Is It Better to Operate Congenital Lung Malformations when Patients are Still Asymptomatic?

CONTEXT: Congenital lung malformation (CLM) is a rare developmental anomaly of the lower respiratory tract. The purposes are to define if the presence of respiratory symptoms, in CLM may affect surgical outcomes and to define optimal timing for surgery in asymptomatic patients. SETTINGS AND DESIGN: Retrospective review of patients with CLM from 2004 to 2018. Asymptomatic and symptomatic patients were compared. Moreover, asymptomatic patients were stratified according to age at surgery (< or >6 months). SUBJECTS AND METHODS: Demographic data, prenatal diagnosis, symptoms, CLM's characteristics, operative and postoperative data were collected. Patients were divided into two groups based on the presence or none of respiratory symptoms. STATISTICAL ANALYSIS: Data were compared using the Fisher's exact test for qualitative values and Mann-Whitney test for quantitative values P < 0.05 was statistically significant. RESULTS: One hundred and eighty-six patients were treated. Asymptomatic were 137 (74%), while symptomatic were 49 (26%). The most common presenting symptoms were respiratory distress (n = 30, 61%) followed by pneumonia (n = 18, 38%). Prenatal diagnosis of CLM was performed in 98% of asymptomatic patients compared to 30% of symptomatic (P = 0.001). Surgical excision was performed in all cases, and in 50% by thoracoscopy, without difference between the two groups. In 97% of all cases, a lung sparing surgery was performed without difference between the groups. Symptoms are significantly associated with older age, location in the upper lobe, and lobar emphysema. Length of stay in intensive care, postoperative complications, and reintervention rate were higher in the symptomatic group. Eighty-one asymptomatic patients underwent surgery <6 months of life; they had a lower rate of surgical complications (2%) compared with those >6 months (7%). CONCLUSIONS: The present study describes a comprehensive picture of CLM. In addition, we emphasize the role of early postnatal management and thoracoscopic surgery, also before 6 months of life, to prevent the onset of symptoms that are associated with worse outcomes.

The APpendicitis PEdiatric (APPE) score: a new diagnostic tool in suspected pediatric acute appendicitis.

Our aim was to develop an APpendictis-PEdiatric score (APPE score) in quantifying risk of acute appendicitis based on combination of clinical and laboratory markers. 1025 patients were classified in: acute appendicitis (AA) and non-appendicitis. Demographic/clinical features, and laboratory were collected. They were compared for quantitative-variables and categorical-variables. Significant predictors (P=<0,05) were included in logistic regression model. Based on regression-coefficients, a diagnostic score was tested by calculating the area under the ROC curve. Two cut-offs were established to define classes of risk of AA. 9 variables were identified as potentially predictors for AA. Those underwent logistic regression and a score was assigned, for maximum 21-points. The score showed an area under the curve: 0.831 and a linear proportion with the state of appendicular inflammation (R20.85). Patients with a score ≤8 were at low risk of AA (sensitivity 94%); those with a score ≥15 were at high risk for AA (specificity 93%). Those between 8 and 15 were defined at intermediate risk class. APPE-score guides clinicians in classifying patients with suspected-AA according to clinical and laboratory findings in order to improve their management.

Laparoscopic-Assisted Anorectal Pull-Through for High Imperforate Anus: 14 Years Experience in a Single Center.

PURPOSE: To report our 14 years experience with the laparoscopic-assisted anorectal pull-through (LAARP) for the treatment of male neonates with high imperforate anus. MATERIALS AND METHODS: We reviewed all medical charts of patients who underwent LAARP from January 2001 to January 2015 collecting information regarding demographic data, associated anomalies, type of fistula, pelvic floor muscles tropism, surgery (operative time, conversion to open technique, and complications), and follow-up. Follow-up data were obtained through the use of validated questionnaires that aim to assess the intestinal function in terms of constipation and continence. RESULTS: At the Department of Pediatric Surgery of the University of Bologna, 13 male patients were operated in the study period (our protocol consisted of colostomy at birth followed by delayed LAARP). Mean age at operation was 4.75 months (range 1-14). There was one conversion to open technique due to a strong tension from the colostomy (this patient was excluded). Functional results were acceptable at a minimum 2-year-long follow-up. CONCLUSIONS: LAARP is a good choice for the correction of the high imperforate anus. Short-term outcomes are similar to those after posterior sagittal anorectoplasty (PSARP). Long-term outcomes should be better assessed.

Vaginal malformations: a proposed classification based on embryological, anatomical and clinical criteria and their surgical management (an analysis of 167 cases).

BACKGROUND: Although it is virtually impossible to formulate a scheme that can satisfactorily collect all different types of vaginal malformations, a simple classification would be of a considerable value and would permit logical operative decisions. Many classifications of anomalies of uterus and vagina have been proposed: we find them unsatisfactory and confusing, being either too simple or too complex. We propose a new classification, focused only on vagina and based on embryological, anatomical, clinical and surgical criteria. METHODS: In over 30 years, 167 females with vaginal malformations have been observed in our department. Intersex cases were excluded. The encountered anomalies have been divided into six types and ten subtypes: type I vaginal agenesis (IA associated with uterine agenesis/17 Pts, IB isolated/1 Pt); type II vaginal atresia (IIA proximal/1 Pt, IIB distal/4 Pts); type III vaginal atresia with urethrovaginal fistula-urogenital sinus (IIIA proximal fistula-high sinus/42 Pts, IIIB distal fistula-low sinus/55 Pts); type IV vaginal atresia with transverse septum (IVA transverse septum/6 Pts, IVB imperforate hymen/17 Pts); type V disorders of müllerian ducts fusion (VA vaginal duplication/4 Pts, VB longitudinal septum/4 Pts); type VI cloaca/16 Pts. Of each type and subgroup of malformation the appropriate surgical correction is reported. Types of malformation and surgical treatment are analysed: IA vaginal reconstruction using a sigmoid conduit, IB vaginal reconstruction using a sigmoid conduit, atresic cervical resection and uterus-new vagina anastomosis according to Schmid; II perineal vaginal pull-through; IIIA anterior sagittal transanorectal vaginal pull-through, IIIB perineal flap vaginoplasty; IVA excision with abdominovaginal approach, IVB hymen incision; VA tubularization, VB septectomy via perineal approach; VI posterior sagittal anorectal-vaginal-urethroplasty. RESULTS: Most of the patients had good aesthetic and functional results. Type III showed relatively more complications: four redo operations (IIIA), four revisions of the vaginoplasty (IIIB). One patient is still waiting for definitive surgical correction. CONCLUSIONS: An early diagnosis is desirable to correct adequately vaginal malformations, which becomes mostly evident around puberty. The better timing for surgery is early age, to obtain better results and to avoid many of the psychological problems that arise at a later age.

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.