Three-dimensional anatomy of the pelvis in bladder exstrophy: description of bone pathology by using three-dimensional computed tomography and its clinical relevance.

The alterations in pelvic structure that occur in bladder exstrophy were evaluated in six children (one girl and five boys) by using three-dimensional computed tomography (3D-CT). The length and angle of anterior and posterior segments and the slanting angle of iliac wings were measured. 3D-CT scans of the pelvis of four patients with a normal pelvis were obtained to serve as controls. Both anterior and posterior segments were abnormally rotated externally in patients with bladder exstrophy. Whereas the length of the posterior segment is normal, the length of the anterior segment is significantly shorter, and the iliac wings projected more inward than those in the age-matched control subjects.

Unilateral inguinal ectopic scrotum and imperforate anus: a case report.

A case of an ectopic scrotum located in the right inguinal area is described. The left hemiscrotum was in normal location, and each hemiscrotum contained testis. The boy also had an infralevator-type anorectal malformation. Initially, anoplasty was performed, and scrotal reconstruction was carried out 1 month later. The authors also reviewed the embryological explanation of ectopic scrotum in the literature.

Postpneumonic empyema in children treated by early decortication.

We performed early decortication in 32 cases of postpneumonic empyema during the period of February 1991 to December 1995. Twenty of them were male and 12 of them were female. We evaluated these cases retrospectively. Decortication was saved for the patients where antimicrobial therapy and closed-tube drainage had failed to achieve a cure and was performed on the 10-15th day after the diagnosis was established. Indications for the decortication were persistent fever (9), pulmonary air leakage (7), localized effusion (7), persistent respiratory distress (5) and pleural thickening without resolution (28). Decortication was performed through the standard posterolateral thoracotomy. Patients were discharged on the 8th postoperative day with minimal morbidity and no mortality. Because of the simplicity of the procedure (short hospitalization periods, low mortality and morbidity rates) good results were achieved with early decortication.

A case of anterior sacral meningocele totally excised using the posterior sagittal approach.

Anterior sacral meningocele is a rare congenital anomaly. The authors present a case of anterior sacral meningocele successfully excised using the posterior sagittal approach. The diagnosis and treatment of this unusual anomaly are discussed.

Congenital lumbar hernia associated with the lumbocostovertebral syndrome: two cases.

Congenital hernias in the lumbar region are very uncommon. Two cases with congenital lumbar hernia associated with the lumbocostovertebral syndrome are presented. The first case of a 14-month-old girl presented with the type of a superior lumbar triangle hernia. The second case was a 2-year-old girl presenting with diffuse lumbar hernia. The anatomy, embryology and treatment of this congenital abnormality are discussed.

A pseudoexstrophy with penile anomaly.

Although classical exstrophy of the bladder is not a rare anomaly, variants of exstrophy are extremely uncommon. We report a male pseudoexstrophy case with appearance of a low set umbilicus associated with umbilical hernia and penile anomaly. Although this mild exstrophy variant does not normally require surgical management, penile reconstruction was necessary in our patient.

A case of ectopic anus localized on the right gluteus.

A case of a gluteus-localized ectopic anus on the right side is described. A 5-month-old boy had a severe sacral anomaly with atrophy of the right lower extremity and a pes equinus deformity. Transcutaneous muscle stimulation showed a well-developed muscle complex, and parasagittal muscle fibers have been localized obliquely. Although he has severe sacral anomalies, the posterior sagittal anorectoplasty procedure could be performed. This unique case provides a good demonstration of not only the ectopically localized anus but also the ectopically localized muscle complex.

A very rare type of multiple intestinal atresias: "a string of pearls".

Intestinal atresia is the most frequently encountered cause of ileus in the neonate. Of all atresias combination of small and large bowel atresias is extremely rare. In 1973, Guttman presented a case with multiple, widespread atresias of small and large bowel, intraluminal calcifications and a hereditary nature. This paper describes the detailed pathological findings of a similar case of multiple intestinal atresias and discusses for the pathogenesis of this rare condition.

Pelvic ganglioneuroblastoma totally excised using posterior sagittal and abdominal approach.

Neuroblastoma usually presents as an upper abdominal mass arising from the adrenal gland. Recent experience with neuroblastoma of the spermatic cord, bladder, and pelvis demonstrates the propensity of this tumor to arise in unusual areas. A 1-year-old boy was admitted with pollakiuria, acute urinary retention, and chronic constipation. Computed tomographic and ultrasonographic examinations revealed a 5×5.5×4-cm pelvic mass, which was totally excised using a posterior sagittal and abdominal approach. The pathologic diagnosis was ganglioneuroblastoma and 1-year follow-up was uneventful.

Lumbar teratoma with intraspinal extension.

Teratomas are childhood tumours especially notable for their diversity of anatomic locations and biological behaviour. We present an extremely rare occurrence of a lumbar teratoma with intraspinal extension. The importance of thorough preoperative evaluation and magnetic resonance imaging for tumours in this location is also emphasised.

Juvenile xanthogranuloma: three cases.

Juvenile xanthogranuloma (JX) is a regressing fibrous histiocytoma occurring during infancy and characterized by cutaneous papules and nodules and less often by additional lesions in deep tissue and organs. It has a special place among childhood masses by presenting different outcomes and spontaneous regression. We report three cases of JX that were detected after birth and resected.

Measurement of testicular volume by ultrasonography.

Twenty-five patients with scrotal hernia and hydrocele were studied. The testicular sizes of the patients were measured by using ultrasonography and the sliding caliper was used for calculations during surgery. The actual testicular volumes and those obtained by ultrasonography were not statistically different (p > 0.05). It is suggested that ultrasonography may be an alternative to orchidometers.

A case of Waardenburg syndrome and aganglionosis.

Waardenburg's syndrome is characterized by a broad nasal root, pigmentation disturbance and congenital deafness while aganglionosis is described as the partial or complete lack of ganglion cells in the alimentary tract. This report describes a five-day-old male infant with Waardenburg's syndrome associated with total aganglionosis of the colon, ileum and distal jejunum and draws attention to the causal relationship between these two entities.

Congenital bifid sternum with partial ectopia cordis.

A newborn infant with bifid sternum and partial ectopia cordis is reported and the pertinent literature is briefly reviewed. Its successful surgical management is described. Early repair is recommended.