Increasing Trend of Calretinin-Positive Mucosal Innervation from Aganglionic Zone toward Transition Zone in Hirschsprung's Disease.

INTRODUCTION: Diagnosis of very short-segment Hirschsprung's disease (vsHD) by rectal suction biopsy is challenging as its aganglionic zone (AZ) overlaps with physiologic hypoganglionic zone and calretinin-positive mucosal nerves may extend from the transition zone (TZ) into AZ. We studied whether an increasing trend/gradient of calretinin-positive mucosal nerves along the distance from AZ toward TZ aids in diagnosis of HD. MATERIALS AND METHODS: In this study, 46 rectal suction biopsies from non-HD and HD, and 15 pull-through specimens from short-segment HD were evaluated by mucosal calretinin immunostain (CI) and image processing and analysis (IPA) to measure pixel count (PC, the percentage of calretinin stained pixels in the mucosa). Consecutive longitudinal sections of proximal AZ toward distal TZ in HD pull-through specimens were utilized as a vsHD surrogate model. First, we studied variability of mucosal CI in non-HD biopsies along the distance from dentate line. Second, we determined a cutoff point of mucosal CI by IPA that separated non-HD versus HD and applied this cutoff to longitudinal sections from proximal AZ to distal TZ segments in HD pull-through specimens. Third, we studied whether an increasing trend of mucosal CI was universally observed in HD pull-through. RESULTS: Our findings included a significant variability in PC along the biopsy distance in non-HD cases. Positive mucosal CI was found in proximal AZ in 6 (43%) of 14 HD pull-through, among which 1 case lacked submucosal nerve hypertrophy in the proximal AZ. All 14 HD pull-through cases showed an increasing trend/gradient of PC from AZ toward TZ. CONCLUSION: Based on our findings, the presence or absence of mucosal CI positivity and submucosal nerve hypertrophy may not reliably diagnose vsHD in rectal suction biopsy. While we acknowledge that the density of mucosal innervation in variable contexts and anatomical locations is unknown and yet to be explored, our study suggests that an increasing trend of positive mucosal CI from AZ toward TZ by IPA might prove to be a useful tool for the diagnosis of vsHD in the future.

Transition Zone in Total Colonic Aganglionosis and Colorectal Hirschsprung's Disease Shows a Similar Trend of Mucosal Innervation: Image Processing and Analysis Study.

The aganglionic segment of bowel in Hirschsprung's disease (HD) varies in length. It is not clear whether total colonic aganglionosis (TCA) merely represents a long form of HD or a different phenotype of the disease. Animal model studies suggest that TCA may have a longer transition zone (TZ) than conventional colorectal HD. We compared mucosal innervation of TZ in 2 TCA cases and 10 conventional colorectal HD cases by quantifying calretinin-positive mucosal nerve fibers using image processing and analysis. One TCA was associated with esophageal atresia-tracheoesophageal fistula, the other with trisomy 21. The gradients of calretinin-stained pixel count increase per distance from the beginning of TZ (slope) for TCA were not significantly different from those for the conventional HD group. Given this observation, it is speculated that the length of TZ in TCA may fall within the range of and may not be much longer than conventional colorectal HD.

Image Processing and Analysis of Mucosal Calretinin Staining to Define the Transition Zone in Hirschsprung Disease: A Pilot Study.

PURPOSE: Quantification of calretinin-stained mucosal nerve fibers by image processing and analysis (IPA) may objectively define the transition zone (TZ) of Hirschsprung disease (HD). We tested the utility of IPA as an adjunctive tool in HD. MATERIALS AND METHODS: Calretinin immunostain was performed on 15 HD pull-through specimens, and multiple images were captured from the proximal aganglionic zone, TZ, and probable normal zone (NZ). Pixel count (PC), defined as the percentage of brown-stained pixels in the mucosa, was quantified and plotted against distance from the rectal distal end. To validate the method, PCs from 45 images were compared with three-tiered visual scoring by five pathologists. Results were correlated against pertinent variables, which were retrieved from the clinical record. RESULTS: The PC gradually increased in the TZ toward the proximal resection margin in 10/13 (77%) cases. The PC variation in the probable NZ and around the circumference was substantial by the coefficient of variation. The mean PC of images with a visual score of 1 was less than scores of 2 and 3 by all five (100%) pathologists (p < 0.01). One patient had possible TZ pull-through that was clinically confirmed. CONCLUSION: While the mucosal calretinin staining gradually increases in the TZ, for now, the boundaries of the TZ cannot be accurately defined by mucosal biopsies given the substantial variation of staining around the circumference at the same distance and in the NZ. However, the IPA technique does provide a continuous variable and warrants further utility in HD studies.

Long term outcome of the Bascom cleft lift procedure for adolescent pilonidal sinus.

PURPOSE: The purpose of this study was to assess whether adolescent patients who heal their pilonidal sinus disease with the Bascom cleft lift have a sustained result, or if younger age puts them at increased risk of relapse. METHODS: Twenty-two consecutive patients (18 male, median age 16years at surgery) who underwent Bascom procedures were contacted regarding long term outcomes following surgery. Additional clinical data were collected from the medical records of the subjects. RESULTS: Seventeen of 22 (77%) patients studied were completely healed at median 27 (range 9-268) days after a single procedure. Three of 22 (14%) required a second procedure during follow up. Of these, all three healed fully after the second procedure. Based on intention to treat, 20 of 22 (91%) patients had complete healing documented at a median of 31days after initial surgery. Half of the patients were fully healed and back to normal activities by one month and 68% by two months. Two patients (9%) failed surgery and had ongoing pilonidal disease. Fifteen of 22 (68%) patients completed a follow-up survey at a median of 44months elapsed from surgery. All responders were fully healed, and none reported relapse or further surgeries. CONCLUSIONS: Excision of pilonidal sinus disease by the Bascom procedure resulted in lasting cure for most adolescent patients studied. A small proportion of patients failed to heal in the first year and had ongoing pilonidal disease. No late failures occurred. LEVEL OF EVIDENCE: Level III, nonexperimental observational study.

Surgical site infections in the NICU.

BACKGROUND: Surgical site infections (SSI) increase morbidity and mortality. In adult and pediatric populations, the incidence ranges from 1.5-12%. Studies in neonates have shown an association between preoperative stay in an intensive care unit and development of SSI. To date, there has only been a single study looking exclusively at SSI in the Neonatal Intensive Care Unit (NICU). Additionally, there has been a suggestion that prematurity may be a risk factor for SSI, but this has come from studies looking at all neonates less than 28days, rather than only neonates hospitalized in a NICU. OBJECTIVE: Primary outcome variable was to calculate the incidence of SSI in a NICU population. Secondary outcome variables were to determine if SSI is more common in premature infants and to identify additional risk factors for the development of SSI. METHODS: An IRB-approved retrospective chart review of all patients undergoing surgical procedures in a level IIIC NICU over a 2-year period was used. We utilized the CDC's definitions of surgical procedures and SSI. An epidemiologist reviewed charts if the diagnosis of SSI was questionable. Statistical analysis was done with t test and Fisher's exact test. RESULTS: We identified 165 patients who underwent 264 surgical procedures. Incidence of SSI was 11.7%. There were 31 SSI that occurred in 29 neonates over the 2-year period, with no mortality in that group. In patients who developed an SSI, 34.5% occurred after the 1st procedure, 41.4% occurred after a 2nd procedure, and 24.1% occurred after the 3rd or later procedure. There was no difference in perioperative antibiotic usage. CONCLUSIONS: This study describes SSI in a strictly neonatal population in a large academic NICU. Prematurity does not appear to be a risk factor for SSI. SSI is more common in neonates who have undergone an abdominal procedure or multiple procedures. Perioperative antibiotics are not significantly associated with prevention of SSI.

Anoplasty for low anorectal malformation.

BACKGROUND: The presence of a slightly anterior, stenotic anus is a low anorectal malformation. It is a type of rectoperineal fistula. The cardinal symptoms are constipation and straining at stool. Hendren described a technically simple anoplasty for this condition in 1978. Controversy about terminology has led to a lack of clarity about this condition. METHODS: Clinical data were reviewed about the authors' patients, who had Hendren anoplasty, from 2009 to 2015. Followup data were obtained from office visits and telephone interviews with families and primary care doctors. RESULTS: Seven patients (4 boys) presented with intractable constipation. All had a stenotic anal orifice, located anterior to the center of the anal wink, within the pigmented perianal skin. In 4 of 7 cases, the lesion was not recognized at birth. Anoplasty was performed at a median age of 8months (range 6-28). Late followup information was obtained on six of the seven patients at a median of 32months (range 28-61). Four reported no or minimal laxative requirement and two reported daily laxative use but good symptom control. CONCLUSIONS: Low anorectal malformation/rectoperineal fistula may be overlooked in the newborn. When symptomatic, it may be corrected by a simple anoplasty with excellent results.

Robotic Single- and Multisite Cholecystectomy in Children.

BACKGROUND: Elective cholecystectomy is a high-volume, simple procedure, well suited for the development of a pediatric robotic surgery program. Surgical robot software, by "reversing" the surgeon's hands, simplifies single-site cholecystectomy through a single incision at the umbilicus. MATERIALS AND METHODS: Data were reviewed on the first nine children who had robotic cholecystectomy, with the Da Vinci® Surgical System (Intuitive Surgical Inc., Sunnyvale, CA) device, at our institution. All cases were performed by a single surgeon, proctored by an experienced robotic general surgeon. RESULTS: There were 9 patients: the first 4 patients had robotic multiport cholecystectomy, and the last 5 had robotic single-site cholecystectomy. Eight were girls. They were 10-18 years of age (median, 14 years). Diagnoses were biliary dyskinesia (n = 5) and symptomatic cholelithiasis (n = 4). Median body mass index was 24.9 (range, 20.2-43.8) kg/m(2). Median anesthesia time for multiport cholecystectomy was 139 (range, 120-162) minutes; median anesthesia time for single-site cholecystectomy was 169 (range 122-180) minutes. Median console time for multiport cholecystectomy was 47 (range, 44-58) minutes; median console time for single-site cholecystectomy was 69 (range, 66-86) minutes. Eight of the 9 patients went home on the day of surgery, and 1 stayed overnight. Patients were seen at 13-20 days after surgery (median, 14 days). There were no complications. There were no conversions to open surgery and none from single-site to multiport surgery. CONCLUSIONS: Robotic cholecystectomy is safe and efficacious and is a suitable introductory procedure for pediatric surgeons considering a robotic surgery program. Single-site robotic cholecystectomy is a cosmetically attractive option but takes longer than multiport robotic cholecystectomy.

Laparoscopic-assisted percutaneous cecostomy for antegrade continence enema.

PURPOSE: The antegrade continence enema (ACE) is an option in the management of fecal incontinence and chronic constipation. We report our experience with a simple laparoscopic technique. SUBJECTS AND METHODS: Data were collected on 16 children (8 boys) who underwent laparoscopic cecostomy for ACE. Success was defined as cessation of fecal soiling with no need for diapers. RESULTS: Mean age at laparoscopic cecostomy was 11 years (range, 6-16 years). Mean follow-up after initial cecostomy was 22 months (range, 6-51 months). Diagnoses in 16 patients were functional constipation with soiling (n=14), incontinence after surgery for Hirschsprung's disease (n=1), and constipation secondary to mitochondrial disease (n=1). Seven had significant developmental or psychiatric problems. Three patients had primary placement of a trapdoor device (Chait); 13 had placement of a long tube, with later replacement by a skin-level device. We have evolved a laparoscopic-assisted percutaneous technique, using metallic anchor sutures on the cecum, and a dilator and peel-away sheath for introduction of the catheter. Complications occurred in 5 patients; 3 returned to the operating room: 1 for tube occlusion, 1 for suture granuloma, and 1 for a dislodged tube at 7 months postoperatively. One patient received intravenous antibiotics because of suspected peritonitis on the first postoperative day. One was re-admitted with abdominal pain. Five of 16 patients have failed therapy (four tubes removed and one tube in situ). Three have had only minor improvement. Eight have had successful ACE management, of whom 1 patient has had his tube removed after resolution of symptoms. Of 8 patients with no or minimal improvement with ACE, 5 have significant psychiatric problems. CONCLUSIONS: Laparoscopic-assisted percutaneous cecostomy has an excellent safety profile and patient comfort. The procedure is simple, secure, and reversible. Results were excellent in half of the patients. Associated psychiatric or behavioral problems may predict poor response to ACE.

Outpatient interval appendectomy after perforated appendicitis.

BACKGROUND: Interval appendectomy may be advisable after successful nonoperative treatment of perforated appendicitis. To reduce the perceived morbidity of interval appendectomy, we sought to determine if the operation could be done on an outpatient basis. This study is focused on patient comfort and safety after laparoscopic interval appendectomy (LIA). METHODS: This is a retrospective review of the clinical course and length of stay of 24 children who had LIA during a 4-year period. RESULTS: Of the 24 patients, 12 were discharged on the evening of surgery without incident. Nine additional patients were observed for the first postoperative night-2 for short episodes of temperature elevation, 3 for pain treated within the first 4 hours of recovery and requiring no further treatment, and 4 because the idea of outpatient appendectomy had yet to become popular. None of these patients was febrile overnight, none required narcotic or parenteral analgesics after leaving the recovery room, and all accepted feedings without nausea or vomiting. It is likely that all 9 of these patients could have been discharged on the day of operation. Three other patients stayed in the hospital for treatment of pain, low-grade fever, or slow resumption of feeding. CONCLUSION: Of 24 patients, 21(88%) were or could have been discharged on the day of operation. When interval appendectomy is indicated, LIA can be performed safely as an outpatient surgical procedure in most children.

Early decisions in perforated appendicitis in children: lessons from a study of nonoperative management.

BACKGROUND: In nonoperative management of perforated appendicitis, some children do not respond to treatment. This study sought early identifiers of failure to help in surgical decision making. METHODS: Fifty-eight patients with computed tomographic (CT)-proven perforated appendicitis were treated according to a nonoperative protocol. Patients who recovered were considered "successes;" those who did not improve underwent appendectomy and were scored as "failures" of nonoperative treatment. RESULTS: Thirty-six (62%) of 58 patients responded to treatment and 22 (38%) failed. Three parameters distinguished the 2 groups: the number of band forms on the admission white blood cell count, the body temperature response after 24 hours of treatment, and the areas of the abdomen involved in the CT scan. Patients in whom nonoperative treatment failed stayed in the hospital longer (17 vs 9 days) and had more complications (46% vs 0%). CONCLUSIONS: Because failure of nonoperative management is associated with a high complication rate, it is important to make an early decision about appendectomy. Persistence of fever after 24 hours of treatment, bandemia on admission, and multisector involvement on CT scan identify most patients who fail nonoperative management. When combined with clinical judgment, these are useful indicators to guide early decisions.

Laparoscopic excision of a gastric diverticulum in a child.

Gastric diverticulum is a rare entity; the patient usually presents with vague abdominal pain, nausea, vomiting, and weight loss. Diverticulectomy is required for patients with intractable symptoms or complications. We report the laparoscopic excision of a gastric diverticulum in a 15-year-old girl who had been symptomatic for 5 years. A five-port technique and a linear cutting and stapling device were used.

Nonoperative management of perforated appendicitis in children: can CT predict outcome?

BACKGROUND: The optimal treatment of perforated appendicitis remains controversial, but there is a trend toward nonoperative management. CT scanning might be helpful in determining which patients could benefit from this treatment option. OBJECTIVE: To determine the value of CT imaging in predicting clinical success or failure in children with nonoperative management of perforated appendicitis. MATERIALS AND METHODS: Admission CT scans of 34 children with perforated appendicitis treated nonoperatively between January 2003 and June 2006 were retrospectively reviewed. All children were given intravenous antibiotics. Clinical outcome was correlated with imaging findings including the maximal area, number and complexity of collections, presence of an appendicolith or extraluminal air, and extent of intra-abdominal disease outside the right lower quadrant. Patients with an accessible simple collection were drained if their clinical condition did not improve. RESULTS: Successful nonoperative management was achieved in 20 patients; 14 patients failed nonoperative therapy. The presence of collections in three or more sectors (defined as the pelvis and four abdominal quadrants) correlated strongly with clinical failure (P < 0.05), while there was no correlation found between clinical outcome and the presence of an appendicolith, extraluminal air, distant ascites, and collection size or complexity. CONCLUSION: In the nonoperative management of children with perforated appendicitis, admission CT findings demonstrating disease beyond the right lower quadrant correlate with treatment failure.

Nontraumatic compartment syndrome of the extremities in children.

Acute compartment syndrome of an extremity poses a threat to both life and limb. The usual cause in children is trauma, especially fractures and burns. Two recent cases of nontraumatic compartment syndrome of the extremities in children are presented, both owing to infection followed by myositis or fasciitis. Both cases required urgent decompression by fasciotomy, resulting in limb salvage and full recovery of neuromuscular function of the extremity. The value of prompt recognition and treatment of this condition is emphasized.