Abdominoscrotal Lymphangioma Masquerading as a Communicating Hydrocele: A Case Report.

A 5-year old boy presented with a picture of communicating hydrocele and was discovered to have an abdominoscrotal lymphangioma after undergoing hydrocele surgery. Upon initial presentation the lymphangioma was missed and the child underwent inguinal approach surgery for hydrocele. The lymphangioma was then noticed as an abdominal lump due to a rapid increase in size within 1 week following the hydrocele surgery. The sudden enlargement of the lymphangioma was due to intra-cystic hemorrhage post-operatively. The lymphangioma was then completely excised with no recurrence noted after 1 year of follow up. This is a rare case of a retroperitoneal, abdominoscrotal lymphangioma masquerading as a communicating hydrocele. Keywords: Communicating Hydrocele, Abdominoscrotal lymphangioma, Scrotal Swelling.

Stage 4S Neuroblastoma: A Report of Two Cases Presenting with Extremes of Biological Behavior.

Neuroblastoma is the most common extracranial solid tumor in childhood. Stage 4S is a special stage of neuroblastoma in which majority of cases may have spontaneous regression; however, in some cases the tumor is rapidly progressive with poor prognosis and thus requires aggressive therapy. Dilemmas in its management and therapy will be discussed. We report two cases of stage 4S neuroblastoma exemplifying these two extreme behaviors. The first case is of a four-month-old baby who initially presented with a thigh lump, labial and foot nodules as well as hepatic and adrenal involvement. Following the confirmation of stage 4S Neuroblastoma with favorable histology and N-MYC negative amplification, a watchful observation approach was elected. Currently, the patient has completed two years of uneventful follow-ups with normal development. The second case is of a full-term new born baby who presented with abdominal distention and respiratory distress. Stage 4S Neuroblastoma was confirmed with an unfavorable histology and metastasis to the liver and the left adrenal gland. Due to the deterioration of the patient's condition, chemotherapy with carboplatin and etoposide was initiated for six cycles with a good and rapid response. The patient completed two years of follow up without recurrence.

Anogenital distance is determined during early gestation in humans.

STUDY QUESTION: Does cord blood androgen level obtained at birth affect the AGD in human newborns? SUMMARY ANSWER: In human newborns, though males have a significantly longer AGD compared to females (as early as 22 weeks of gestation) the AGD is not affected by androgen levels at birth in both the sexes. WHAT IS KNOWN ALREADY: Animal studies have reported a critical time period in early fetal life, termed the masculinization programming window (MPW) during which AGD is fixed by in utero androgen action and is unaffected by testosterone levels later during gestation. Thus, AGD may serve as a lifelong biomarker of androgen exposure during this window. This MPW is hypothesized to occur in humans at 8-14 weeks of gestation during which AGD is fixed. The effect of androgens (testosterone) on AGD after the MPW in humans is not known. Furthermore, altered AGD has been associated with various human reproductive health disorders in both males and females. STUDY DESIGN, SIZE, DURATION: A prospective descriptive cohort study was performed using data from randomly selected neonates (n = 205) born at a single center over a period of 1 year (August 2015 to August 2016). PARTICIPANTS/MATERIALS, SETTING, METHODS: AGDs in male (n = 117) and female infants (n = 88) together with penile width, glans girth and stretched penile length were measured by trained caregivers. Gestation ranged from 22 to 41 weeks and infants were examined within 24 h of birth (within 48-72 h in very sick preterm infants after clinical stabilization). AGD-1 was measured from the center of the anus to the posterior base of scrotum in males or to the posterior fourchette in females. AGD-2 was measured from the center of the anus to the anterior base of the penis in males or to the clitoris in females. Sex steroid hormones (testosterone, 17-OH progesterone (17-OHP) and androstenedione) were measured in serum prepared from umbilical cord blood samples taken at birth, using liquid chromatography-tandem mass spectrometry. MAIN RESULTS AND THE ROLE OF CHANCE: Males had a significantly lower gestational age (mean ± SD; 34.6 ± 4.9 versus 36.1 ± 4.1 weeks, P = 0.04), and a significantly longer AGD-1 (mean ± SD; 21.6 ± 6.0 versus 12.7 ± 3.8 mm, P < 0.001) and AGD-2 (41.9 ± 8.7 versus 33.9 ± 7.1 mm, P = 0.004) compared to female infants, respectively. The cord serum testosterone levels were significantly higher for male than female infants [median, interquartile range; 13.0 (7.3, 20.5) versus 4.1 (2.5, 5.9), ng/dl, P < 0.001]. There was no difference in levels of 17-OHP (P = 0.697) or androstenedione (P = 0.601) between the two sexes. On multiple regression analysis after adjusting for potential confounders, none of the AGD's in both males and females correlated with any sex steroid hormonal levels. We also provide normative charts for penile length, penile width and glans girth in preterm and term infants. LIMITATIONS, REASONS FOR CAUTION: No data were collected on family history of genital malformation, infertility or hormonal disorders, parental endocrine-disrupting chemical exposure or diet pattern, any of which might have influenced the AGD and/or sex steroid hormone levels in the offspring. WIDER IMPLICATIONS OF THE FINDINGS: Our results suggest that AGD in humans, like animals, is fixed in early gestation (likely during the hypothesized MPW) and is unaffected by androgen levels thereafter. Thus, AGD can serve as a biomarker of in utero androgen action during early gestation (likely 8-14 weeks) in humans. As such, causes of human newborn and adult reproductive health disorders, such as endocrine disruptors, should be explored during early gestation. However, further larger studies are needed to help corroborate these findings. STUDY FUNDING/COMPETING INTERESTS: No specific funding was obtained for this study, and all authors have no conflict of interest to declare.

Shorter anogenital distance correlates with the severity of hypospadias in pre-pubertal boys.

STUDY QUESTION: Do pre-pubertal boys with hypospadias have a shorter anogenital distance (AGD) than boys with normal genitalia? SUMMARY ANSWER: AGD is significantly shorter in boys with hypospadias and decreases with the severity of hypospadias. WHAT IS KNOWN ALREADY: Animal studies have shown that androgen disruption and exposure to endocrine disrupting chemicals during a critical time period in early gestation, termed the male programming window (MPW), result in hypospadias and reduced AGD; and the severity of hypospadias correlates with the reduction in AGD. However, this correlation has not been established in humans. STUDY DESIGN, SIZE, DURATION: A prospective descriptive study involving measurement of AGD in pre-pubertal boys (n = 458) presenting to our pediatric urology clinic with hypospadias and normal genitalia was performed over a period of 3 years. PARTICIPANTS/MATERIALS, SETTING, METHODS: AGD was measured in pre-pubertal boys from 5 months to 14 years of age presenting to our clinic with hypospadias (n = 180: four were excluded) and compared with randomly selected boys with normal genitalia (controls, n = 274). Three variants of AGD, from the midpoint of the anus to base of the scrotum (AGD-AS), to the anterior base of penis (AGD-1) and to the posterior base of penis (AGD-2), were measured and assessed for correlation with the severity of hypospadias. Severity of hypospadias was classified as anterior, middle and posterior according to the meatal location. MAIN RESULTS AND THE ROLE OF CHANCE: No significant difference in weight (P = 0.123), age (P = 0.162) or height (P = 0.591) between the two groups was observed. Only AGD-AS was significantly shorter in boys with hypospadias compared with controls (mean ± SD: 40.6 ± 9.7 mm versus 45.6 ± 9.4 mm, P < 0.001). This relation persisted after adjusting AGD for weight, height and age (ß = 0.016, 95% confidence interval: 0.10-0.21; P < 0.001). The Spearman test showed a significant negative correlation for the severity of hypospadias with all the three AGD measures. Analysis of variance between anterior, middle and posterior subgroups showed a significant reduction in mean AGD-AS (P = 0.003) and AGD-2 (P = 0.008). LIMITATIONS, REASONS FOR CAUTION: No data were collected pertaining to in utero exposure to endocrine disrupting chemicals (EDCs) or cigarette smoke, or current diet and environmental exposure to EDCs, which may have influenced the AGD. Family history of genital malformation and use of IVF were not known. There may have been a selection bias as only boys presenting to our clinic were included. WIDER IMPLICATIONS OF THE FINDINGS: The findings suggest that prenatal androgens during early gestation influence development of the male reproductive system and support the existence of a MPW in humans. Of the three AGDs, AGD-AS may be the most reliable biomarker of this in utero androgen action. However, no direct link to any specific exposure leading to shortened AGD in pre-pubertal boys with hypospadias could be determined. Further large scale multi-center studies are needed to understand this association better. STUDY FUNDING/COMPETING INTERESTS: Funding was from the Hypospadias Foundation. No conflicts of interest to disclose.

Transverse preputial onlay island flap urethroplasty for single-stage correction of proximal hypospadias.

AIMS AND OBJECTIVES: Transverse preputial onlay island flap urethroplasty (TPOIF) was described initially for distal hypospadias, but has seen extended application for proximal hypospadias. We describe a set of modifications in the technique and results in a large series of proximal hypospadias. MATERIALS AND METHODS: All children who underwent TPOIF repair for proximal hypospadias (proximal penile, penoscrotal and scrotal) from June 2006 to June 2013 by a single surgeon were prospectively followed till June, 2014. A standard technique and postoperative protocol were followed. Salient points to be emphasized in the technique: (1) dissection of the dartos pedicle till penopubic junction to prevent penile torsion, (2) incorporation of the spongiosum in the urethroplasty, (3) midline urethral plate incision in glans (hinging the plate), (4) Dartos blanket cover on whole urethroplasty. RESULTS: Out of 136 children with proximal hypospadias, 92 children who underwent TPOIF formed the study group. Out of 92 children, 48 (52 %) children required a tunica albuginea plication for chordee correction. In total, 16 (17 %) patients developed 24 complications and 11 children (12 %) required second surgeries: fistula closure in 7 (with meatoplasty in 5), glansplasty for glans dehiscence in 2 and excision of diverticulum in 2. Two children required a third surgery. Only 5 children had a noticeable penile torsion (less than 30 degree), and 7 had a patulous meatus. CONCLUSIONS: Transverse preputial onlay island flap urethroplasty can deliver reliable cosmetic and functional outcomes in proximal hypospadias.

Undescended testis and torsion: is the risk understated?

Undescended testis (UDT) is seen in 3% to 5% of all newborn boys. Complications such as infertility and malignant transformation have been well documented in UDT. However, torsion of a UDT can also occur and the diagnosis is often missed or delayed, leading to loss of testis. This event may occur even before the currently recommended age for surgery, which is at 6-9 months. We present a case series of six children with torsion of undescended testes and their subsequent diagnosis and management. The risk of torsion of UDT is understated. Paediatricians should be educated about this complication and torsion should be included in the differential diagnosis when a boy with an empty scrotum presents with acute abdomen or red and tender swelling in the groin, as early detection and intervention can help salvage the testes.

Local steroid therapy as the first-line treatment for boys with symptomatic phimosis - a long-term prospective study.

AIM: Phimosis is a common paediatric urological disorder and often necessitates circumcision. We prospectively evaluated local steroid therapy (LST) as the first choice therapy for such children. METHODS: Two hundred and sixty symptomatic boys up to 15 years of age (mean 34 months) with phimosis were started on betamethasone dipropionate (0.05%) application on gently stretched prepuce twice a day. Follow-up visits were arranged at the end of weeks 1, 2 and 4 and 6 months. Grade of phimosis was objectively graded. RESULTS: Ninety one percent of the boys showed a successful outcome at the end of 4 weeks; 72% responded in first week, further 16% responded in week 2, and only 2.6% achieved alleviation of phimosis on further application of LST beyond 2 weeks. Fourty two (17.8%) boys had a recurrence of phimosis on a long-term follow-up (mean - 25.4 months, range 6-48 months); thus, the long-term success rate was 77%, while 60 (23%) boys underwent surgery. CONCLUSION: Local steroid therapy is safe and successful in alleviating symptomatic tight foreskin in a large majority of children. The response can be seen as early as 1 week; most of the children respond by week 2 and continuing therapy further may not be very effective.

3D multidetector CT angiographic evaluation of intralobular bronchopulmonary sequestration.

We report a case of intralobar pulmonary sequestration with special emphasis on computed tomography (CT) angiography in determining the arterial supply and venous drainage, thus providing a detailed knowledge of the vasculature, which is of vital importance in surgery. The 3D volume rendering technique and maximum intensity projection images provide the vascular road map for the surgeon.

Lower urinary tract obstruction secondary to congenital bladder diverticula in infants.

PURPOSE: Congenital primary bladder diverticulum is a rare condition and may present with urinary infection; other forms of presentation are rare. We present a series of infants who presented with urinary retention secondary to large primary bladder diverticulum. METHODS: Seven infants were evaluated for symptoms of lower urinary tract obstruction. All seven were infant boys; three were neonates. Investigations included ultrasonogram, voiding cystourethrogram (VCUG) and cystoscopy. RESULTS: Six infants had single large diverticulum while one had bilateral diverticula. VCUG was diagnostic in all cases demonstrating the mechanism of obstruction clearly except one where bilateral diverticula was diagnosed only on cystoscopy prior to definitive surgery. Five children (including one neonate) underwent successful definitive repair consisting of diverticulectomy and ureteral re-implant while two neonates were planned for a staged correction. One neonate later in the series underwent definitive primary repair as bladder was good sized. All five children have done well after definitive repair at a follow-up of 6-72 months. CONCLUSION: Primary bladder diverticulum in infants may present with lower urinary obstructive symptoms indistinguishable from posterior urethral valves. A carefully done VCUG can help in diagnosis. Primary definitive repair can be undertaken even in first few months of life with good results.

Preduodenal portal vein in association with midgut malrotation and duodenal web-triple anomaly?

Preduodenal portal vein (PDPV) is a rare anomaly in which the portal vein passes anterior to the duodenum rather than posteriorly. Generally asymptomatic, PDPV may rarely cause duodenal obstruction or may coexist with other anomalies. We report a neonate who presented with duodenal obstruction and was found out to have 3 coexisting anomalies, each of which can lead to duodenal obstruction independently-PDPV, midgut malrotation, and duodenal web. A duodenoduodenostomy and a Ladd procedure were done, and the child recovered uneventfully. The mechanism of obstruction, interesting metabolic aberrations observed, outcome, and relevant literature are presented.

Transmural intrathoracic lipoma with intraspinal extension.

A rare case of transmural dumbbell intrathoracic lipoma with intraspinal extension is reported. Preoperative diagnosis was possible with a computed tomographic scan and fine-needle aspiration cytology. Complete excision was curative. The chest wall defect was repaired with Prolene mesh. Only one similar case has been previously reported in the English literature.

Bilateral communicating bronchopulmonary foregut malformations in a child.

Communicating bronchopulmonary foregut malformations are rare anomalies. The complex anatomy requires innovative surgical techniques. We report a child with bilateral sequestrations communicating with the lower esophagus. The sequestrations were excised through a single thoracotomy incision and the esophagus was repaired. Postoperatively the child has remained asymptomatic.

Oesophageal duplication cyst causing neonatal haemoptysis.

Duplication anomalies of the alimentary tract often present with myriad clinical symptoms and signs. There have been no reports of haemoptysis as the presenting feature and oesophageal duplication cyst is not considered as a cause for haemoptysis. An infant presented with this unusual manifestation. Chest X-ray showed persistent right upper zone haziness and contrast enhanced computed tomographic scan showed a multiloculated mass on the right side of the chest. The cystic mass was excised via a right thoracotomy and dense adhesions were noted with the oesophagus. The postoperative period was uneventful and histopathological examination revealed it to be a duplication cyst with ectopic gastric mucosa. Thus, the diagnosis was made retrospectively.