Frequency of lichen sclerosus in children presenting with phimosis: A systematic histological study.

BACKGROUND: The incidence of preputial lichen sclerosus (PLS) among children presenting with phimosis varies from 10 to 95%, depending on the age, the protocol for the treatment of pediatric phimosis, the method of diagnosis (clinical versus histological), and case mix (congenital versus acquired phimosis). OBJECTIVE: PLS may not be clinically obvious. Our aim is to show that a systematic histological examination of the prepuce may diagnose PLS in clinically unsuspected cases. METHODS: Prospective observational study of the histology of all prepuces resected from boys undergoing circumcision for phimosis but not clinically suspected to have PLS. RESULTS: PLS was diagnosed histologically in 22 boys (32%). Boys with PLS were significantly older (mean 8.4 versus 4.7 years old). Diagnosis of PLS was not related to the degree of phimosis (summary figure). In three patients (grade 4 phimosis) glans discoloration was observed during surgery, and all had PLS. Follow up for boys found to have PLS ranged from 1 to 10 years. One patient developed recurrent phimosis, attributed to inappropriate conservative resection, and required further surgery. There were no cases of meatal stenosis. DISCUSSION: Subtle cases of PLS may be difficult to detect clinically. Children are frequently asymptomatic, except for being unable to retract the prepuce. Physical examination has a low negative predictive value for the diagnosis of PLS. Complete removal of the prepuce with permanent glans exposure is regarded as essential to cure PLS and to avoid recurrent phimosis, but our patients were treated with partial circumcisions for cultural reasons. Only one needed reoperation for recurrent phimosis. CONCLUSION: Histological PLS was present in approximately 1/3 of boys with phimosis, frequently without typical manifestations. Those patients may be cured with partial circumcisions.

Thyroid-like follicular carcinoma of the kidney presenting on a 10 year-old prepubertal girl.

The very rare thyroid-like carcinoma of the kidney (TLCK) is microscopically similar to thyroid follicular cell carcinoma (TFCC). Differential diagnosis with secondary thyroid tumors depends on non-reactivity to immunohistochemical (IHC) markers for TFCC (thyroglobulin - TG and TTF1). We herein describe the fourth Pediatric case in literature and extensively review the subject. Only 29 cases were published to the moment. Most cases were asymptomatic and incidentally detected. Most tumors are hyperechoic and hyperdense with low grade heterogenous enhancement on CT and MRI. Most patients were treated with radical nephrectomy, but partial nephrectomy was used in some cases, apparently with the same results. Metastases are uncommon and apparently do not change prognosis, but follow-ups are limited. Up to the moment, TLCK presents as a low grade malignancy that may be treated exclusively with surgery and frequently with partial kidney renal preservation. A preoperative percutaneous biopsy is a common procedure to investigate atypical tumors in childhood and adult tumors. To recognize the possibility of TLCK is fundamental to avoid unnecessary thyroidectomies in those patients, supposing a primary thyroid tumor.

Gender dysphoria and XX congenital adrenal hyperplasia: how frequent is it? Is male-sex rearing a good idea?

INTRODUCTION: The frequency of gender dysphoria (GD) among 46,XX congenital adrenal hyperplasia (CAH) patients is unknown. These data are needed to put into perspective the debate about the timing of reconstructive surgery and possible male-raising of the most severely virilized children. OBJECTIVE: To analyze the frequency of female to male GD between 46,XX individuals raised as females; to identify subgroups with higher chances of showing GD; to describe the results of male-raising among 46,XX CAH patients. MATERIALS AND METHODS: Analytic/descriptive literature review (January 1988 to April 2018). RESULTS: Female-raised patients frequently report the desire to be male, adopt male-typical behavior and are frequently homosexual/bisexual as adults, but this does not correspond to GD. Declared GD among 46,XX CAH patients attained 9% of the reported cohorts, generally in late adolescence/adulthood. We could not prove a relationship between inadequate treatment, null-genotype, late diagnoses, a higher degree of virilization, type of CAH or higher levels of androgens and female to male GD, but this may be due to statistical limitations. Male gender raised patients (MGR) were 10.1% of CAH cohorts included in this review, mostly from underdeveloped countries, with a high proportion of late diagnoses (76.3%) and familial choices. GD was more common in this group than among female-raised patients. Opting for male gender relates to a short final height, the need for multiple surgeries, surgical castration before puberty and infertility. CONCLUSION: Both male to female and female to male GD may present in 46,XX CAH patients in a contemporaneous cohort. The proportion of GD is higher among patients raised as males. DSD patients sexual maladjustments are complex and not comparable to the transgender population. Many 46,XX CAH patients with GD define themselves as gender-fluid and do not seek for legal/formal transition. Male-raising Prader 4/5 46,XX CAH patients imply infertility and multiple surgeries. There is no proof that any subgroup of CAH is more prone to GD, despite null genotypes, salt wasting phenotypes and Prader 4/5 cases being related to male-typical behavior and female homosexuality. TYPE OF STUDY: Descriptive/analytic non-systematic REVIEW. LEVEL OF EVIDENCE: 3.

Negative pressure wound therapy in pediatric surgery: How and when to use.

INTRODUCTION: Negative pressure wound therapy (NPWT) has been widely adopted to treat laparostomy, abdominal compartment syndrome (ACS) and complicated wounds associated with tissue loss. The method presents specific aspects, advantages and indications in Pediatrics. Our aim is to review the evidence available about NPWT in children. METHODS: Active search for papers about NPWT in Pediatric patients. Papers referring to orthopedic problems, wound complications after Cardiac Surgery or burns were excluded. RESULTS: The method shows good results to treat ACS, complicated wounds and abdominal wall malformations in neonates, including prematures. Periwound skin protection, monitoring of fluid losses and fine tuning of negative pressure levels according to age are necessary. Less pain, quicker recovery, less frequent dressing changes, possible recovery of exposed surgical hardware, granulation and shrinkage of the wound are advantages of the method over other kinds of dressing. NPWT is contraindicated over blood vessels and exposed nerves. Debridement is needed before usage over necrotic areas. Enteric fistulae are not contraindications. Complications are rare, mainly foam retention and dermatitis/skin maceration. The possibility of fistulae being caused by NPWT remains debatable. CONCLUSION: NPWT is widely used in Pediatrics, including neonates and premature, but the evidence available about the method is scarce and low quality. Complications are uncommon and mostly manageable. A possible causal relationship between NPWY and enteric fistula remains unclear. Adult devices and parameters have been adapted to children's use. Extra care is needed to protect the delicate tissues of Pediatric patients. Comparative research to define differential costs, indications and advantages of the method, specific indications and limits of NWTP in Pediatrics is needed. TYPE OF STUDY: Review. EVIDENCE LEVEL: IV.

Transperineal Ultrasound as a Tool to Plan Surgical Strategies in Pediatric Urology: Back to the Future?

OBJECTIVE: To demonstrate the usefulness and advantages of transperineal ultrasound (TPUS) on planning the surgical tactics to treat childhood pelviperineal disease (CPPD). METHODS: A cohort of CPPD is reviewed to provide a pictorial review of TPUS as imaging method variety of CPPD. Other imaging methods are compared with TPUS. RESULTS: TPUS studies of patients showing different conditions on the spectrum of pelviperineal malformation are shown in detail (pictorial review, graphically shown-see figures in the article and as supplementary material), highlighting the advantages of the method and comparing TPUS findings with other imaging techniques. CONCLUSION: Magnetic resonance imaging, contrast genitograms, voiding cystourethrography, and genital or urologic endoscopy have some important disadvantages, especially radiation exposure, high cost, not easily available equipment, and the need of general anesthesia or deep sedation in children. TPUS is easily available, including in impoverished environments, portable, painless, reproducible, inexpensive, and capable of providing detailed and specific information about pelviperineal malformation with accuracy. Data provided by TPUS are comparable with other imaging techniques (Table 1). Its main disadvantage is the dependency on the expertise of the operator to obtain high-quality, well-interpreted images.