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{{CMG}}; {{AE}}


== tab ==
{{CMG}}; {{AE}}{{Qurrat}}
 
 
==Managemnet of Congenital melanocytic Nevi==
 
https://www.uptodate.com/contents/congenital-melanocytic-nevi?search=melanocytic%20nevus%20pathophysiology&sectionRank=1&usage_type=default&anchor=H2&source=machineLearning&selectedTitle=1~44&display_rank=1#H2
 
MANAGEMENT
 
Small/medium CMN — Small and medium-sized CMN are managed on an individual basis depending on factors that affect ease of monitoring (eg, color, thickness/topography, and location), clinical history, parents' anxiety, and cosmetic concerns [4]. As an example, a multinodular black CMN on the scalp that is partially obscured by dense hair growth would be difficult to follow clinically, whereas a thin light brown lesion on the face would be relatively simple to observe. However, the latter might be removed for cosmetic reasons, and the former may spontaneously lighten during childhood.
 
Periodic evaluation of small- and medium-sized CMN is most important after puberty, since the risk of melanoma arising within these lesions during childhood is extremely low. Baseline photographs can be helpful, and dermoscopy represents a useful tool for assessing changes. (See "Dermoscopic evaluation of skin lesions".)
 
Patients and parents should be instructed to perform skin self-examinations and to bring focal changes in color, border, or topography (eg, a red or black papule, nodule, or crust) to the clinician's attention. (See "Screening and early detection of melanoma in adults and adolescents", section on 'Patient self-examination'.)
 
Large CMN — Early surgical removal is often desired for large CMN because of their cosmetic and psychosocial sequelae and concern for possible malignant transformation. Complete excision is difficult to achieve; however, resection of bulky and cumbersome portions of large CMN can be beneficial for some patients. Elimination of every nevus cell may be impossible because of the large area of skin affected, the anatomic site (eg, distal extremity, periocular area, genitalia), and involvement of deeper structures (eg, fat, fascia, muscle). Even theoretically complete surgical excision cannot completely eliminate future risk of melanoma, as some melanomas in these patients may develop in the CNS or retroperitoneum. In many cases, close clinical observation with no surgical removal of the lesion is a reasonable choice.
 
Factors that affect the decision to perform surgery as well as to determine the timing of surgery include the size and location of the large CMN, the technical difficulty of the procedure(s) required, and anesthesia options. When possible, complete removal of large CMN usually necessitates staged excision with the use of tissue expanders and, occasionally, skin grafting [45].
 
When surgical excision is not feasible, cosmetic benefit may potentially be obtained from procedures such as curettage, dermabrasion, and ablative laser therapy (eg, carbon dioxide or erbium:yttrium aluminum garnet lasers, sometimes combined with pigment-directed lasers). During the neonatal period, there is a lower risk of excessive scarring following such interventions, and nevus cells are more accessible because they are concentrated in the upper dermis [46,47]. Curettage can be performed during the first two weeks of life, taking advantage of a cleavage plane between the upper and mid-dermis exclusive to neonatal skin. However, nevus cells remain in the dermis after all of these procedures, as evidenced by frequent repigmentation as well as several reports of the subsequent development of melanoma in treated areas [48-52]. This underscores the need for lifelong clinical observation.
 
Regardless of the treatments employed, patients with large CMN (or scars after their excision) should be followed with periodic skin and general physical examinations. Palpation of the nevus and/or scars is essential for detection of focal induration. Histologic evaluation is indicated for firm nodules or indurated areas. Even theoretically complete removal of a large CMN does not eliminate the risk of melanoma, since melanoma of the CNS and other visceral primary sites (eg, the retroperitoneum) may still occur [53].
 
Proliferative nodules that develop within large CMN during infancy can have histologic features of melanoma yet behave in a benign manner. Techniques such as comparative genomic hybridization can help to distinguish proliferative nodules (usually having no chromosomal aberrations or only numeric changes) from melanoma (typically demonstrating gains/losses of chromosomal fragments) [40]. Mass spectroscopy imaging proteomic analysis may also help differentiate proliferative nodules from melanoma [29]. (See 'Proliferative nodules' above.)
 
Surveillance for neurocutaneous melanosis — Patients with a large CMN plus multiple (especially >20) satellite nevi or with multiple medium-sized CMN are at risk for NCM and should be followed with serial head circumference measurements, neurologic examinations, and developmental assessments [3,37,39]. This monitoring includes evaluation for signs and symptoms of increased intracranial pressure, mass lesions, and spinal cord compression [3,39].
 
Gadolinium-enhanced magnetic resonance imaging (MRI) of brain and spine should be performed in any high-risk patient exhibiting neurologic symptoms, and we suggest that asymptomatic high-risk patients also be screened for NCM with gadolinium-enhanced MRI of the brain and spine, ideally during the first six months of life before myelination, which may obscure evidence of melanosis [42]. For very young infants, it may be possible to obtain initial high-quality MRI images without general anesthesia using "feed and wrap" techniques that allow a swaddled infant to sleep during the imaging procedure [54].
 
Given the poor prognosis, aggressive surgical procedures for CMN removal should be postponed in patients with symptomatic NCM. NCM in an asymptomatic patient does not necessarily preclude skin surgery.
 
 
 
 
 
 


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! colspan="7" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para-clinical findings
! colspan="7" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para-clinical findings
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Comments
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Additional findings
|-
|-
| colspan="6" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
| colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
! colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
|-
|-
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
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! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
|-  
|-  
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Constipation/Diarrhea
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptom 1
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Blood in stool
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptom 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Abdominal pain
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptom 3
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other symptoms
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical exam 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Anemia
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical exam 2
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Tumor marker
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical exam 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Endoscopy
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |CT scan
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other imaging study
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab 3
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 1
| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Adenocarcinoma]]
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 2
| style="background: #F5F5F5; padding: 5px;" | +/-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 3
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +/-
| colspan="3" style="background: #F5F5F5; padding: 5px;" |
* [[Tenesmus]]
* Diminished caliber of stools
* [[Mucus]] in stools
| style="background: #F5F5F5; padding: 5px;" | +
| colspan="2" style="background: #F5F5F5; padding: 5px;" |[[CEA]]+
| style="background: #F5F5F5; padding: 5px;" |
* [[Sigmoidoscopy]] may show [[Polyp|polyps]], [[Ulceration|ulcerating]] and infiltrating [[lesions]]
* [[Colonoscopy]] can reveal detail images of [[polyps]] and [[cancerous]] [[lesions]]
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*Luminal narrowing and [[bowel obstruction]]
*Circumferential thickening of the bowel wall
*[[Enlarged lymph nodes]]
*Pulmonary [[metastases]]
*Peritoneal metastases
*[[Metastases|Hepatic metastases]]
| style="background: #F5F5F5; padding: 5px;" |
* [[PET scan|PET scans]] for detailed images
* [[Barium enema]] shows the [[luminal]] abnormalities
* [[Genetic testing]] to see hereditary etiology
| style="background: #F5F5F5; padding: 5px;" |
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| style="background: #F5F5F5; padding: 5px;" |
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |AVM<ref name="pmid28139503">{{cite journal |vauthors=Lee HH, Kwon HM, Gil S, Kim YS, Cho M, Seo KJ, Chae HS, Cho YS |title=Endoscopic resection of asymptomatic, colonic, polypoid arteriovenous malformations: Two case reports and a literature review |journal=Saudi J Gastroenterol |volume=23 |issue=1 |pages=67–70 |date=2017 |pmid=28139503 |pmc=5329980 |doi=10.4103/1319-3767.199111 |url=}}</ref>
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| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| colspan="3" style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| colspan="2" style="background: #F5F5F5; padding: 5px;" |
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| style="background: #F5F5F5; padding: 5px;" |
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Diverticulitis<ref name="pmid16187597">{{cite journal |vauthors=Shen SH, Chen JD, Tiu CM, Chou YH, Chiang JH, Chang CY, Lee CH |title=Differentiating colonic diverticulitis from colon cancer: the value of computed tomography in the emergency setting |journal=J Chin Med Assoc |volume=68 |issue=9 |pages=411–8 |date=September 2005 |pmid=16187597 |doi=10.1016/S1726-4901(09)70156-X |url=}}</ref>
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" | +
| colspan="3" style="background: #F5F5F5; padding: 5px;" |
* Fever+/- chills
* Nausea+/- vomiting
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* Leukocytosis
| colspan="2" style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Evidence of inflammation
* Outpouchings of the colonic wall
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| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Hemorrhoid
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
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| style="background: #F5F5F5; padding: 5px;" |
| colspan="2" style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
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|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Anal fissure
| style="background: #DCDCDC; padding: 5px; text-align: center;" |
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|-
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |Infectious colitis
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|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Peutz-Jeghers syndrome<ref name="pmid27298573">{{cite journal |vauthors=Zhong ME, Niu BZ, Ji WY, Wu B |title=Laparoscopic restorative proctocolectomy with ileal pouch-anal anastomosis for Peutz-Jeghers syndrome with synchronous rectal cancer |journal=World J. Gastroenterol. |volume=22 |issue=22 |pages=5293–6 |date=June 2016 |pmid=27298573 |doi=10.3748/wjg.v22.i22.5293 |url=}}</ref>
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|-
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
!Diseases
| colspan="6" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
!Symptom 1
! colspan="7" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para-clinical findings
! colspan="1" rowspan="1" |Symptom 2
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
!Symptom 3
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Comments
!Physical exam 1
! colspan="1" rowspan="1" |Physical exam 2
!Physical exam 3
!Lab 1
!Lab 2
!Lab 3
!Imaging 1
!Imaging 2
!Imaging 3
!Histopathology
|'''Gold standard'''
!Additional findings
|-
|-
| colspan="6" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
| style="background: #DCDCDC; padding: 5px; text-align: center;" |
|-
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Constipation/Diarrhea
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Blood in stool
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Abdominal pain
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other symptoms
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |CBC
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Stool test
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Colonoscopy
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |CT scan
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Carcinoids<ref name="pmid20011309">{{cite journal |vauthors=Chung TP, Hunt SR |title=Carcinoid and neuroendocrine tumors of the colon and rectum |journal=Clin Colon Rectal Surg |volume=19 |issue=2 |pages=45–8 |date=May 2006 |pmid=20011309 |pmc=2780103 |doi=10.1055/s-2006-942343 |url=}}</ref>
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|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Juvenile Polyposis Coli
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|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Gastrointestinal Stromal Tumors (GIST)<ref name="pmid24778074">{{cite journal |vauthors=Niazi AK, Kaley K, Saif MW |title=Gastrointestinal stromal tumor of colon: a case report and review of literature |journal=Anticancer Res. |volume=34 |issue=5 |pages=2547–50 |date=May 2014 |pmid=24778074 |doi= |url=}}</ref>
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|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Hamartoms
| style="background: #DCDCDC; padding: 5px; text-align: center;" |
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|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Colorectal Lymphoma<ref name="pmid20011310">{{cite journal |vauthors=Quayle FJ, Lowney JK |title=Colorectal lymphoma |journal=Clin Colon Rectal Surg |volume=19 |issue=2 |pages=49–53 |date=May 2006 |pmid=20011310 |pmc=2780105 |doi=10.1055/s-2006-942344 |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 1
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|-
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |Kaposi's sarcoma
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 2
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|-
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |Ulcerative colitis<ref name="pmid25075198">{{cite journal |vauthors=Fakhoury M, Negrulj R, Mooranian A, Al-Salami H |title=Inflammatory bowel disease: clinical aspects and treatments |journal=J Inflamm Res |volume=7 |issue= |pages=113–20 |date=2014 |pmid=25075198 |pmc=4106026 |doi=10.2147/JIR.S65979 |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 3
| style="background: #F5F5F5; padding: 5px;" |Diarrhea
| style="background: #F5F5F5; padding: 5px;" |<big>+</big>
| style="background: #F5F5F5; padding: 5px;" |LLQ<ref name="pmid25075198">{{cite journal |vauthors=Fakhoury M, Negrulj R, Mooranian A, Al-Salami H |title=Inflammatory bowel disease: clinical aspects and treatments |journal=J Inflamm Res |volume=7 |issue= |pages=113–20 |date=2014 |pmid=25075198 |pmc=4106026 |doi=10.2147/JIR.S65979 |url=}}</ref>
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* Vitamin B12 defi. anemia<ref name="pmid25075198">{{cite journal |vauthors=Fakhoury M, Negrulj R, Mooranian A, Al-Salami H |title=Inflammatory bowel disease: clinical aspects and treatments |journal=J Inflamm Res |volume=7 |issue= |pages=113–20 |date=2014 |pmid=25075198 |pmc=4106026 |doi=10.2147/JIR.S65979 |url=}}</ref>
* Autoimmune hemolytic anemia
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* Continuous lesions, presence of crypts, formation of residual mucosal tissue<ref name="pmid25075198">{{cite journal |vauthors=Fakhoury M, Negrulj R, Mooranian A, Al-Salami H |title=Inflammatory bowel disease: clinical aspects and treatments |journal=J Inflamm Res |volume=7 |issue= |pages=113–20 |date=2014 |pmid=25075198 |pmc=4106026 |doi=10.2147/JIR.S65979 |url=}}</ref>
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* Mucosal and submucosal inflammation<ref name="pmid25075198">{{cite journal |vauthors=Fakhoury M, Negrulj R, Mooranian A, Al-Salami H |title=Inflammatory bowel disease: clinical aspects and treatments |journal=J Inflamm Res |volume=7 |issue= |pages=113–20 |date=2014 |pmid=25075198 |pmc=4106026 |doi=10.2147/JIR.S65979 |url=}}</ref>
* Hemorrhage or inflammatory polymorphonuclear cells aggregate in the lamina propria
* Distorted crypts
* Crypt abscess
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* Endoscopy and a mucosal biopsy<ref name="pmid16902215" />
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |Crohn's disease<ref name="pmid25075198">{{cite journal |vauthors=Fakhoury M, Negrulj R, Mooranian A, Al-Salami H |title=Inflammatory bowel disease: clinical aspects and treatments |journal=J Inflamm Res |volume=7 |issue= |pages=113–20 |date=2014 |pmid=25075198 |pmc=4106026 |doi=10.2147/JIR.S65979 |url=}}</ref>
| style="background: #F5F5F5; padding: 5px;" |Diarrhea
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* Vitamin B12 defi. anemia<ref name="pmid25075198">{{cite journal |vauthors=Fakhoury M, Negrulj R, Mooranian A, Al-Salami H |title=Inflammatory bowel disease: clinical aspects and treatments |journal=J Inflamm Res |volume=7 |issue= |pages=113–20 |date=2014 |pmid=25075198 |pmc=4106026 |doi=10.2147/JIR.S65979 |url=}}</ref>
* Autoimmune hemolytic anemia
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* Discontinuous lesions, strictures, linear ulcerations<ref name="pmid25075198">{{cite journal |vauthors=Fakhoury M, Negrulj R, Mooranian A, Al-Salami H |title=Inflammatory bowel disease: clinical aspects and treatments |journal=J Inflamm Res |volume=7 |issue= |pages=113–20 |date=2014 |pmid=25075198 |pmc=4106026 |doi=10.2147/JIR.S65979 |url=}}</ref>
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* Transmural pattern of inflammation<ref name="pmid25075198">{{cite journal |vauthors=Fakhoury M, Negrulj R, Mooranian A, Al-Salami H |title=Inflammatory bowel disease: clinical aspects and treatments |journal=J Inflamm Res |volume=7 |issue= |pages=113–20 |date=2014 |pmid=25075198 |pmc=4106026 |doi=10.2147/JIR.S65979 |url=}}</ref>
* Mucosal damage
* Focal infiltration of leukocytes into the epithelium
* Granulomas
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* Endoscopy and a mucosal biopsy<ref name="pmid16902215">{{cite journal |vauthors=Collins P, Rhodes J |title=Ulcerative colitis: diagnosis and management |journal=BMJ |volume=333 |issue=7563 |pages=340–3 |date=August 2006 |pmid=16902215 |pmc=1539087 |doi=10.1136/bmj.333.7563.340 |url=}}</ref>
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |Irritable bowel syndrome
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Appendicitis]]
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |Paralytic ileus
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!Diseases
!Diseases
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!Additional findings
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |[[Strangulated hernia]]
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 4
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |Prostate Cancer
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 5
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |Bowel endometriosis
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 6
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==Table for Differential Diagnosis of Small Intestine Cancer==
'''<small>ABBREVIATIONS''':
'''N/A''': Not available, '''NL''': Normal,</small><small><nowiki/></small><small><nowiki/></small>
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Latest revision as of 16:22, 17 May 2019


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Qurrat-ul-ain Abid, M.D.[2]


Managemnet of Congenital melanocytic Nevi

https://www.uptodate.com/contents/congenital-melanocytic-nevi?search=melanocytic%20nevus%20pathophysiology&sectionRank=1&usage_type=default&anchor=H2&source=machineLearning&selectedTitle=1~44&display_rank=1#H2

MANAGEMENT

Small/medium CMN — Small and medium-sized CMN are managed on an individual basis depending on factors that affect ease of monitoring (eg, color, thickness/topography, and location), clinical history, parents' anxiety, and cosmetic concerns [4]. As an example, a multinodular black CMN on the scalp that is partially obscured by dense hair growth would be difficult to follow clinically, whereas a thin light brown lesion on the face would be relatively simple to observe. However, the latter might be removed for cosmetic reasons, and the former may spontaneously lighten during childhood.

Periodic evaluation of small- and medium-sized CMN is most important after puberty, since the risk of melanoma arising within these lesions during childhood is extremely low. Baseline photographs can be helpful, and dermoscopy represents a useful tool for assessing changes. (See "Dermoscopic evaluation of skin lesions".)

Patients and parents should be instructed to perform skin self-examinations and to bring focal changes in color, border, or topography (eg, a red or black papule, nodule, or crust) to the clinician's attention. (See "Screening and early detection of melanoma in adults and adolescents", section on 'Patient self-examination'.)

Large CMN — Early surgical removal is often desired for large CMN because of their cosmetic and psychosocial sequelae and concern for possible malignant transformation. Complete excision is difficult to achieve; however, resection of bulky and cumbersome portions of large CMN can be beneficial for some patients. Elimination of every nevus cell may be impossible because of the large area of skin affected, the anatomic site (eg, distal extremity, periocular area, genitalia), and involvement of deeper structures (eg, fat, fascia, muscle). Even theoretically complete surgical excision cannot completely eliminate future risk of melanoma, as some melanomas in these patients may develop in the CNS or retroperitoneum. In many cases, close clinical observation with no surgical removal of the lesion is a reasonable choice.

Factors that affect the decision to perform surgery as well as to determine the timing of surgery include the size and location of the large CMN, the technical difficulty of the procedure(s) required, and anesthesia options. When possible, complete removal of large CMN usually necessitates staged excision with the use of tissue expanders and, occasionally, skin grafting [45].

When surgical excision is not feasible, cosmetic benefit may potentially be obtained from procedures such as curettage, dermabrasion, and ablative laser therapy (eg, carbon dioxide or erbium:yttrium aluminum garnet lasers, sometimes combined with pigment-directed lasers). During the neonatal period, there is a lower risk of excessive scarring following such interventions, and nevus cells are more accessible because they are concentrated in the upper dermis [46,47]. Curettage can be performed during the first two weeks of life, taking advantage of a cleavage plane between the upper and mid-dermis exclusive to neonatal skin. However, nevus cells remain in the dermis after all of these procedures, as evidenced by frequent repigmentation as well as several reports of the subsequent development of melanoma in treated areas [48-52]. This underscores the need for lifelong clinical observation.

Regardless of the treatments employed, patients with large CMN (or scars after their excision) should be followed with periodic skin and general physical examinations. Palpation of the nevus and/or scars is essential for detection of focal induration. Histologic evaluation is indicated for firm nodules or indurated areas. Even theoretically complete removal of a large CMN does not eliminate the risk of melanoma, since melanoma of the CNS and other visceral primary sites (eg, the retroperitoneum) may still occur [53].

Proliferative nodules that develop within large CMN during infancy can have histologic features of melanoma yet behave in a benign manner. Techniques such as comparative genomic hybridization can help to distinguish proliferative nodules (usually having no chromosomal aberrations or only numeric changes) from melanoma (typically demonstrating gains/losses of chromosomal fragments) [40]. Mass spectroscopy imaging proteomic analysis may also help differentiate proliferative nodules from melanoma [29]. (See 'Proliferative nodules' above.)

Surveillance for neurocutaneous melanosis — Patients with a large CMN plus multiple (especially >20) satellite nevi or with multiple medium-sized CMN are at risk for NCM and should be followed with serial head circumference measurements, neurologic examinations, and developmental assessments [3,37,39]. This monitoring includes evaluation for signs and symptoms of increased intracranial pressure, mass lesions, and spinal cord compression [3,39].

Gadolinium-enhanced magnetic resonance imaging (MRI) of brain and spine should be performed in any high-risk patient exhibiting neurologic symptoms, and we suggest that asymptomatic high-risk patients also be screened for NCM with gadolinium-enhanced MRI of the brain and spine, ideally during the first six months of life before myelination, which may obscure evidence of melanosis [42]. For very young infants, it may be possible to obtain initial high-quality MRI images without general anesthesia using "feed and wrap" techniques that allow a swaddled infant to sleep during the imaging procedure [54].

Given the poor prognosis, aggressive surgical procedures for CMN removal should be postponed in patients with symptomatic NCM. NCM in an asymptomatic patient does not necessarily preclude skin surgery.




Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Symptom 1 Symptom 2 Symptom 3 Physical exam 1 Physical exam 2 Physical exam 3 Lab 1 Lab 2 Lab 3 Imaging 1 Imaging 2 Imaging 3
Diseases Symptom 1 Symptom 2 Symptom 3 Physical exam 1 Physical exam 2 Physical exam 3 Lab 1 Lab 2 Lab 3 Imaging 1 Imaging 2 Imaging 3 Histopathology Gold standard Additional findings
Differential Diagnosis 1
Differential Diagnosis 2
Differential Diagnosis 3
Diseases Symptom 1 Symptom 2 Symptom 3 Physical exam 1 Physical exam 2 Physical exam 3 Lab 1 Lab 2 Lab 3 Imaging 1 Imaging 2 Imaging 3 Histopathology Gold standard Additional findings
Differential Diagnosis 4
Differential Diagnosis 5
Differential Diagnosis 6

Table for Differential Diagnosis of Small Intestine Cancer

ABBREVIATIONS:

N/A: Not available, NL: Normal,

References