Congenital atrichia and hypotrichosis

Background  

Alopecia present from birth includes a broad differential diagnosis and often represents a diagnostic and therapeutic challenge for the involved physician.     

High flow priapism: diagnosis and treatment in pediatric population

Purpose  

To present three cases of arterial high flow priapism (HFP) and propose a management algorithm for this condition.     

Laparoscopic management of impalpable testes: comparison of different techniques

Purpose  

Laparoscopy is an important modality for management of impalpable testes. We present long-term outcomes of intra-abdominal testes managed by either single stage orchidopexy or two-stage Fowler Stephen’s orchidopexy (FSO) over 12 years.     

Hemangioendothelioma of the right atrial appendage associated with pericardial effusion in an infant

Background  

Cardiac hemangioma is a rare cause for pericardial effusion. We present a case of hemangioendothelioma of the right atrial appendage associated with pericardial effusion in an infant.     

Single payer health insurance in pediatric surgery: US impressions and Canadian experience

Context  

Some advocate single payer national health insurance, present in Canada, as a solution to problems in US health care.     

Recurrent bacterial meningitis caused by an occult basilar skull fracture

Background  

We present a rare case of recurrent bacterial meningitis caused by an occult basilar skull fracture.     

Surgical complications after living donor liver transplantation in patients with biliary atresia: a relatively high incidence of portal vein complications

Background/purpose  

The aim of this study is to present the surgical complications in living donor liver transplantation (LDLT) for biliary atresia (BA) as a treatment for end stage liver disease.     

Minimally invasive partial or total nephrectomy in children: a comparison between transperitoneal and retroperitoneal approaches

Introduction and objectives  

We present data from a single surgeon experience of paediatric transperitoneal (TP) and retroperitoneal (RP) minimally invasive approaches for partial (PN) and total nephrectomy (TN).     

Causes of Child Deaths in India, 1985–2008: A Systematic Review of Literature

Objective  

To understand the causes of child deaths in order to implement appropriate child survival interventions in the country. We present a systematic review of studies reporting causes of child, infant, and neonatal deaths from India for 1985 to 2008.     

Recurrent rectal prolapse following primary surgical treatment

Purpose  

The management of recurrent rectal prolapse following initial surgical procedures remains unclear. We present a series of pediatric patients with rectal prolapse and describe their surgical management, and the subsequent care for those children developing recurrence.     

Use of a combined oxygen/nitrous oxide/morphine chlorydrate protocol for analgesia in burned children requiring painful local care

Purpose  

We present the results of the use of a protocol of inhalational oxygen/nitrous oxide mixtures associated with oral opioids on a prospective cohort of 33 children undergoing local care for acute but limited burned skin lesions.     

Glutamine prevents intestinal mucosal injury induced by cyclophosphamide in rats

Purpose  

High doses of anticancer drugs often damage the intestinal mucosa. The purpose of the present study was to examine the effect of glutamine on mucosal damage induced by cyclophosphamide in a rat model, and to elucidate the mechanisms responsible for its protective effects.     

Interventional occlusion of congenital vascular malformations

Background  

New materials and devices have been used in the management of cardiac malformations. In this paper, we present our experience with interventional occlusion of congenital vascular malformations.     

Image-defined risk factors in localized thoracic neuroblastoma and ganglioneuroma

Background

The pretreatment International Neuroblastoma Risk Group Staging System (INRGSS) discriminates localized tumors L1/L2 depending on the absence/presence of image-defined risk factors (IDRFs) at diagnosis. Referring to this new staging system, we assessed initial imaging of localized thoracic neuroblastoma (NB) and ganglioneuroma (GN) and the extent of initial tumor resection.

Methods

Patients with localized thoracic NB/GN from the German clinical trials NB97 and NB2004 were included. Imaging at diagnosis and operative reports were reviewed retrospectively. IDRFs were assessed centrally and correlated to International Neuroblastoma Staging System (INSS) stage and extent of tumor resection. Additionally, we analyzed data on surgery-related complications.

Results

Imaging series of 88 patients were available for central review. In 18 children, no IDRF was present, 28 exhibited one IDRF, 42 two or more IDRFs, resulting in 70 patients with L2 disease. The most frequently observed IDRF was encasement of any vessel (n = 38). Initial surgical resection was aimed for in 45 patients (L1: n = 11; L2: n = 34). Complete and gross total resection rates were higher children with L2 NB (n = 8/25 L1, n = 17/25 L2 vs. n = 2/15 L1, n = 13/15 L2, respectively). The proportion of surgical complications was very similar between INRGSS L1 and L2 (n = 4/11 vs. n = 17/34). All complications were manageable, and no surgery-related deaths were observed.

Conclusion

In this retrospective cohort, the extent of resection and the rate of surgical complications did not differ substantially between patients classified as L1/L2, indicating that INRGSS L2 does not equate unresectability. It appeared that individual IDRFs differ in value. Larger studies are needed to assess the significance and therapeutic/prognostic impact of such findings.     

Ischiopubic and odontoid synchondrosis in a boy with progressive pseudorheumatoid chondrodysplasia

Purpose  

To present the case of a 14-year-old boy with clinical and radiographic features of pseudorheumatoid chondrodyspalsia with additional, potentially serious, cervical malformations.     

Pseudopapillary tumor in pediatric age: clinical and surgical management

Background  

Solid pseudopapillary tumors of the pancreas are rare exocrine pancreatic tumors. Through a review of pediatric cases in a single Institution, we present the clinical and surgical management of this neoplasm.     

Lower urinary tract obstruction secondary to congenital bladder diverticula in infants

Purpose  

Congenital primary bladder diverticulum is a rare condition and may present with urinary infection; other forms of presentation are rare. We present a series of infants who presented with urinary retention secondary to large primary bladder diverticulum.     

Favourable long-term outcome after immediate treatment of neonatal hyperammonemia due to N-acetylglutamate synthase deficiency

Introduction  

N-Acetylglutamate synthase (NAGS) deficiency is a rare urea cycle disorder, which may present in the neonatal period with severe hyperammonemia and marked neurological impairment.     

Poor catch-up growth after proctocolectomy in pediatric patients with ulcerative colitis receiving prolonged steroid therapy

Purpose  

The aim of the present study was to review the complications and growth after proctocolectomy and ileal J-pouch anal anastomosis (IPAA) in pediatric ulcerative colitis (UC) patients receiving prolonged steroid therapy.     

Bowel injuries caused by ingestion of multiple magnets in children: a growing hazard

Purpose  

There are no definite guidelines on the management of ingested magnetic objects in children. The aim of this study was to present our experience and to highlight the importance of strict follow-up.