| Xanthogranulomas
are rare benign nodules that usually arise in the subcutaneous tissue.
When they appear in early infancy or childhood they are called juvenile
xanthogranulomas (JXG). Approximately 20% are present at birth and most
arise in the first year of life. The anatomic predilection of
appearance of juvenile xanthogranuloma in children occurs in the head
and neck region, though appearance on the trunk, extremities and
extracutaneous locations has also been reported. Skin lesions are
self-limited and can vary in size; they can be solitary or multiple. In
the eye, particularly the uveal tract, is the most frequent site of
extracutaneous involvement. JXG are composed of collections of
histiocytes, foamy cells and Touton giant cells. The diagnosis of JXG
requires histologic analysis of a biopsy specimen or the nodule
itself. JXG is a benign cutaneous fibrohistiocytic lesion with a
type of granulomatous process. Clinically the lesion is papulonodular
with a tan to orange color and several millimeters in size. Children
with multiple skin lesions or younger than two years of age are at a
greater risk for ocular involvement. Systemic JXG which involves the
visceral organs is rare occurring in less than 5% of cases. Systemic
JXG can appear in the lung, heart, gastrointestinal tract, CNS, adrenal
gland, pituitary gland, bones or kidney. There is a sex male
predilection for JXG. Spontaneous regression of the cutaneous lesion is
frequent. Differential diagnosis includes spitz nevi, mastocytomas, and
dermatofibromas. Should the cutaneous nodule keep growing or cause
functional impairment it should be removed surgically. Removal must be
complete to avoid recurrence of the lesion. JXG generally carries a
good prognosis. Congenital giant JXG is a rare subtype most
commonly affecting newborn females. References: 1- Pajaziti L, Hapaiu SR, Pajaziti A: Juvenile xanthogranuloma: a case report and review of the literature. BMC Res Notes. 7:174, 2014 2- Logue ME(1), Elwood H, Smidt A: Congenital juvenile xanthogranuloma with ulceration: a pediatric case report. Dermatol Online J. 23(7), 2017 3- Paxton CN, O'Malley DP, Bellizzi AM, et al: Genetic evaluation of juvenile xanthogranuloma: genomic abnormalities are uncommon in solitary lesions, advanced cases may show more complexity. Mod Pathol. 30(9):1234-1240, 2017 4- Mitra S, Gupta S, Menon P, Rao KL, Bal A: Juvenile Xanthogranuloma: Presenting as an Isolated Renal Involvement. Fetal Pediatr Pathol. 35(6):420-424, 2016 5- Zahir ST, Sharahjin NS, Vahedian H, Akhavan A: Juvenile xanthogranuloma presenting as a large neck mass and ocular complications: a diagnostic and therapeutic dilemma. BMJ Case Rep. 2014 Apr 15;2014 6- Berti S, Coronella G, Galeone M, Balestri R, Patrizi A, Neri I: Giant congenital juvenile xanthogranuloma. Arch Dis Child. 98(4):317, 2013 |
| Detailed
surgical anatomy provided by today imaging technology is essential in
planning preoperative surgical decisions, surgical approach and
ultimate management of many conditions. Virtual 3 dimensional (3-D)
reconstruction techniques have been developed to help surgeons have a
more detailed situation of the anatomy of the disease. Two types of 3-D
rendering techniques are commonly utilized to visualize volumetric 3-D
data: surface shade display and volume rendering. These techniques
provide a global overview of volumetric data along with detailed
anatomical and pathological information which is usually more difficult
to discern in conventional 2-D images. In 2016 a new technique of 3-D
visualization of cross sectional image data called cinematic rendering
(CR) was developed. Cinematic rendering is a physically based
volume-rendering method that works with random sampling computational
algorithms. Multiple light maps and transfer function are used to
generate a realistic depiction of medical imaging data. This computer
animation program was first used by the entertainment industry. Data is
retrieved from either conventional CT and MRI scans to produce the
cinematic rendering images. CR helps to transfer complex surgical
anatomical information to physicians. CR visualization improves the
correctness of image interpretation. Moreover, CR enables the display
of CT image data in a colored fashion. Also, a 3-D printed anatomical
model can be reconstructed before the surgical procedure enabling the
surgeon to study the precise anatomic relationship of the tumor with
its vascular supply, an integral part of a surgical resection. Resident
surgeons benefit more than attending surgeons when using CR imaging as
it enhances anatomic education. CR imaging speeds up and improves the
understanding of complex anatomical situations compared with
conventional CT Scanning in the chest, abdomen and
pelvis. References: 1- Elshafei M, Binder J, Baecker J, et al: Comparison of Cinematic Rendering and Computed Tomography for Speed and Comprehension of Surgical Anatomy. JAMA Surg. Doi:10.1001/jamasurg.2019.1168 2- Yang J, Li K, Deng H, Feng J, Fei Y, Jin Y, Liao C, Li Q: CT cinematic rendering for pelvic primary tumor photorealistic visualization. Quant Imaging Med Surg. 8(8):804-818, 2018 3- Rowe SP, Johnson PT, Fishman EK: Initial experience with cinematic rendering for chest cardiovascular imaging. Br J Radiol. 2018 Feb;91(1082):20170558. doi: 10.1259/bjr.20170558. Epub 2017 Oct 27. 4- Dappa E, Higashigaito K, Fornaro J, Leschka S, Wildermuth S, Alkadhi H: Cinematic rendering - an alternative to volume rendering for 3D computed tomography imaging. Insights Imaging. 7(6):849-856, 2016 5- Eid M, De Cecco CN, Nance JW Jr, et al: Cinematic Rendering in CT: A Novel, Lifelike 3D Visualization Technique. AJR Am J Roentgenol. 209(2):370-379, 2017 6- Chu LC, Johnson PT, Fishman EK: Cinematic rendering of pancreatic neoplasms: preliminary observations and opportunities. Abdom Radiol (NY). 43(11):3009-3015, 2018 |
| Enhanced
recovery after anesthesia (ERAS) is a recently developed program of
standardization of perioperative care driven by clinical pathways
impacting both surgical outcomes and efficiency of care in high-volume
centers. ERAS pathways have been shown to reduce length of stay, reduce
complication rates, reduce providers communication errors and improve
patient satisfaction in some adults surgical procedures such as
colorectal surgery. Few studies have investigated the applicability of
this paradigm to pediatric populations. The ERAS society initially
developed in Europe organized the protocol into three contexts of care,
namely preoperative, intraoperative and postoperative. This include a
multidisciplinary team approach encompassing patient education,
increased preop volume loading, optimize nutrition, appropriated
antibiotic prophylaxis, short-acting anesthetics, maintenance of
normothermia, minimally invasive surgical techniques, avoiding drains,
using epidural for chest procedures, minimizing opioids analgesics,
limiting intra- and postop fluid resuscitation, early removal of drains
and catheters, prevention of nausea and vomiting, early oral nutrition,
early postop mobility and reporting outcomes. ERAS programs in adults
have reduced length of stay by one to two days without increasing
readmission rates, decrease postop complications by 30% and shortened
return of bowel function by one day. ERAS protocols offer an easy way
to improve the cost of care. ERAS protocol applied to the appropriate
pediatric surgical populations may be associated with decreased length
of stay, decreased narcotic use, and no detectable increase in
complications. Pediatric studies using ERAS have demonstrated less
intervention than the 20 components suggested for adults. These few
pediatric studies have demonstrated that ERAS implementation reduces
length of stay by one day with no increase in complications or
readmissions, less use of IV fluid in the operating room, less narcotic
during hospitalization with early tolerance of feedings. Shorter stay
becomes a patient safety benefit as errors and nosocomial infections
are reduced upon early discharge. ERAS protocol is well accepted by
patients and families. Recent poll demonstrated that pediatric surgeons
are already executing several individual ERAS interventions outside of
an official protocol. References: 1- Shinnick JK, Short HL, Heiss KF, Santore MT, Blakely ML, Raval MV: Enhancing recovery in pediatric surgery: a review of the literature. J Surg Res. 202(1):165-76, 2016 2- Leeds IL, Boss EF, George JA, Strockbine V, Wick EC, Jelin EB: Preparing enhanced recovery after surgery for implementation in pediatric populations. J Pediatr Surg. 51(12):2126-2129, 2016 3- Pearson KL, Hall NJ: What is the role of enhanced recovery after surgery in children? A scoping review. Pediatr Surg Int. 33(1):43-51, 2017 4- Short HL, Heiss KF, Burch K, Travers C, Edney J, Venable C, Raval MV: Implementation of an enhanced recovery protocol in pediatric colorectal surgery. J Pediatr Surg. 53(4):688-692, 2018 5- Short HL, Taylor N, Thakore M, Piper K, Baxter K, Heiss KF, Raval MV: A survey of pediatric surgeons' practices with enhanced recovery after children's surgery. J Pediatr Surg. 53(3):418-430, 2018 6- Rove KO, Edney JC, Brockel MA: Enhanced recovery after surgery in children: Promising, evidence-based multidisciplinary care. Paediatr Anaesth. 28(6):482-492, 2018 |
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