Sparing-lung surgery for the treatment of congenital lung malformations.

PURPOSE: Congenital lung malformations (CLM) are traditionally treated by pulmonary lobectomy. The foremost indication for surgery is that these lesions predispose to respiratory tract infections and to malignancy. Owing to the improvement of prenatal diagnosis, most patients are operated in a timely manner and prophylactically. In this context, lung-sparing surgery (LS) has gained interest as a lung preservation strategy, especially for asymptomatic patients. In the present study, we evaluated both thoracoscopic and open lung-preserving resections as an alternative to lobectomy for CLM resection. METHODS: We retrospectively reviewed all patients who underwent lung-sparing resection (segmentectomy and atypical resection) for CLM from 2001 to 2010. Data were collected regarding preoperative diagnostic workup, type of intervention, and follow up. RESULTS: Fifty-four patients received LS for CLM during the study period. Twenty-six were approached thoracoscopically, with 18 cases requiring open conversion for a complete resection. There were six postoperative complications: three asymptomatic pneumothoraces that resolved without intervention, one tension pneumothorax that required replacement of a drainage catheter, and three instances of intra-operative bleeding requiring blood transfusion. Mean duration of follow-up was 65.2 months. Two patients experienced pneumonia during the follow-up period. A third patient had a cystic lung lesion on postoperative computed tomography (CT) which required a second-look surgery. CONCLUSIONS: LS for CLM is a safe and effective means of lung parenchymal preservation in pediatric patients. Complication rates are comparable to that of traditional lobectomy. In our experience, this type of lung surgery does not carry a higher risk of residual disease and recurrence if accurately planned in selected patients, i.e., those with small asymptomatic lesions. The complication rate is acceptable and apparently not affected by preoperative symptoms. The thoracoscopic approach is recommended, although open conversion should be advocated to avoid too long operative times.

A Rare Case of Chylothorax due to Pulmonary Lymphangiectasia in a 7-Year-Old Boy.

Pulmonary lymphangiectasia (PL) is a rare condition characterized by dilatation of the lymphatic vessels. Post-neonatal PL is usually associated with pleural effusion and should therefore be suspected in the presence of chylothorax. We describe a post-neonatal manifestation of PL in a 7-year-old boy presenting chylothorax. Radiological examinations included thorax X-ray, ultrasound, and computed tomography scans. After the failure of conservative management (maintenance of the chest tube, total parenteral nutrition, administration of somatostatin synthetic analogues) we performed a thoracoscopic massive ligation of the thoracic duct's collateral along with a lung biopsy. Histology was compatible with type 1 congenital pulmonary lymphangectasia. One month after surgery a thoracoscopic pleurodesis was required for persistent chylothorax. The boy is now doing well 1 year after surgery.

One-trocar-assisted nephrectomy (OTAN): initial experience and codification of a technique.

BACKGROUND: Laparoscopic nephrectomy in children has gradually become a reasonable alternative to open nephrectomy and, besides, a retroperitoneal approach seems more logical than transperitoneal approach to perform nephroureterectomy for benign disease, as in open surgery. To further reduce the access-related complications, we propose a retroperitoneal one trocar-assisted nephrectomy (OTAN). We report our experience with minimally invasive OTAN for the treatment of benign renal disease. METHODS: A total of 27 OTANs were performed at our institution between 2003 and 2009. The median patient age was 7.6 (range, 2-32 months). Indications for unilateral nephrectomy were multicystic dysplastic kidney (MCDK) in 23 cases (85.2%), dysplastic kidney in 3 cases (11.1%), and reflux nephropathy in 1 case (3.7%). RESULTS: The median operative time from the initial incision to skin closure was 60 min. Perioperative transfusion was not required in any cases. No major perioperative complications developed. Conversion to open surgery was necessary in four cases (14.8%): in three cases for a small working space arising from a peritoneal perforation, and in one case for a difficult visualization of the parenchyma (renal fusion not detected by preoperative ultrasound evaluation). Most patients were allowed oral intake on postoperative day 1. The median hospital stay was 2 (range, 2-3) days; the cosmetic results were excellent. Convalescence was uneventful in all patients. CONCLUSIONS: Although the indications for a nephrectomy in case of benign disease remain limited, when a little child has small, poorly functioning kidneys that must be removed, a one trocar-assisted approach for nephrectomy is usefully a safe and effective treatment choice. The procedure can be easily performed through a small incision with minimal morbidity, comparable operative time, and excellent cosmesis without excessive postoperative pain issues, allowing early discharge home.

Vaginal replacement in the pediatric age group: a 34-year experience of intestinal vaginoplasty in children and young girls.

BACKGROUND/PURPOSE: The absence of vagina is rare in the pediatric population. It can occur as a result of congenital malformations such as an aplasia of mullerian ducts (46,XX Mayer-Rokitansky-Küster-Hauser syndrome) or a complete androgen insensitivity syndrome (46,XY testicular feminizing syndrome). Intersex patients, who underwent reassessment of a female sex, need a genital reconstruction toward a feminine phenotype. Patients with congenital adrenogenital syndrome with high urogenital sinus could have a severe hypoplastic vagina. In all these cases, a vaginal replacement is required. We reviewed our experience of vaginal replacement using a sigmoid conduit. METHODS: In 34 years, we evaluated 47 patients. The observation period was from 1 to 34 years (mean: 12 years). The preoperative diagnosis was Mayer-Rokitansky-Küster-Hauser syndrome in 17 cases, androgen insensitivity syndrome in 24 cases, adrenogenital syndrome with high urogenital sinus in 5 and 1 patient was affected by penile agenesis. Forty-six patients were treated with vaginal reconstruction by interposition of sigmoid colon. Only in 1 case we performed a vaginal construction with an ileal loop: in this case, the sigmoid colon was extremely dilated by a chronic constipation secondary to a high anorectal malformation corrected at birth. RESULTS: The outcome for 47 patients is excellent: 18 are sexually active and 4 are married. Only 1 patient with adrenogenital syndrome died of endocrine problems. Complications occurred in 17 cases: in 1 patient a necrosis of the replaced vagina occurred, thus requiring vaginal exeresis; now she is waiting for a second operation. Another patient had an abdominal abscess, which was surgically treated. In 12 cases a second procedure was required: 6 had stenotic new-vaginal introitus, 4 had new-vaginal prolapse, and 2 had intestinal obstruction. CONCLUSIONS: We believe that the preferable technique for vaginal replacement is the use of intestinal conduit. The sigmoid colon is the best intestinal tract to be used owing to its size, location and preserved blood supply. Our experience leads us to believe that the sigmoid segment is the segment of choice, although we consider ileal loop as a good alternative when the sigmoid colon is not available.