To examine pathophysiology of parameatal urethral cyst (PUC) with comparison between the surgical excision group and the spontaneous resolution group.
We retrospectively reviewed all patients ...diagnosed with PUC and aged ≤15 years. Initially, all the patients received observation without any treatment. The indication of surgical excision was family preference. The patients were analyzed according to their sex, age, symptoms, clinical course, and pathological examination findings.
Among the 54 boys visited our hospital for PUC. The median age at presentation was 35 (IQR: 12-50) months, including 7 (13.0%) neonates cases. In total, 38 (70.4%) patients were underwent surgical excision, 12 (22.2%) had spontaneous resolution during observation, 1 (1.9%) had observation without resolution, and 3 (5.6%) were lost to follow-up. The median size of PUC at the first visit was significantly larger in the surgical excision group (5 mm median, IQR 3-5) than in the spontaneous resolution group (1.5 mm median, IQR 1-2.5) (P <.0001). In this study, PUC ≥5 mm in diameter did not have spontaneous resolution.
This is the largest study of PUC conducted at a single institute. This study indicated that 22% of PUCs had spontaneous resolution, and PUCs <5 mm had potential for spontaneous resolution. The results recommend an observation of at least 5.5 months for patients with asymptomatic PUC <5 mm. By contrast, we recommend the surgical excision for symptomatic PUC ≥5 mm according to the family preference.
Purpose
Recent reports suggest that the COVID-19 pandemic may be influencing disease morbidity. The purpose of this study was to investigate pandemic-related changes in the incidence of pediatric ...surgical emergencies.
Methods
Data from patients with one of 8 typical conditions considered to be pediatric emergencies who presented at 3 hospitals close to central Tokyo were collated retrospectively from accident and emergency (AE) department records for 2020 and compared with data for 3 years prior to 2020.
Results
All subjects had similar demographic profiles. The total number of pediatric AE attendances from 2017 to 2020 was 2880 (2017:
n
= 600, 2018:
n
= 736, 2019:
n
= 817, and 2020:
n
= 727). Annual attendances were similar. Of the 8 conditions, there were significantly less cases of intussusception in 2020 than previously (23/727; 3.1% versus 132/2153; 6.1%)
p
< 0.01 and the number of emergency surgical interventions for intussusception was also significantly less in 2020 (0/23; 0% versus 13/132; 9.8%)
p
< 0.01.
Conclusion
The implementation of preventative measures to combat the COVID-19 pandemic in 2020 would appear to have influenced the etiopathogenesis of intussusception enough to significantly decrease its overall incidence and the requirement for emergency surgical intervention.
A modified pull-through (PT) distinguished by complete full-thickness removal of the posterior rectal cuff, initially developed as an open procedure in 1980, has been performed with laparoscopic ...assistance since 1997. Postoperative bowel dysfunction improved when the anatomic landmark for PT surgery was revised from the dentate line (DL) to the anorectal (or Herrmann's) line (ARL) in 2007. A 40-year (1980-2019) review of 153 consecutive rectal/rectosigmoid type Hirschsprung's disease (HD) patients is presented.
Data for postoperative bowel dysfunction and Hirschsprung-associated enterocolitis (HAEC) classified according to the American Pediatric Surgical Association (APSA) scale were obtained retrospectively.
PT was open (
= 43) and laparoscopic (
= 110). Dissection was DL (
= 57) and ARL (
= 96). Over 40 years, 5/153 patients (3.3%) had postoperative obstructive symptoms (POS), and 10/153 patients (6.5%) had 13 episodes of postoperative HAEC; APSA grades were: I (
= 4); II (
= 8); and III: (
= 1) presenting with explosive diarrhea (10/13; 76.9%), fever (10/13; 76.9%), abdominal distension (9/13; 69.2%), or bloody stools/shock (1/13 with grade III; 7.7%). The grade III case had histologically-proven transitional zone PT. Postoperative HAEC developed in 3/5 (60.0%) POS+ patients and 7/148 (4.7%) POS- patients (
= .002). Symptom duration and treatment were not correlated with APSA grades.
Complete full-thickness posterior rectal cuff excision and using the ARL reduced postoperative HAEC significantly in this series. Despite being anatomically distinct, the DL is inadequate as a precise landmark for PT surgery because it lacks functional relevance. The APSA scale could benefit from timely review to improve its clinical and prognostic value.
Abstract Background In 2007, we began using the anorectal line (ARL) as the landmark for commencing rectal mucosal dissection (RMD) instead of the dentate line (DL) during laparoscopy-assisted ...transanal pull-through (L-TAPT) for Hirschsprung's disease (HD). We conducted a medium-term prospective comparison of postoperative fecal continence (POFC) between DL and ARL cases to follow our short-term study. Methods POFC is assessed by scoring frequency of motions, severity of staining, severity of perianal erosions, anal shape, requirement for medications, sensation of rectal fullness, and ability to distinguish flatus from stool on a scale of 0 to 2 (maximum: 14). Results Patient demographics were similar for ARL (2007–2014: n = 33) and DL (1997–2006: n = 41). There were no intraoperative complications and 2 cases of postoperative colitis in both ARL (6.1%) and DL (4.9%). Mean annual medium-term POFC scores for the 4–7 term of this study were consistently better in ARL: 9.7 ± 1.4*, 10.1 ± 1.6*, 10.6 ± 1.6, and 11.3 ± 1.4* in ARL and 8.6 ± 1.5, 9.1 ± 1.6, 9.8 ± 1.9, 10.0 ± 1.6 in DL (*: p < 0.05). Conclusions Medium-term POFC is better when the ARL is used as the landmark for RMD during L-TAPT for HD.
Aim
Detailed implications of age at laparoscopic transanal pull-through (LTAPT) on postoperative bowel function (POBF) in Hirschsprung’s disease (HD) are somewhat obscure because of a spectrum of ...factors.
Methods
Age at surgery was used to categorize 106 consecutive postoperative HD cases treated by our modified LTAPT (JLTPAT) between 1997 and 2015; group A: < 3 months old (
n
= 31); group B: 3–11 months old (
n
= 44); group C: 1–3 years old (
n
= 19); and group D: ≥ 4 years old (
n
= 12). POBF was assessed by reviewing outpatient records 1, 3, 5, 7, and 10 years after JLTAPT prospectively and scoring each of 5 criteria on a scale of 0–2; best score = 10.
Results
Only operative time was statistically longer in group D versus groups A, B, and C. Differences in gender ratios, blood loss, duration of follow-up, and POBF scores were not statistically significant. Mean POBF scores over time were: group A: 6.8, 7.6, 8.4, 8.6, and 8.4; group B: 7.1, 7.8, 8.3, 8.5, and 9.0; group C: 6.9, 7.9, 8.1, 8.3, and 8.6; group D: 7.0, 7.4, 8.2, 8.1, and 8.5, respectively.
Conclusion
Age at JLTAPT was not correlated with POBF in HD.
Purpose
The incidence of perforation during antibiotic therapy (AT) of children triaged as non-complicated acute appendicitis (NC-Ap) was investigated.
Methods
Abdominal ultrasonography (US) and/or ...computed tomography (CT) scans from cases of perforation identified at appendectomy for failed AT were reassessed blindly by a panel of board-certified specialists for any evidence of pre-AT morbidity suggestive of perforation.
Results
Of 521 cases triaged as NC-Ap, symptoms resolved with AT in 452 cases (86.8%). All 69/521 (13.2%) cases with persistent symptoms had urgent appendectomy, and 12/521 (2.3%) were found to have perforated. Blind reassessment of US and/or CT scans from these cases identified seven with evidence of perforation when they were triaged as NC-Ap. Thus, the actual incidence of perforation during AT for NC-Ap was actually 12–7 = 5/521 (0.95%).
Conclusions
Perforation is generally believed to be a complication of AT, but inappropriate triaging of cases for AT can bias results by artificially inflating the number of perforations, in this study, by more than double. We are the first to assess the unbiased incidence of perforation during AT for NC-Ap, by reassessing pre-AT US and/or CT scans. The incidence of perforation during AT is actually negligible.
Dysgerminoma is a malignant ovarian germ cell tumor, and unlike sex-cord stromal tumors, endocrine manifestation is considered rare. Here, we report the first case of dysgerminoma presenting ...precocious puberty. The patient is a 7-year-old girl who presented with a breast development in Tanner stage 3. Serum estradiol (E2) was markedly elevated while luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were suppressed below the detection limit. Microscopically, the right ovarian mass displayed nests of large polygonal cells and fibrous septa which were focally concentrated by theca-like plump spindle cells. Immunohistochemistry revealed that the spindle cells expressed various steroidogenic enzymes involved in estrogen biosynthesis including P450 aromatase. The tumor was diagnosed with pure dysgerminoma with estrogen-producing functioning stroma. After the operation, serum E2 declined below the detection limit; LH and FSH returned within the normal range. This case demonstrates that even a conventional dysgerminoma can present endocrine manifestation through functioning stroma.
Background/aim
The behavior of enteric neural crest-derived cells (ENCC) during enteric nervous system (ENS) development is being gradually understood with the introduction of live-cell imaging. ...However, many of the analyses to date are two-dimensional and the precise multidirectional migration of ENCC has been challenging to interpret. Mice lacking the endothelin-B receptor gene,
Ednrb
−/−
mice, are widely used as a model for Hirschsprung’s disease (HD). We have recently developed a
Sox10
transgenic (Tg) mouse to visualize ENCC with enhanced green fluorescent protein (Venus). By breeding these two models, we have created a Venus-positive,
Sox10
Tg mouse with a deletion of the Ednrb gene,
Sox10
-Venus
+
/
Ednrb
−/−
mouse, to investigate the ENS in HD. The aim of this study was to investigate the behavior of migrating ENCC in the hindgut of the
Sox10
-Venus
+
/
Ednrb
−/−
mouse using three-dimensional and four-dimensional image analysis software.
Methods
To compare the ENCC behavior when the wavefront of ENCC reaches the mid-hindgut between HD mouse and control, we harvested the fetal hindguts of
Sox10
-Venus
+
/
Ednrb
−/−
mice on embryonic day 15.5 (E15.5) and
Sox10
-Venus
+
/
Ednrb
+/+
mice on E12.5, which was used as control. Dissected hindguts were cultured for 360 min and the time-lapse images were obtained using a confocal laser-scanning microscope. Each ENCC at the wavefront was tracked after adjusting the longitudinal axis of the gut to the
Y
axis and analyzed using Imaris software.
Results
Track displacement (TD)-Y indicates ENCC advancement in a rostral-caudal direction. TD-X and TD-Z indicate ENCC advancement perpendicular to the rostral–caudal axis. Mean TD-Y was 34.56 µm in HD, but 63.48 µm in controls. TD-Y/TD-XZ was not significantly different in both groups. However, the mean track speeds were significantly decreased in HD (72.87 µm/h) compared to controls (248.29 µm/h).
Conclusions
Our results showed that the track speed of ENCC advancement was markedly decreased in the HD mice compared to controls. This technique provides added information by tracking ENCC with depth perception, which has potential for further elucidating the altered behavior of ENCC in HD.
Oral‐facial‐digital syndrome type 1 (OFD1; MIM 311200) is characterized by multiple anomalies of the oral cavity, face and digits. We report a family with OFD1, where two female siblings and their ...mother shared the same mutation of the responsible gene (OFD1) c.1193_1196delAATC. Phenotypic variability was observed among them; the mother showed minimal features of OFD1, whereas her two daughters showed partial features and the full spectrum of OFD1, respectively. Thus, OFD1 was suspected only after a health check‐up during pregnancy of the second patient showing fetal brain anomaly and maternal polycystic kidney. For these reasons, there was a delay in the recognition of OFD1 in this family. Patients with OFD1 show phenotypic variability, which poses challenges for genetic counseling.
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