the labia minora. Typically, the fusion originates from the posterior fourchette and advances toward the clitoris. Complete labial fusion may conceal the vaginal introitus completely. Partial labial fusion is also possible and may occur near the posterior fourchette.

Introduction: Pancreatic heterotopia has been described most frequently in stomach and duodenum. Isolated occurrence of heterotopic pancreas in ileum is exceptional and is usually discovered incidentally during surgery. In rare cases, heterotopic pancreas has been reported as a lead point of ileoileal intussusception. case report: A one-year-old boy was presented with recent onset of constipation and abdominal distention. radiologic investigation suggested a diagnosis of midgut volvulus with ileoileal intussusception. resection anastomosis of the intussusception was performed. Histologic examination of the resected specimen showed heterotopic pancreas in the wall of intussusceptum at its tip. No other significant pathology was found. conclusion: Ileal heterotopic pancreas is a rare cause of intussusception at any age and is usually not evident clinically. Hence, careful examination of the lead point of intussusceptum in resection specimens is mandatory to delineate the underlying etiology of these cases.

Clitoromegaly in pediatric and adolescent age group is usually indicative of a disorder of sexual differentiation. We report a girl child presenting with clitoral enlargement due to an epidermoid cyst. The cyst was excised with complete cosmetic recovery.

Introduction: Hypospadias repair is done in many centers using tubularized incised plate urethroplasty technique. A varying length of distal urethra in almost one-fourth of patients is devoid of corpus spongiosum. Traditionally, this segment is incised proximally till 'healthy' urethra is reached. It makes hypospadias more proximal and thus increases chances of failure. Materials and Methods: We tried to preserve this thin so-called hypoplastic urethra in nine patients. The meatus was distal penile in four, mid-penile in four and proximal penile in one patient. Another patient with chordee without hypospadias with hypoplastic urethra underwent chordee correction without sacrificing any length of urethra. Results: No residual chordee was seen in any of the patients. Only two patients (22%) developed subcoronal fistulas needing secondary repair. No patient had urethral diverticulum or stricture. Conclusions: We thus recommend preservation of hypoplastic urethra whenever possible.

Aim: We present here experience of a single surgeon with Mitchell's procedure for correction of epispadias. Materials and Methods: Nine boys (mean age 5½ years, range 9 months to 16 years) underwent Mitchell's repair in Department of Pediatric Surgery over a period of 5½ (September 1999 to March 2005) for correction of epispadias. Six of these patients had come for the second stage of exstrophy-epispadias repair after primary bladder closure; the other three had incontinent penopubic epispadias. Results: The penis was cosmetically acceptable as regards to size, glans shape and peno-pubic angle in all the patients. However, there was a high incidence of penopubic fistula (44%). These patients with penopubic fistula also required postoperative urethral dilatations, at times repeated. One of the common factors to these subset patients was their younger age when Mitchell's urethroplasty was performed. Limitations: The series is descriptive in nature, short in numbers and does not provide statistical comparison of Mitchell's procedure with the previously done procedures. Conclusions: Mitchell's complete penile disassembly technique for epispadias repair is more acceptable anatomical procedure that results in near-pendulous penis. However, when performed at young age, it is fraught with the complication of penopubic fistula similar to that as seen with Cantwell-Ransley's procedure. Mitchell's procedure creates a hypospadiac meatus initially and the meatal advancement is required as for any other distal penile/coronal hypospadias.