Manuel G. Tellado

Coronal suturectomy through minimal incisions and distraction osteogenesis are enough without other craniotomies for the treatment of plagiocephaly due to coronal synostosis.

In the last few years, many surgeons have tried to reduce the damage produced during surgical approaches by trying to apply the principles of minimal invasive surgical techniques in every type of surgery. Some endoscopic techniques, added to orthopedic mechanisms, allow us to reduce the size of the incisions needed for the craniosynostosis surgery. We present a conservative surgical option in the treatment of one frequent craniofacial malformation due to synostosis: plagiocephaly due to coronal synostosis. We have operated on 10 patients with unilateral coronal synostosis. In all cases, a unique craniotomy, just in the coronal suture, was made without other accessory craniotomies. Mobilization of the frontal bone was not done at time of surgery, but it was done slowly with a distractor device later. All the patients were younger than 14 months, and the minimum follow-up has been 1 year after the operative course, and in all the patients, the anthropometric results were satisfactory both for the families and the medical team. The treatment of coronal synostosis can be made with both a simple coronal suturectomy, using small incisions under endoscopic control, and the application of a unidirectional bone distraction system. An orthopedic helmet can help to gain better results for reshaping a patients cranial vault, during the last part of the postoperative period.

Predictive value of clinical factors for successful endoscopic correction of primary vesicoureteral reflux grades III–IV

OBJECTIVE To evaluate the clinical factors that influence the outcome of endoscopically treated primary grade III and IV vesicoureteral reflux (VUR). MATERIALS AND METHODS Excluding secondary VUR and patients with incomplete follow up, a retrospective and prospective cohort observational study was performed on 90 children (130 renal units) with primary grade III or IV reflux treated endoscopically with Polytef or Macroplastique from March 1997 to January 2004. All patients underwent voiding cystourethrography (or galactose-based sonocystourethrography), dimercaptosuccinic acid (DMSA) scan, renal ultrasound and clinical exploration of micturition. Data were collected on age, gender, urinary tract infection (UTI), bilaterality, renal scarring, voiding dysfunction, and success rate. A new factor was defined, ureteral dilatation of the renal unit (not related to grade according to findings of pelvi-calyceal images), and graded by a single pediatric urologist blinded to its International Classification grade. RESULTS The male-to-female ratio of patients was 2.91. Reflux was bilateral in 44.4%. There was UTI at presentation in 64 cases, and 21 newborns had been prenatally diagnosed (ratio M/F 5.2:1). We found a strong relation between the incidence of renal scarring at presentation and voiding dysfunction (P<0.01). Cure rate at first injection was 64%. While univariate analysis appeared to show a strong association between resolution at first injection and bilaterality, voiding dysfunction, nephropathy and ureteral dilatation grade, with simultaneous logistic regression, the only statistically significant predictor was the presence of high-grade dilatation vs low ureteral dilatation (P<0.001; OR 0.045; 95% CI 0.01-0.16). Progression of renal damage was noted especially in patients with voiding dysfunction (P=0.053), bilaterality (P=0.034), and severe ureteral dilatation (P<0.001). CONCLUSIONS The relationship between success rate of endoscopic treatment and grade of ureteral dilatation is demonstrated. Other factors significantly involved in the resolution of VUR are voiding dysfunctional syndrome, bilateralism of reflux, and scarring on initial DMSA. Gender, age, number of infections during follow up and prenatal diagnosis do not influence the outcome of endoscopic treatment.

Kaposiform Hemangioendothelioma Associated With Milroy's Disease (Primary Hereditary Lymphedema)

Kaposiform infantile hemangioendothelioma (KHE) is a rare recently characterized, locally aggressive, endothelial-derived neoplasm that occurs exclusively in the pediatric age group. Milroy-Nonne disease (primary hereditary lymphedema) is an uncommon congenital entity with familiar history of lower limb edema as typical clinical features. An 8-year-old boy developed a hard painless mass in the right leg 7 years after the diagnosis of congenital primary lymphedema of the right lower extremity. Histopathological analysis of the tumor showed the typical findings of the KHE. To our knowledge this is the first reported case of a KHE engrafting on this infrequent benign lymphatic anomaly.

Extreme penoscrotal transposition

enoscrotal transposition (PST) is an extremely rare congenital malformation, usually associated with a wide variety of other anomalies. We describe an exceptional case of a newborn with extreme PST and severe hypospadias but normal upper urinary tract and no other associated malformations.A newborn male delivered at term with no reported antenatal complications. Three routine obstetrical sono-graphy showed normal fetal development. There was no evidence of mother being exposed to teratogens during pregnancy. On physical examination, an abnormal appearance of the external genitalia could be seen. There was a complete rotation of the external genitalia with a normal scrotum and testis but a rudimentary penis with a severe proximal hypospadias and chordee just attached anterior to the anal margin. Urinalysis, renal and vesical sonography and voiding cystourethrography showed no other urological abnormalities. There were no associated cardiological or gastrointestinal anomalies.Complete PST is a very uncommon heterogeneous condition in which the scrotum is positioned superior and anterior to the penis. It was fi rst recognized in 1923 by Appleby in an adult patient.

Prenatal diagnosis of fetal bowel obstruction complicated by in utero acute intestinal perforation.

A 33 year old primigravid woman attended the Fetal Unit of our hospital at 21 weeks of gestation for prenatal examination. An ultrasound scan showed a single anechoic cystic mass measuring 3 cm in diameter (3 3 cm) in the abdomen of the fetus, with no dilatation of other parts of the intestine. At 25 weeks, an ultrasound scan showed distended bowel loops with decrease in size of the fluidfilled lesion seen on the first scan. At 32 weeks of gestation, the cystic mass could not be seen but the bowel distension had increased. Magnetic resonance imaging showed dilated fluid-filled intestinal loops (Fig. 1) and the site of intestinal atresia. The colon and the small intestine beyond the atresia were normal. At 35 weeks of gestation, an ultrasound scan showed a decrease in size of the dilated loops, moderate fetal ascites, absence of peristalsis and echogenic material outside the bowel. All these findings suggested intrauterine intestinal perforation. An elective caesarean section was performed at 35 weeks of gestation. A baby boy was born weighing 2800 g. He had abdominal distension and moderate scrotal swelling. The infant underwent a transverse laparotomy immediately after delivery. An ileal perforation was seen proximal to an atretic segment of bowel (ileal atresia type II) (Fig. 2). Meconium peritonitis was confirmed. The bowel walls showed mild inflammation without ischaemic changes consistent with a recent intestinal perforation. A primary ileostomy was performed with resection of 5 cm of distended ileum. His post-operative course was uneventful and intestinal continuity was restored by end-to-end anastomosis 14 days after the first surgical procedure. The baby started oral feeds seven days after the second operation. During this period, he received total parenteral nutrition. The boy is now 15 months old and has no problems.

Reflujo vesicoureteral grados III-IV: factores implicados en la efectividad del tratamiento endoscópico en pacientes pediátricos

Resumen es: Objetivo: El Reflujo Vesicoureteral (RVU) es una de las anomalias congenitas urologicas mas frecuentes y se detecta hasta en el 30-50% de los pacientes p...