Elke Ruttenstock

Pediatric Ovarian Tumors - Dilemmas in Diagnosis and Management

BACKGROUND Ovarian tumors are rare in the pediatric age group and thus diagnostic and treatment strategies are heterogeneous. This study aims to evaluate ovarian tumors with a focus on age at presentation, imaging characteristics, diagnostic strategy, tumor presentation and management. METHODS Data was collected retrospectively from patients admitted between 1991 and 2008 for the evaluation and therapy of ovarian tumors. RESULTS Twenty-five patients were identified with neoplastic ovarian lesions (mean age 10.7 years). Sixteen patients (64%) underwent surgery for benign and 9 (36%) for malignant tumors. Benign tumors (n=16) had a mean diameter of 10.7 cm and mean age at presentation was 9.6 years compared to a diameter of 18.6 cm and 12.3 years in the malignant group (n=9). Elevated tumor markers were observed in 3 (12.5%) benign tumors and in 7 (77.8%) malignant tumors. In preoperative ultrasound investigation, cyst formation was identified in 4 benign tumors and solid tumor mass in 2 malignant tumors. A minimally invasive surgical approach was chosen in two patients, while open surgery was opted for in the rest. CONCLUSION Cyst formation, small tumor size and younger age at presentation were characteristic of benign tumors. Malignant tumors often presented with elevated tumor markers, a larger size and a solid consistency. Diagnostic dilemmas remain for both tumor groups due to the different tumor types and the heterogeneity of presentation.

Testicular torsion: a 15-year single-centre clinical and histological analysis

Aim:  This study reviewed the demographic, epidemiological and clinical factors of boys seen at a single centre who underwent surgical exploration for testicular torsion.

Closure of Bronchopleural Fistula With Porcine Dermal Collagen and Fibrin Glue in an Infant

The management of an 11-month-old infant who developed a bronchopleural fistula (BPF) 3 weeks after video-assisted thoracic surgery for congenital cystic adenomatoid malformation of the right lower pulmonary lobe is presented. Being refractory to treatment with chest tubes, the BPF was managed using a bronchoscopic approach using porcine dermal collagen (PDC) combined with a fibrin glue plug. The single session was sufficient to manage the BPF and the postoperative course was uneventful. This case highlights the novelty in the successful management of BPF in infants after pulmonary surgery using PDC and fibrin glue using the minimal access bronchoscopic approach.

Antegrade nailing can prevent cubitus varus and valgus after pediatric supracondylar fractures with impacted columns.

Summary: Supracondylar fractures are commonly encountered in the pediatric population. An impacted radial or ulnar column may result in the deviation of the elbow axis in the frontal plane clinically seen as cubitus varus or valgus. Antegrade nailing has become a feasible alternative to treat supracondylar fractures. It is not known whether it can prevent the development of cubitus varus or valgus in the cases of fractures with impacted columns. Between 1994 and 2009, 264 supracondylar humeral fractures were treated. Nineteen patients presented with either an impacted ulnar (n = 12) or radial (n = 7) column and were included in the study. The average elbow angle in fractures with impacted radial column was 159° mean (range 153–167°) compared with 179° (range 173–184°) in fractures with an impacted ulnar column. After closed reduction and antegrade nailing, the elbow angle was restored to normal values. At follow-up examination after a mean of 3.8 years (range 2.4–7.6 years), no further changes of the humeral–ulnar angle were seen. We are able to show that antegrade nailing is a safe method to treat supracondylar humeral fractures with impacted columns.

Pre- and postoperative rectal manometric assessment of patients with anorectal malformations: Should we preserve the fistula?

BACKGROUND: Surgical correction of congenital anorectal malformations could be complicated by fecal incontinence. Some authors believe that preservation of the fistula is associated with improved outcome. Rectal manometry is a well-established method to evaluate postoperative functional outcome in these patients and can demonstrate successful transplantation of the fistula. OBJECTIVE: Herein, we report the results of our series of patients with anorectal malformations and an externally accessible fistula, who underwent pre- and postoperative rectal manometry studies. DESIGN: This is a prospective cohort study. SETTINGS: This study was conducted at a tertiary neonatal and pediatric surgical center. PATIENTS: Patients with anorectal malformations, who underwent preoperative rectal manometry of the fistula and postoperative rectal manometry of the neoanus between January 2002 and December 2011 were included. MAIN OUTCOME MEASURES: Pre- and postoperative rectal manometry results were compared by using paired t test or contingency tables (p values <0.05). RESULTS: Twelve female patients with rectoperineal (n = 7, 58%) or rectovestibular (n = 5, 42%) fistula were treated by anterior sagittal anorectoplasty or minimal posterior sagittal anorectoplasty. Complete transposition of the fistula was achieved in all patients. Normal presence of rectoanal inhibitory reflex was demonstrated in all pre- and postoperative rectal manometry studies. There were no differences between pre- and postoperative rectal manometry in the length of the high-pressure zone (2.3 ± 0.6 cm vs 2.5 ± 0.8cm (p = 0.5)) and resting pressure (59.4 ± 18.2 mm Hg vs 62.1 ± 19.2 mm Hg (p = 0.62)). At a median follow-up of 665 days (range, 290–1165 days), all patients have voluntary bowel movements, with no incontinence or soiling. LIMITATIONS: This study is limited by its small sample size and by single-institution bias. CONCLUSION: Preoperative rectal manometry of rectoperineal or rectovestibular fistula showed the presence of functional anal structures within the fistula in all patients. We speculate that fistula-preserving surgery in patients with anorectal malformations is associated with improved bowel function outcome.

Detection of immediate post‐operative abdominal compartment after congenital diaphragmatic hernia closure

In newborns with congenital diaphragmatic hernia (CDH), reduction in the hernia contents and its closure can be associated with an elevation of intra-abdominal pressure (IAP). In some cases, abdominal compartment syndrome (ACS) with its deleterious effects can emerge after reduction in extensively herniated abdominal viscera into an underdeveloped abdominal cavity. Especially, the coexistence of lung hypoplasia or cardiac anomalies in CDH offers challenges in the diagnosis of ACS immediately after diaphragmatic closure. We report the successful early detection of ACS after CDH closure in an emergency setting without the application of conventional measuring techniques. A neonate with post-natal diagnosis of right-sided CDH was delivered in the 38 GW with 2450-g BW. Immediately after birth, the newborn presented with cyanosis and required emergency ventilatory support. Echocardiography demonstrated a left pulmonary artery stenosis and a persistent ductus arteriosus (PDA) with a left to right shunt. After cardiopulmonary stabilization, right-sided thoracotomy was performed on the third day of life. The herniated viscera that included large segments of the liver, gall bladder, small and large intestines were reduced into the abdominal cavity, and a Goretex (WL Gore Medical, Flagstaff, AZ) patch with a 3 cm diameter was employed for diaphragmatic closure. On completion of the procedure, difficulties in ventilation were encountered along with an extensive metabolic acidosis (pH = 7.28, BE = 14.0) for which an echocardiography was performed to rule out a contributing cardiopulmonary pathology. Serum lactate was elevated to 4.5 mmol/L (0.55–2.2 mmol/L). Hepatic hypoperfusion was evident on abdominal Doppler ultrasound, which along with deviation in laboratory parameters pointed to the presence of an ACS. An emergency decompression midline laparotomy was therefore performed, and a Goretex patch of 3 9 4 cm diameter was used for tension-free temporary abdominal wall closure. The postoperative course was uneventful, and after a period of 3 weeks, the patch was removed and abdominal wall closure was performed. According to the World Society of ACS criteria, ACS is defined as a sustained IAP >20 mm Hg (with or without an abdominal perfusion pressure of 60 mmHg) that is associated with new organ dysfunction or failure. Intra-abdominal hypertension levels are defined as Grade 1: IAP 12– 15 mmHg; Grade 2: IAP 16–20 mmHg; Grade 3: IAP 21– 25 mmHg; and Grade 4: IAP >25 mmHg (1). With increasing repair of congenital malformations with visceral herniations, ACS has received wider attention and recognition in newborns. These conditions along with CDH are associated with insufficient space in the abdominal cavity leading to an elevation of IAP when reduction in viscera is performed. However, gradual development of ACS after CDH closure has been only reported in one adolescent patient in whom ACS detection methods included urine output and urine catheter manometry for intravesical pressure (IVP) measurement (2). Other techniques such as intragastric pressure monitoring by manometry, tonometry or pH studies have also been described for ACS detection (3). Nevertheless, these techniques requiredelicate equipment andexperience in interpretationof their resultsandare thereforeseverely limited in their implementation in neonates for a rapidly developing ACS. Respiratory monitoring has also been employed in the detection of ACS with a recent report indicating plateau pressures (PRP) (PRP represents the static end-respiratory elastic recoil pressure of the total respiratory system) to be a reliable predictor of ACS in neonates after gastroschisis closure (4). However, these measurements are unsuitable for newborns with CDH because this group of patients

Pediatric bilateral Morgagni-Larrey diaphragmatic hernia: Is diagnosis with computed tomography required in the era of laparoscopic approach?

The foramen of Morgagni is a small anterior medial subcostosternal defect that extends from the sternum medially to the eight costal cartilages laterally. Morgagni-Larrey’s hernia is considered to be rare in the pediatric age group and has been estimated to account for 1–6% of all types of congenital diaphragmatic hernias [1]. Morgagni-Larrey’s hernia occurs more often on the right side (90%) and is bilateral in 7% cases. The decreased incidence on the left side has been attributed to the reinforcement of the diaphragm by the pericardium. During childhood, these patients are usually asymptomatic or present with repeated bouts of chest infection, or vague gastrointestinal presentations, which can be overlooked if not properly investigated. It is also well known that these hernias can be associated with other anomalies and its link with Down’s syndrome is well established [2]. Once the presence of a Morgagni-Larrey’s hernia is conWrmed on chest Wlms (Fig. 1), various diagnostic and surgical options available should be considered. The laparoscopic approach seems to be the most appropriate since it enables both (a) diagnosis: in the determination of the presence of bilateral hernias and (b) surgical management: repair of the defect (Fig. 2). After suspicion of Morgagni-Larrey’s hernia on chest Wlms, computed tomography has emerged to be the second line of investigation for conWrmation of these hernias and to determine the presence or to rule out bilateral hernias [3]. During the past decades, the use of computed tomography to diagnose conditions in the pediatric setting has increased [4]. Infants and children often require procedural sedation to maintain a motionless state to ensure highquality imaging. Various medication regimens have been recommended to achieve satisfactory sedation for this painless procedure. While the incidence of adverse events is low, procedural sedation carries the risk of serious morbidity and mortality. The use of evidence-based, structured approaches to procedural sedation should be used to reduce