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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.

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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.

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INTRODUCTION: Concealed penis (CP) may vary in severity and includes megaprepuce (MP) as a variant. Many different surgical strategies have been described in order to maximize penile exposure and to deal with skin deficiency. We describe the strategies that we use to overcome technical problems in severe cases of CP. MATERIALS AND METHODS: Six consecutive cases of severe CP (including 3 with MP) were treated in a 2-year period between January 2011 and April 2013. These patients were treated using extensive degloving, removal of dysplastic dartos, Alexander's preputial flap, scrotal flaps and skin grafts. Three patients had been previously circumcised. Cases associated with hypospadias, obesity, disorders of sexual differentiation and micropenises were excluded. RESULTS: All six patients attained good results, with good exposure of the penis, ability to void standing with a well-directed flow and reasonable esthetic results. A technical algorithm for the treatment of primary or recurring cases of CP is proposed. CONCLUSION: Alexander' s distally based ventral preputial flap is a useful technical resource to treat MP cases. Free skin grafts and/or laterally based scrotal flaps may be used to cover the penis after release in severe cases of CP.

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Epidermolysis bullosa causes serious blistering of the skin even with minor trauma. The use of standard electrodes, tapes, and adhesives is restricted in these patients because it exposes the skin to stretching and shearing forces that cause traumatic blister formation. We propose herein an alternative electrocardiographic monitoring technique to minimize cutaneous trauma.

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OBJECTIVE: To describe an adolescent with pathologic aerophagia, a rare condition caused by excessive and inappropriate swallowing of air and to review its treatment and differential diagnoses. CASE DESCRIPTION: An 11 year-old mentally impaired blind girl presenting serious behavior problems and severe developmental delay with abdominal distension from the last 8 months. Her past history included a Nissen fundoplication. Abdominal CT and abdominal radiographs showed diffuse gas distension of the small bowel and colon. Hirschsprung's disease was excluded. The distention was minimal at the moment the child awoke and maximal at evening, and persisted after control of constipation. Audible repetitive and frequent movements of air swallowing were observed. The diagnosis of pathologic aerophagia associated to obsessive compulsive disorder and developmental delay was made, but pharmacological treatment was unsuccessful. The patient was submitted to an endoscopic gastrostomy, permanently opened and elevated relative to the stomach. The distention was resolved, while maintaining oral nutrition COMMENTS: Pathologic aerophagia is a rare self-limiting condition in normal children exposed to high levels of stress and may be a persisting problem in children with psychiatric or neurologic disease. In this last group, the disease may cause serious complications. Pharmacological and behavioral treatments are ill-defined. Severe cases may demand surgical strategies, mainly decompressive gastrostomy.

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Transperineal exposure of the high portion of the vagina is limited. These limitations can be circumvented using ASTRA (anterior sagittal transrectal approach). We report the use of this surgical strategy for the treatment of a case of acquired vaginal atresia after posterior anorectoplasty due to anorectal malformation.

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Stevens-Johnson syndrome frequently affects the genitalia. Vaginal problems have been recognized in female patients; however, reports about the problem and its treatment are rare. Labial sinechiae have not yet been reported as sequelae of Stevens-Johnson syndrome. Amenorrhea, cyclical abdominal pain, and a hypogastric mass in girls affected by Stevens-Johnson syndrome could indicate acquired vaginal obstruction. Extensive labial sinechiae in such patients can cause dysuria, urinary tract infection, and sexual dysfunction. After a diagnosis of Stevens-Johnson syndrome in girls, it is prudent to schedule a prepubertal genital examination to diagnose genital disease preemptively and avoid obstructed menstruation and future sexual problems.

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OBJECTIVE: This report presents a case of femoral osteomyelitis secondary to urinary tract infection in a female neonate with bilateral urinary duplication and right ectopic ureterocele. METHODS: A female neonate with a history of late perinatal sepsis presented to the emergency department and was found to have left distal femoral osteomyelitis. A complex urological malformation was diagnosed (bilateral renal duplication with right ectopic ureterocele associated with nonfunctional superior pole of the ipsilateral kidney). The baby was submitted to prolonged antibiotic therapy and upper pole nephrectomy of the right kidney. RESULTS: The pathology report confirmed chronic pyelonephritis and dysplasia in the resected specimen. After 1.5 years the patient is asymptomatic and developing normally, with no apparent deformity, but manifests asymptomatic vesicoureteral reflux. CONCLUSIONS: Urinary tract infections must always be excluded as the primary infection focus in neonates with sepsis. Perinatal osteomyelitis is almost always a complication of neonatal sepsis and is associated with orthopedic sequelae. This is the first report in literature of a case of osteomyelitis complicating complex urinary duplication and urinary tract infection.

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OBJECTIVE: To define the distribution of Pediatric Surgeons in Brazil and the distribution of job offered for specialists in the country. To compare the professional profile of Brazilian Pediatric Surgeons with other countries. METHODS: Statistical data derived from Brazilian Institute of Geography and Statistics (IBGE), Brazilian Medical Council (CFM) and Brazilian Society of Pediatric Surgery (CIPE) were used to define the professional profile of pediatric surgeons in Brazil. RESULTS: Eight-hundred-and-five pediatric surgeons are needed in Brazil only to treat neonates and toddlers. Professionals are excessively concentrated in South and Southeast and insufficient numbers are found in North and Northeast. Data about the number of pediatric surgeons working in Brazil are conflicting: data obtained from FIOCRUZ differ from those from CFM and CIPE. The working routine of Brazilian pediatric surgeons is different from that of North-American and European specialists, from where the major part of our references is extracted. CONCLUSION: Brazil needs a minimum of 805 pediatric surgeons working full time only to treat nenonates and toddlers. Specialists are not well distributed throughout the country. The southeast is a training center and exports specialists to the rest of the country. Epidemiological data concerning the distribution of professionals in Brazil are conflicting.

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The authors present a case of intestinal tuberculosis affecting exclusively the left colon causing severe undernourishment, abdominal pain, and bowel obstruction with a sealed colonic fistula in a 10-year-old child. These clinical characteristics and difficulties led to a diagnosis of intestinal tuberculosis in childhood. Intestinal tuberculosis affecting exclusively the colon is very rare, and differential diagnosis with Crohn's disease is difficult. Surgical complications are frequent, especially intestinal obstruction, and can be treated in most cases by resection of the affected segment and primary anastomosis.

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OBJECTIVE: To study clinical and radiological data and surgical treatment for symptomatic ureteral duplications in childhood, especially the results for superior polar heminephrectomies. METHODS: Prospective observational study concerning 15 pediatric patients surgically treated following a protocol with a minimum follow-up of 6 months. RESULTS: 93% of the patients presented urinary infection and/or fetal hydronephrosis during their first year of life. These were the usual reasons for evaluation. Superior polar heminephrectomy was indicated for 80% of the patients, all of whom presented clinical and radiological improvement after the surgery. There were no significant complications. All children with severe vesicoureteral reflux to the duplicated superior pole presented symptomatic reflux to the stump after the surgery and were submitted to stumpectomy. None of the patients without preoperative reflux needed any other surgical procedure. CONCLUSION: Superior polar heminephrectomy is safe and efficient in order to treat ureteral duplications in childhood. If the patient does not present vesicoureteral reflux this will probably be the only surgery needed. Most patients with reflux to the resected superior pole will need stumpectomy subsequently.

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