Necrotizing Liver Granuloma/Abscess and Constrictive Aspergillosis Pericarditis with Central Nervous System Involvement: Different Remarkable Phenotypes in Different Chronic Granulomatous Disease Genotypes

Chronic granulomatous disease (CGD) is a primary immune deficiency causing predisposition to infections with specific microorganisms, Aspergillus species and Staphylococcus aureus being the most common ones. A 16-year-old boy with a mutation in CYBB gene coding gp91phox protein (X-linked disease) developed a liver abscess due to Staphylococcus aureus. In addition to medical therapy, surgical treatment was necessary for the management of the disease. A 30-month-old girl with an autosomal recessive form of chronic granulomatous disease (CYBA gene mutation affecting p22phox protein) had invasive aspergillosis causing pericarditis, pulmonary abscess, and central nervous system involvement. The devastating course of disease regardless of the mutation emphasizes the importance of early diagnosis and intervention of hematopoietic stem cell transplantation as soon as possible in children with CGD

General complications after surgery for anorectal malformations

WOS: 000516302200001PubMed: 32086570The introduction of posterior sagittal anorectoplasty (PSARP) by deVries and Pena in the early 80s has impacted to the treatment of patients with anorectal malformations (ARM). It gained great recognition worldwide in a very short time, and since then, surgeons dealing with the treatment of this complex malformation could achieve tremendous progress in contemporary management of this anomaly. Despite the growing experience and body of information globally, the treatment of ARMs continues to be a challenge to the pediatric surgeons due to the nature and the variability of the anomaly, and short- and long-term problems continue to exist even after nearly 40 years of the PSARP era. Today, knowing more about it, pediatric surgeons are committed to do more for their ARM patients to have them as physically and socially healthy individuals

Obstructive uropathies in children

WOS: 000277931100022The causes of urinary obstruction in chidhood is mostly congenital, and the diagnosis can usually be made prenatally. The most common cause of hydronephrosis is ureteropelvic junction obstruction. However, not all hydronephrotic kidneys are really obstructed. If renal parenchyme is preserved, patients with hydronephrosis are followed nonoperatively, and many of them resolve spontaneously in the first few years of life. A detailed ultrasonography and diuretic renogram (dynamic renal scintigraphy) are the two present most reliable diagnostic tools. IVU is not useful in early childhood. An increase in hydronephrosis and/or a decrease in the differential renal function are the major indications for surgical treatment. Depending on the nature, and location of the obstruction, a variety of surgical methods are available, many of which can be performed by endoscopy or laparoscopy. (Turk Arch Ped 2010; 45 Suppl: 85-9

Single-incision laparoscopy-assisted gastric transposition combined with thoracoscopic esophagectomy for esophageal replacement

WOS: 000474040900024PubMed ID: 30549226Introduction We present a novel approach to single-incision laparoscopy-assisted gastric transposition combined with thoracoscopic esophagectomy in a child with long-segment corrosive esophageal stricture (CES). Materials and Surgical Technique A 2.5-year-old boy with a history of caustic ingestion underwent periodic esophageal dilatations at 3-week intervals, four sessions of topical mitomycin C application, and a strict antacid/H-2 antagonist therapy for 8 months. The esophageal replacement was indicated because of persistent corrosive esophageal stricture. First, thoracoscopic native esophagus dissection was performed. After gastric mobilization with single-incision laparoscopy, esophagectomy and esophagogastric anastomosis were performed through the cervical incision. There was no complication, and the patient had gained weight by the end of the 12-month follow-up. Discussion Single-incision laparoscopy-assisted gastric transposition combined with thoracoscopic esophagectomy is feasible, safe, and effective in patients with corrosive esophageal stricture

Laparoscopic adrenalectomy in children: A 25-case series and review of the literature

WOS: 000442653100029PubMed ID: 29254846Background: Laparoscopic adrenalectomy (LA) is the gold standard and is widely performed in adults, but its use in children is relatively new. We aim to present our experience in twenty-five children with diverse adrenal pathologies and to discuss an extensive review of pediatric LA in English literature. Methods: Medical records of children with adrenal tumors admitted to a tertiary center and treated with LA were reviewed. Characteristics and outcome of patients were compared with results of a systematic Pubmed/Medline literature review. Results: Transperitoneal LAs were performed for 12 malignant and 14 benign adrenal masses (size range: 2-8 cm) in 25 children (median age: 63 months). Fourteen lesions were on the right side, and there was one bilateral case. There were no conversion and no complication within 36 months of follow-up. A literature review revealed 437 pediatric LAs with left side predomination (51.4%). The indication was a malignant lesion in 60.2% of the cases (sizes range: 1-10 cm). The transperitoneal route was preferred in 94.2% of the procedures. Conversion and complication rates were 7.5% and 3.1%, respectively. Conclusion: LA should be preferred in selected children with adrenal pathologies. The transperitoneal route seems to be the standard approach for pediatric surgeons. Preoperative planning and surgical expertise are the keys to success. (C) 2017 Elsevier Inc. All rights reserved

Can increased intra-abdominal pressure (IAP) be treated more effectively with intravesical pressure measurement in high-risk patients?

WOS: 000328883200011PubMed ID: 24347217BACKGROUND: Increased intra-abdominal pressure (IAP) can result in multiorgan failure. Trauma, mechanical bowel obstruction (MBO), abdominal wall defects (AWD) and fluid resuscitation in septic shock are conditions associated with a high risk of increased IAP in children. It may be possible to treat these conditions more effectively using intravesical pressure measurement. MEHTOHDS: This prospective study was performed between December 2009 and October 2010. Intravesical pressure (IVP) measurement was used to determine IAP. The presence of Intra-abdominal hypertension (IAP > 12 mmHg) and abdominal compartment syndrome (IAP > 15 mmHg + new organ dysfunction) was evaluated in all clinical decisions. RESULTS: IVP monitoring was applied in all patients with abdominal trauma (14), AWD (8), fluid resuscitation for septic shock (7), and MBO (6). A diagnosis of ACS was determined in 14% of trauma patients and required surgery. IAH/ACS occurred in 38% of AWD cases, which required closure. IAH occurred in 43% of patients undergoing fluid resuscitation for septic shock, requiring decompressive intervention. IAH/ACS occurred in 50 % of MBO patients, all of whom required surgery. DISCUSSION: IVP measurement is a useful method to identify candidates for surgical treatment in cases of trauma and MBO. Similarly, IVP monitoring can facilitate the closure of abdominal wall defects and the application of fluid resuscitation in septic shock

Laparoscopic treatment of median arcuate ligament syndrome: A rare cause of chronic severe abdominal pain

Median arcuate ligament syndrome is a rare disorder characterized by chronic postprandial abdominal pain and weight loss caused by compression on celiac artery. A 17-year-old girl with chronic severe abdominal pain and weight loss was referred to our clinic. Other causes of chronic abdominal pain were investigated and excluded. The compression on celiac artery was detected on Doppler ultrasound and diagnosis was confirmed by computed tomography angiography. The patient underwent laparoscopic release of median arcuate ligament. There were no intraoperative complications; however, partial pain response was observed postoperatively that necessitated para-spinal ganglion blockage. The patient is symptom-free in 1-year follow-up period