Focal acantholytic dyskeratosis has a distinctive histological pattern that is associated with various clinical expressions. It rarely occurs on the lip or the perianal area. We report a patient with focal acantholytic dyskeratosis occurring on both the upper lip and the anal canal. Histopathologically, the lesions showed hyperkeratosis, suprabasilar clefting, epidermal acantholysis and dyskeratosis. This case represents the first report of a focal acantholytic dyskeratosis occurring on both the lip and the anal canal.
Acantholysis/*pathology ; Adult ; Anus Diseases/*pathology ; Human ; Keratosis/*pathology ; Lip Diseases/*pathology ; Male

Intersphincteric resection (ISR) is the ultimate sphincter-preserving surgical technique for low rectal cancer. To promote the standardized implementation of ISR, this review discusses the important issues regarding the clinical application of ISR with reference to the latest Chinese expert consensus on ISR. In terms of ISR-related pelvic anatomy of the rectum/anal canal, hiatal ligament is not identical with the anococcygeal ligament. At the level where the rectourethralis muscle continuously extends to the posteroinferior area of the membranous urethra from the rectum, the neurovascular bundle is identified between the posterior edge of rectourethralis muscle and the anterior edge of the longitudinal muscle of the rectum. This knowledge is crucial to detect the anterior dissection plane during ISR at the levator hiatus level. The indication criteria for ISR included: (1) stage I early low rectal cancer; (2) stage II-III low rectal cancer undergoing neoadjuvant treatment, and supra-anal tumors and juxta-anal tumors of stage ycT3NxM0, or intra-anal tumors of stage ycT2NxM0. However, signet ring cell carcinoma, mucinous adenocarcinoma and undifferentiated carcinoma should be contraindicated to ISR. For locally advanced low rectal cancer (especially anteriorly located tumor), neoadjuvant treatment should be carried out in a standardized manner. However, it should be recognized that neoadjuvant chemoradiotherapy was a risk factor for poor anal function after ISR. For surgical approaches for ISR, including transanal, transabdominal, and transanal transabdominal approaches, the choice should be based on oncological safety and functional consequences. While ensuring the negative margin, maximal preservation of rectal walls and anal canal contributs to better postoperative anorectal function. Careful attention must be paid to complications regarding ISR, with special focus on the anastomotic complications. The incidence of low anterior resection syndrome (LARS) was higher than 40%. However, this issue is often neglected by clinicians. Thus, management and rehabilitation strategies for LARS with longer follow-ups were required.
Humans ; Rectal Neoplasms/pathology* ; Postoperative Complications ; Laparoscopy/methods* ; Anal Canal/pathology* ; Anus Neoplasms/pathology* ; Anus Diseases/surgery* ; Low Anterior Resection Syndrome ; Carcinoma, Signet Ring Cell/pathology* ; Treatment Outcome

OBJECTIVETo investigate magnetic resonance imaging(MRI) manifestations of perianal abscess and to evaluate MRI diagnosis for perianal abscess.

METHODSFifty cases of perianal abscess between July 2007 and March 2009 were included in this study. MRI was performed using T1 weighted sequence in axial plane, T2 weighted sequence with fat saturation in axial, coronal and/or sagittal plane, and T1 weighted enhanced sequence with fat saturation in axial, coronal and/or sagittal plane. The location, size, shape, signal characteristics, and enhancement patterns of perianal abscess were analyzed.

RESULTSFifty cases with 51 perianal abscesses were identified. The abscess appeared slight hypo- or isointense signal on T1 weighted imaging, obviously hyperintense signal at cavity and isointense signal at wall on T2 weighted with fat saturation imaging, and marked enhancement at wall. The largest diameters ranged between 0.5 cm and 9.0 cm (mean 3.4±1.7 cm ) in abscess, between 0.2 cm and 8.0 cm(mean 2.7±1.7 cm) in cavity. The abscess was round, oblong and crescent in 23, 18 and 7 cases respectively, and was unilocular in 41 cases and multilocular in 10 cases. The abscess located between levator ani and intersphincteric groove in 23 cases, under intersphincteric groove in 3 cases, across levator ani and intersphincteric groove in one case, on levator ani in 7 cases, on intersphincteric groove in 16 cases and above levator ani in one case.

CONCLUSIONMRI is a noninvasive, convenient and highly accurate procedure for diagnosing perianal abscess, which can demonstrate the anatomical relationship between abscess and anal canal.

Abscess ; diagnosis ; pathology ; Adolescent ; Adult ; Aged ; Anus Diseases ; diagnosis ; pathology ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

BACKGROUND/AIMS: Pediatric inflammatory bowel disease (IBD) has been increasing worldwide. The characteristics of pediatric-onset IBD have mainly been reported in Western countries. We investigated the clinical characteristics of pediatric IBD in Korea and compared these with the data from the 5-year European multicenter study of children with new-onset IBD (EUROKIDS registry). METHODS: Children who were diagnosed with IBD between July 1987 and January 2012 were investigated at five Korean university hospitals. Their clinical characteristics were retrospectively evaluated by medical record review. The results were compared with the EUROKIDS data. RESULTS: A total of 30 children with Crohn's disease (CD) and 33 children with ulcerative colitis (UC) were enrolled. In comparison with the EUROKIDS group, Korean pediatric IBD patients showed a male predominance (86.7% vs 59.2%, p=0.002 in CD; 75.8% vs 50%, p=0.003 in UC). Korean pediatric CD patients had a higher prevalence of terminal ileal disease (36.7% vs 16.3%, p=0.004) and perianal disease (33.3% vs 8.2%, p<0.001) than patients in the EUROKIDS group. Korean pediatric UC patients had a higher prevalence of proctitis than patients in the EUROKIDS group. CONCLUSIONS: Our results suggest that the characteristics of Korean pediatric IBD patients and European pediatric IBD patients may be different.
Adolescent ; Anus Diseases/complications/epidemiology/pathology ; Child ; Colitis, Ulcerative/complications/epidemiology/*pathology ; Crohn Disease/complications/epidemiology/*pathology ; Europe/epidemiology ; Female ; Humans ; Ileal Diseases/complications/epidemiology/pathology ; Male ; Prevalence ; Proctitis/epidemiology/etiology ; Registries ; Republic of Korea/epidemiology ; Retrospective Studies ; Sex Factors

OBJECTIVETo observe the clinical efficacy on spastic pelvic floor syndrome (SPFS) treated with electroacupuncture (EA) at Shangliao (BL 31), Ciliao (BL 32), Zhongliao (BL 33) and Xialiao (BL 34).

METHODSThirty-six cases of SPFS were treated with EA at Shangliao (BL 31), Ciliao (BL 32), Zhongliao (BL 33) and Xialiao (BL 34), intermittent wave, 60 times/min in frequency, retained for 20 min. In general, the acupoints on one side were stimulated in each treatment. The bilateral acupoints stimulation was applied in serious cases. The treatment was given once every two days, five treatments made one session and totally three sessions were required. Before and after treatment, the clinical symptoms, anal kinetic indices defecation radiographic changes were observed. The follow-up observation was done in three months after the end of treatment.

RESULTSIn three-months follow-up after treatment, 14 cases were cured, 18 cases improved and 4 cases failed. The total effective rate was 88. 9%. After treatment, the cases of incomplete defecation, difficult defecation, anal pain and anal obstruction were reduced apparently as compared with those before treatment, indicating the statistically significant differences (all P<0.01). After treatment, rectal anal reflex threshold (ARA) was increased, anal maximal contraction pressure (AMCP) was reduced to (16.62±1.54) kPa and anal rest pressure (ARP) was significantly reduced to (7.22±0.36) kPa, indicating the statistical differences as compared with those before treatment (all P<0.01). After treatment, anorectal angle (ARA) in forceful defecation was increased to (116.55±9.42)°, the distance between the anorectal junction and the pubococcygeal line was decreased, and the impression of puborectal muscle was alleviated apparently as compared with that before treatment (P<0.01).

CONCLUSIONEA at Shangliao (BL 31), Ciliao (BL 32), Zhongliao (BL 33) and Xialiao (BL 34) achieves definite efficacy on SPFS and this therapeutic method obviously relieves the symptoms and deserves to be promoted in clinic.

Acupuncture Points ; Adult ; Aged ; Anus Diseases ; physiopathology ; therapy ; Constipation ; physiopathology ; therapy ; Defecation ; Electroacupuncture ; Female ; Humans ; Male ; Middle Aged ; Muscle Spasticity ; physiopathology ; therapy ; Pelvic Floor ; pathology

OBJECTIVETo study the clinical characteristics and diagnosis of the Johanson-Blizzard syndrome.

METHODThe clinical characteristics and diagnosing procedure of 1 case with Johanson-Blizzard syndrome were analyzed, and genetic analysis was made in diagnosing procedure, and 28 cases of Johanson-Blizzard syndrome with detailed clinical data were reviewed and analyzed.

RESULTA one year and nine months old girl, who was initially admitted to the hospital because of fatty diarrhea and increased frequency of defecation. Imperforate anus, and aplastic alae nasi was noticed after birth. On physical examination, short stature, mental retardation, tooth abnormalities and scalp defects were observed. Fat globule was found by routine stool test. Serum biochemistry showed an exocrine and endocrine pancreatic insufficiency, CT scan of the abdomen demonstrated fatty replacement of the pancreas, UBR1 gene analysis showed heterozygous for two missense changes. In all 29 cases, exocrine pancreatic insufficiency (72.4%) and hypoplasia of the alae nasi (93%) were the most common clinical manifestations, and sensorineural hearing loss (59%), scalp defects (69%) and hair thinning or upsweep of the hair (44.8%), hypothyroidism (44.8%), absence of permanent teeth (44.8%) and imperforate anus (21%) were also very common, but did not include consanguineous marriage of parents (10.3%).

CONCLUSIONJohanson-Blizzard syndrome is a rare autosomal recessive multisystem disorder, it is characterized by the association of congenital exocrine pancreatic insufficiency and hypoplasia or aplasia of the nasal wings, and can be diagnosed by clinical characteristics and UBR1 gene analysis.

Anus, Imperforate ; Deafness ; diagnosis ; genetics ; pathology ; Ectodermal Dysplasia ; diagnosis ; genetics ; pathology ; Female ; Growth Disorders ; Hearing Loss, Sensorineural ; Humans ; Hypothyroidism ; diagnosis ; genetics ; pathology ; Infant ; Intellectual Disability ; Nose ; abnormalities ; pathology ; Pancreatic Diseases ; diagnosis ; genetics ; pathology ; Ubiquitin-Protein Ligases ; genetics