Pancreatic surgery for tumors in children and adolescents

Purpose

Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies.

Methods

We conducted a retrospective review of patients ≤21 years with pancreatic neoplasms who underwent surgery at a single institution between 1995 and 2015.

Results

Nineteen patients were identified with a median age at operation of 16.6 years (IQR 13.5–18.9). The most common histology was solid pseudopapillary neoplasm (SPN) (n = 13), followed by pancreatic neuroendocrine tumor (n = 3), serous cystadenoma (n = 2) and pancreatoblastoma (n = 1). Operative procedures included formal pancreatectomy (n = 17), enucleation (n = 1) and central pancreatectomy (n = 1). SPNs were noninvasive in all but one case with perineural, vascular and lymph node involvement. Seventeen patients (89.5 %) are currently alive and disease free at a median follow-up of 5.7 (IQR 3.7–10.9) years. Two patients died: one with metastatic insulinoma and another with SPN who developed peritoneal carcinomatosis secondary to a concurrent rectal adenocarcinoma.

Conclusions

Pediatric pancreatic tumors are a heterogeneous group of neoplastic lesions for which surgery can be curative. SPN is the most common histology, is characterized by low malignant potential and in selected cases can be safely and effectively treated with a tissue-sparing resection and minimally invasive approach.

     

Citation classics in pediatrics: a bibliometric analysis

Background

Citation analysis provides insights into the history and developmental trajectory of scientific fields. Our objective was to perform an analysis of citation classics in the journals of pediatric specialty and to examine their characteristics.

Methods

Initially, all the journals listed under the category of pediatrics (n = 120) were identified using Journal Citation Reports. Web of science database was then searched (1950–2016) to select the top-100 cited articles in the above identified pediatric journals. The top-100 cited article were categorized according the study design, sub-specialty, country, institutional affiliation, and language.

Results

The top-100 articles were published in 18 different journals, with Pediatrics having the highest numbers (n = 40), followed by The Journal of Pediatrics (n = 17). The majority (n = 62) of classics were published after 1990. The most cited article had citation count of 3516 and the least cited had a citation count of 593. The USA (n = 71) was the most commonly represented country, and 60 institutions contributed to 100 articles. Fifteen authors contributed to more than one classic as first or second author. Observational study (n = 55) was the commonest study design across all decades, followed by reviews (n = 12), scale development studies (n = 11), and guidelines (n = 11). Among the pediatric sub-specialties, growth and development articles were highly cited (n = 24), followed by pediatric psychiatry and behavior (n = 21), endocrinology (n = 15), and neonatology (n = 12).

Conclusions

The top-100 cited articles in pediatrics identify the impactful authors, journals, institutes, and countries. Observational study design was predominant—implying that inclusion among citation classics is not related to soundness of study design.

     

Profile of Pediatric Idiopathic Inflammatory Myopathies from a Tertiary Care Center of Eastern India

Objectives

To report data on Idiopathic inflammatory myopathies (IIM) from eastern India.

Methods

All IIM patients diagnosed over the last 5 y (2011–2016) were included through a retrospective review of records from the hospital and specialty clinic at Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata.

Results

Out of the 11 IIM patients, 9 had Juvenile dermatomyositis (JDM) and 2 had overlap myositis (OM) [with systemic lupus erythematosus (SLE) and scleroderma]. The overall sex ratio was M: F = 1: 2.6. The mean age at diagnosis was 6.94 y for JDM and 7 y for OM. The mean interval from onset to diagnosis was 5.2 mo. All patients had heliotrope rash and proximal myopathy (n = 11,100%). Other findings included Gottron papule (n = 7; 64%), arthritis (n = 6; 54%), malar rash (n = 5; 45%), dysphagia (n = 4; 36%), nasal intonation (n = 3; 27%), subcutaneous nodule (n = 2; 18%), cutaneous sinus (n = 1; 9%), calcinosis universalis (n = 1; 9%), GI bleed (n = 1; 9%). All patients had raised erythrocyte sedimentation rate (ESR), aspartate aminotransferase (AST), lactate dehydrogenase (LDH) while 10 had raised creatine kinase (CK). Three were anti-nuclear-antibody (ANA) positive. Electromyography (EMG) showed proximal myopathy in most cases (n = 9; 82%). All patients received corticosteroid. Intravenous immunoglobulin (IVIG) was given to 2 patients. Two received hydroxychloroquine. Cyclophosphamide and azathioprine were given in one each.

Conclusions

This study, first reported profile of IIM from eastern India, showed JDM as the commonest form of IIM with a female preponderance. Five children had complete and 2 had partial remission. Two patients of JDM and 1 of OM died. Increased awareness, early referral, prompt diagnosis and treatment might improve the outcome and survival.

     

The impact of surgical strategies on outcomes for pediatric chronic pancreatitis

Purpose

To review our institutional experience in the surgical treatment of pediatric chronic pancreatitis (CP) and evaluate predictors of long-term pain relief.

Methods

Outcomes of patients ≤21 years surgically treated for CP in a single institution from 1995 to 2014 were evaluated.

Results

Twenty patients underwent surgery for CP at a median of 16.6 years (IQR 10.7–20.6 years). The most common etiology was pancreas divisum (n = 7; 35%). Therapeutic endoscopy was the first-line treatment in 17 cases (85%). Surgical procedures included: longitudinal pancreaticojejunostomy (n = 4, 20%), pancreatectomy (n = 9, 45%), total pancreatectomy with islet autotransplantation (n = 2; 10%), sphincteroplasty (n = 2, 10%) and pseudocyst drainage (n = 3, 15%). At a median follow-up of 5.3 years (IQR 4.2–5.3), twelve patients (63.2%) were pain free and five (26.3%) were insulin dependent. In univariate analysis, previous surgical procedure or >5 endoscopic treatments were associated with a lower likelihood of pain relief (OR 0.06; 95% CI 0.006–0.57; OR 0.07; 95%, CI 0.01–0.89). However, these associations were not present in multivariate analysis.

Conclusion

In children with CP, the step-up practice including a limited trial of endoscopic interventions followed by surgery tailored to anatomical abnormalities and gene mutation status is effective in ensuring long-term pain relief and preserving pancreatic function.

     

Correction of penile ventral curvature in patients with minor or no hypospadias: a single surgeon’s experience of 43 cases

Purpose

To report our experience of correcting penile ventral curvature associated with minor or no hypospadias.

Methods

We reviewed 43 penile ventral curvature patients treated by a single surgeon from 1997 to 2015. Of these, 23 had minor hypospadias. Curvature was corrected using degloving, chordectomy, dorsal plication (DP), tunica albuginea incision (TAI), or a combination of these. Outcome was confirmed by induced artificial erection and post-operative appearance.

Results

Mean age at curvature correction was 3.2 ± 2.6 years. 17/43 had degloving and chordectomy (DC), 16/43 had DP after DC, and 10/43 had TAI after DC, because of ventral shortening and severe curvature caused by a short hypoplastic urethra. Other procedures required were primary meatoplasty (n = 4) and urethroplasty (UP; n = 1) at the time of curvature correction, and UP after correction of curvature (n = 11). Complications included recurrence of curvature after DP (n = 3/16; 18.8 %) and urethral stenosis after UP with tubed peritoneum (n = 1/10; 10 %). There were no recurrences of curvature in TAI cases. Parents reported penile cosmesis as good (n = 38; 88.4 %), acceptable (n = 4; 9.3 %), or poor (n = 1; 2.3 %).

Conclusion

We recommend TAI followed by UP for correcting penile ventral curvature with short hypoplastic urethra. Tubed peritoneum is not recommended for UP.

     

Improved outcomes after technical modifications in tubularized incised plate urethroplasty for mid-shaft and proximal hypospadias

Purpose

To investigate and compare the outcomes after tubularized incised plate (TIP) urethroplasty in mid-shaft and proximal hypospadias using a standard and a modified technique.

Methods

We conducted a retrospective study in 104 consecutive children who underwent mid-shaft or proximal TIP repairs from Jan 2007 to Sept 2015. Patients in Cohort One had dorsal dartos (DD) neourethral coverage while patients in Cohort Two had either de-epithelialized split preputial (DESP) or tunica vaginalis (TV) flap coverage. TV flap was used only when DESP flap was not sufficient to cover the neourethra.

Results

There were 52 patients each in Cohort One (DD, n = 52) and Cohort Two (DESP, n = 38; TV, n = 14) with no difference in ratio of mid-shaft/proximal between the two cohorts. At a median follow-up of 28 months, 36 patients (34.6 %) developed 47 complications including fistula (n = 19; 18.3 %) and neourethral dehiscence (n = 4; 3.8 %). Cohort One patients had significantly more fistula (28.8 vs 7.7 %; p = 0.005) and neourethral dehiscence (7.7 vs 0 %; p = 0.04) than Cohort Two. There was no difference between the two cohorts in the complication rates of meatal stenosis, recurrent ventral curvature and neourethral stricture.

Conclusions

Both DESP and TV flap appear to be superior to DD in preventing fistula and neourethral dehiscence in non-distal TIP repairs.

     

Fishtail deformity of the distal humerus: association with osteochondritis dissecans of the capitellum

Background

Fishtail deformity is a rare deformity of the humerus exhibiting concavity of the lateral trochlea, resulting in ulnotrochlear joint derangement. We wanted to share our experience that osteochondritis dissecans of the capitellum is a common associated complication.

Objective

To summarize imaging of fishtail deformity in children centered on complications of the radiocapitellar joint.

Materials and methods

From the radiology information system, we identified all patients <18 years with the diagnosis of fishtail deformity. We included only patients with V-shaped deformity of the distal humerus due to concavity at the lateral trochlea (fishtail deformity). Each patient’s initial injury, most recent radiograph and available MRI were evaluated for radiocapitellar joint derangement.

Results

Seven patients (4 males) with a mean age of 12.9 years (range: 9.7 to 14.4 years) were identified. Radiocapitellar joint abnormalities were identified in six patients including osteoarthritis (n=5), flattened and sclerotic capitellum (n=4), osteochondritis dissecans (2 associated with loose body, n=4) and radial head subluxation (n=2). In 4 patients, MRI detected changes of osteoarthritis (n=4), osteochondritis dissecans (n=2) and loose body (n=1) not identified on radiography. Two patients with osteochondritis dissecans underwent surgery and one patient has planned surgery.

Conclusion

Radiocapitellar joint abnormalities (particularly, capitellar osteochondritis dissecans) are common in patients with fishtail deformities. MRI should be performed in these patients since some abnormalities, possibly requiring surgery, are not detected on elbow radiographs.

     

MR elastography: high rate of technical success in pediatric and young adult patients

Background

Magnetic resonance (MR) elastography allows the noninvasive assessment of liver stiffness, which is a surrogate for fibrosis.

Objective

The purpose of this study was to describe our experience using liver MR elastography in a large pediatric population with attention to the frequency and causes of exam failure.

Materials and methods

Imaging records were searched for patients ≤18 years of age who underwent 2-D gradient recalled echo (GRE) MR elastography of the liver between September 2011 and August 2015 on one of two 1.5-T MRI platforms. Imaging reports and clinical records were reviewed for failed MR elastography acquisitions, factor(s) resulting in failure and whether a subsequent successful examination had been performed.

Results

Four hundred sixty-eight MR elastography examinations were performed in 372 patients between 1.5 months and 18 years of age during the study period. Ninety-six percent (450/468) of the examinations were successful. There was no significant difference in mean age (12.6±3.6 vs. 11.2±4.1 years, P=0.12) or body mass index (BMI) (28.2±12.4 vs. 29.5±10 kg/m², P=0.6) between patients with and without successful examinations. MR elastography failures were due to poor paddle positioning resulting in inadequate generation of hepatic shear waves (n=5), iron overload (n=4), patient inability to tolerate MRI (n=3), patient breathing/motion (n=3), artifact from implanted hardware (n=1) and technical malfunction (n=2). Seven of nine (78%) repeat examinations were successful (78%).

Conclusion

Hepatic 2-D GRE MR elastography at 1.5 T is technically robust in children. Exam failure is infrequent and largely reflects patient specific factors, some of which can be mitigated with careful technique.

     

Image-guided placement of percutaneous de novo low-profile gastrojejunostomy tubes in the pediatric population: a study of feasibility and efficacy

Background

De novo low-profile gastrojejunostomy tubes in pediatric patients offer less external catheter bulk and decreased propensity for dislodgement as children become more mobile. While small cohort studies have evaluated de novo placement of coaxial, adjustable-length, percutaneous gastrojejunostomy (GJ) tubes in children, placement of de novo low-profile GJ tubes in pediatric patients has not been analyzed.

Objective

This study evaluates technical feasibility, safety and clinical efficacy of percutaneous, retrograde placement of de novo low-profile GJ tubes in infants and children.

Materials and methods

Following institutional review board approval, all de novo low-profile GJ tube placements in patients were retrospectively reviewed between May 2014 and May 2017. Technical parameters of fluoroscopy time, tube size, T-fasteners and complications were recorded. Clinical data, including age, indication, weight gain and complications, were analyzed.

Results

Thirty-four de novo low-profile GJ tubes were placed in 34 patients (median age: 9.4 months, range: 2 months-11.8 years; median pre-procedural weight: 7.5 kg, range: 2.9-31.6 kg). Twenty-one 14-Fr and 13 16-Fr GJ tubes were placed with technical success rate of 100%. Average weight gain 3 months’ post procedure was 1.1 kg (range: 0.3-4.8 kg) and average weight percentile for age increase was 9.6% (range: -48.9% to 53.5%). One major complication occurred following balloon inflation within the tract causing pain requiring urgent replacement of the GJ tube. Minor complications occurred in 11 patients (32%): accidental dislodgement (n=9), skin irritation (n=4), tube dysfunction (n=2), leakage (n=2) and tube migration into the esophagus (n=1).

Conclusion

Percutaneous, antegrade, image-guided placement of de novo low-profile GJ tubes is technically feasible, safe and clinically efficacious in appropriately selected pediatric patients.

     

Surgical intervention for congenital pulmonary airway malformation (CPAM) patients with preoperative pneumonia and abscess formation: “open versus thoracoscopic lobectomy”

Aim

Thoracoscopic lobectomy (TL) and open lobectomy (OL) were compared for treating congenital pulmonary airway malformation (CPAM) with preoperative complications, specifically pneumonia/abscess formation (PA).

Methods

The medical records of 46 CPAM patients treated by lobectomy at our institution from 1990 to 2014 were reviewed retrospectively. Four groups, TL for patients without PA (n = 17; TL?), TL for patients with PA (n = 8; TL+), OL for patients without PA (n = 16; OL?), and OL for patients with PA (n = 5; OL+) were compared for operative time, intra/postoperative complications, blood loss, duration of chest tube insertion, postoperative analgesia, pre: postoperative white blood cell (WBC) ratio, and duration of hospitalization.

Results

Operative time for TL+ was longest, but not statistically significant. Incidences of intra/postoperative complications were similar in all groups. Blood loss was significantly less for TL+ versus OL+ (p < .05). WBC ratio was significantly lower in TL+ versus OL+ (p < .05), similar for TL+ and TL?, and significantly higher in OL+ versus OL? (p < .01). Chest tube insertion was significantly longer in OL? versus TL? (p < .01).

Conclusion

PA would not appear to be a contraindication to perform TL in CPAM. TL is associated with less surgical stress than OL despite longer operative time.

     

Outcomes following sclerotherapy for mucosal rectal prolapse with oily phenol injection: single-centre review

Aim of the study

To review the outcomes of injection sclerotherapy with oily phenol for mucosal rectal prolapse.

Methods

Retrospective case note review of all children who underwent sclerotherapy with oily phenol injection as primary surgical intervention for mucosal rectal prolapse, from January 2007 to December 2015.

Main results

A total of 31 patients were identified. Mean age at presentation was 4.8 years (range 5 months–12 years). 23 patients with mucosal rectal prolapse underwent injection sclerotherapy with oily phenol as primary procedure. Patients with full-thickness rectal prolapse (n = 6) and 2 with mucosal prolapse who had Thiersch stitch were excluded from the study. The cause for mucosal rectal prolapse was considered to be due to constipation (n = 15), idiopathic (n = 7), spina bifida (n = 1). Follow-up was for minimum 6 months (median = 4 years; range 6 months–17 years). Recurrence following injection sclerotherapy with oily phenol requiring further procedures was 30.4% (7/23).

Conclusions

Injection sclerotherapy with oily phenol is a safe, effective and minimally invasive primary treatment option for mucosal rectal prolapse not responding to conservative management. In case of recurrence, a cautious re-examination under anaesthesia should be undertaken to exclude a missed full-thickness rectal prolapse before reinjecting.

     

Indications and outcomes for tunneled central venous line placement via the axillary vein in children

Purpose

To assess the indications, safety and outcomes of tunneled central venous catheters (CVCs) placed via a cutdown approach into the axillary vein in children, an approach not well described in this population.

Methods

A retrospective cohort study was performed on pediatric patients who received CVCs via open cannulation of the axillary vein or one of its tributaries between January 2006 and October 2016 at two hospitals.

Results

A total of 24 axillary CVCs were placed in 20 patients [10 male (42%); mean weight 7.0 kg (SD 2.9); mean age 10 months (SD 6)]. The most common indications for axillary vein access included neck or chest wall challenges (tracheostomies or chest wall wounds) (n = 18). The median duration of line placement was 140 days (IQR 146). The most common indications for removal were completion of therapy (n = 7, 39%) and infection (n = 5, 28%). There were no early complications. Long-term complications included infection (n = 5) or catheter malfunction (n = 3).

Conclusions

Tunneled CVC placement via a cutdown approach into the axillary vein or its tributary can be an effective alternative approach to obtain long-term vascular access in children. Outcomes may be comparable to lines placed in traditional internal jugular and subclavian vein locations.

     

Comprehensive assessment of prognosis after laparoscopic portoenterostomy for biliary atresia

Purpose

Total bilirubin (T-bil) is used universally for monitoring post-portoenterostomy (PE) biliary atresia (BA) patients although other biochemical markers [BM; AST/ALT and platelet count (PC)] are also prognostic. We compared open PE (OPE) with laparoscopic PE (LPE) using T-bil, AST/ALT, and PC (3BM) as more comprehensive indicators of postoperative clinical status.

Methods

Subjects were 31 PE cases (LPE: n = 17; OPE: n = 14). BA classification was type III (n = 16), type II (n = 1) in LPE and type III (n = 12), type I (n = 1), type II (n = 1) in OPE.

Results

Mean ages and weights at PE were similar: 65.5 days, 4.4 kg (LPE) versus 69.3 days, 4.1 kg (OPE); and mean follow-up was 2.5 years for both LPE and OPE. Jaundice clearance (T-bil ≤1.2 mg/dL) was achieved in 16/17 (94.1 %) after LPE versus 10/14 (71.4 %) after OPE (p = NS), but 3BM were closer to normal after OPE. At the time of review, 13/17 LPE cases (76.5 %) were alive with native livers and 4/17 had received LTx (23.5 %) and 10/14 OPE cases (71.4 %) were alive with native livers and 4/14 had received LTx (28.6 %).

Conclusions

Although JC was better after LPE, 3BM were better after OPE. Further follow-up will prove the comprehensive prognostic value of 3BM.

     

Pigeon chest: comparative analysis of surgical techniques in minimal access repair of pectus carinatum (MARPC)

Background

After minimally invasive repair for pectus excavatum (MIRPE), similar procedures for pectus carinatum were developed. This study aimed to analyse the various published techniques of minimal access repair for pectus carinatum (MARPC) and compare the outcomes.

Data sources

Literature was reviewed on PubMed with the terms “pectus carinatum”, “minimal access repair”, “thoracoscopy” and “children”.

Results

Twelve MARPC techniques that included 13 articles and 140 patients with mean age 15.46 years met the inclusion criteria. Success rate of corrections was n = 125, about 89% in cumulative reports, with seven articles reporting 100%. The complication rate was 39.28%. Since the pectus bar is placed over the sternum and has a large contact area, skin irritation was the most frequent morbidity (n = 20, 14.28%). However, within the complication group (n = 55), wire breakage (n = 21, 38.18%) and bar displacement (n = 10, 18.18%) were the most frequent complications. Twenty-two (15.71%) patients required a second procedure. Recurrences have been reported in four of twelve techniques. There were no lethal outcomes.

Conclusions

MARPC techniques are not standardized, as MIRPE are, so comparative analysis is difficult as the only common denominator is minimal access. Surgical morbidity is high in MARPC and affects > 2/3rd patients with about 15% requiring surgery for complication management.

     

Staged segmental urethroplasty for scrotal/perineal hypospadias: a new concept

Purpose

We report the efficacy of staged segmental urethroplasty (SSUP) versus non-staged urethroplasty (NSUP) for treating scrotal/perineal hypospadias (SPH).

Methods

Between 1997 and 2015, 29 SPH patients underwent UP (SSUP: n = 15; NSUP: n = 14). Incidences of urethrocutaneous fistula (UF), stenosis of the neourethra (SNU), diverticula formation, and residual chordee (RC) were compared. Differences were statistically significant if p < 0.05.

Results

The difference in mean age at NSUP (3.2 ± 1.3 years) and at the final stage of SSUP (5.5 ± 2.4 years) was significant (p < 0.05). Mean operative times for NSUP and SSUP (total for all stages) were not significantly different (231.5 ± 117.5 versus 272.5 ± 99.4 min); however, the incidence of postoperative complications was significantly less in SSUP (n = 1; UF) compared with NSUP (n = 6; 2 cases of UF, 3 cases of SNU, and 1 case of RC; (p < 0.05). Mean follow-up was significantly shorter in SSUP; 1.4 ± 1.2 years versus 7.0 ± 4.5 years in NSUP (p < 0.05).

Conclusion

SSUP would appear to be effective for treating SPH because of a significantly lower incidence of UF, SNU and RC during the first postoperative year, the period when complications have been reported to arise most frequently.

     

Fanconi anemia: correlating central nervous system malformations and genetic complementation groups

Background

Congenital central nervous system abnormalities in children with Fanconi anemia are poorly characterized, especially with regard to specific genetic complementation groups.

Objective

To characterize the impact of genetic complementation groups on central nervous system anatomy.

Materials and methods

Through chart review we identified 36 patients with Fanconi anemia with available brain MRIs at the University of Minnesota (average age, 11.3 years; range, 1–43 years; M:F=19:17), which we reviewed and compared to 19 age- and sex-matched controls (average age, 7.9 years; range, 2–18 years; M:F=9:10). Genotypic information was available for 27 patients (15 FA-A, 2 FA-C, 3 FA-G, and 7 FA-D1 [biallelic mutations in BRCA2 gene]).

Results

Of the 36 patients, 61% had at least one congenital central nervous system or skull base abnormality. These included hypoplastic clivus (n=12), hypoplastic adenohypophysis (n=11), platybasia (n=8), pontocerebellar hypoplasia (n=7), isolated pontine hypoplasia (n=4), isolated vermis hypoplasia (n=3), and ectopic neurohypophysis (n=6). Average pituitary volume was significantly less in patients with Fanconi anemia (P<0.0001) than in controls. Basal angle was significantly greater in Fanconi anemia patients (P=0.006), but the basal angle of those with FA-D1 was not significantly different from controls (P=0.239). Clivus length was less in the Fanconi anemia group (P=0.002), but significance was only observed in the FA-D1 subgroup (P<0.0001). Of the seven patients meeting criteria for pontocerebellar hypoplasia, six belonged to the FA-D1 group.

Conclusion

Patients with Fanconi anemia have higher incidences of ectopic neurohypophysis, adenohypophysis hypoplasia, platybasia and other midline central nervous system skull base posterior fossa abnormalities than age- and sex-matched controls. Patients with posterior fossa abnormalities, including pontocerebellar hypoplasia, are more likely to have biallelic BRCA2 mutations.

     

Prognostic factors indicating survival with native liver after Kasai procedure for biliary atresia

Background

The number of the bile ducts in the portal canal/measured surface area of the portal canal (BDP ratio) indicates prognosis in biliary atresia (BA), as does an elevated cytokeratin 7 positivity percentage (PCK7). We compared these two markers.

Methods

We reviewed 32 BA cases undergoing Kasai operation from 1976 to 2016 with >5 portal canals in biopsy samples. Group I required liver transplantation or died within a year of operation (n = 8). Group II survived with their native liver (n = 24). We determined the BDP ratio (10²/mm²) and PCK7 (%), subdividing patients into three groups by their age at operation: Group A ≤60 days (n = 6, 1 Group I), 60< Group B ≤90days (n = 16, 5 Group I), Group C >90 days (n = 10, 2 Group I).

Results

PCK7 (%) was 2.71 ± 1.87 in Group I and 4.25 ± 2.56 in Group II (p = 0.13). BDP ratio (10²/mm²) was 1.19 ± 0.424 in Group I and 1.64 ± 0.534 in Group II (p = 0.04). Both markers were higher in Group C than in Group A or B (p < 0.01).

Conclusion

The BDP ratio is a better prognostic indicator than PCK7 in BA.

     

Laparoscopic versus open inguinal hernia repair in children ≤3: a randomized controlled trial

Purpose

Laparoscopy is being increasingly applied to pediatric inguinal hernia repair. In younger children, however, open repair remains preferred due to concerns related to anesthesia and technical challenges. We sought to assess outcomes after laparoscopic and open inguinal hernia repair in children less than or equal to 3 years.

Methods

A prospective, single-blind, parallel group randomized controlled trial was conducted at three clinical sites. Children ≤3 years of age with reducible unilateral or bilateral inguinal hernias were randomized to laparoscopic herniorrhaphy (LH) or open herniorrhaphy (OH). The primary outcome was the number of acetaminophen doses. Secondary outcomes included operative time, complications, and parent/caregiver satisfaction scores.

Results

Forty-one patients were randomized to unilateral OH (n = 10), unilateral LH (n = 17), bilateral OH (n = 5) and bilateral LH (n = 9). Acetaminophen doses, LOS, complications, and parent/caregiver scores did not differ among groups. Laparoscopic unilateral hernia repair demonstrated shorter operative time, a consistent finding for overall laparoscopic repair in univariate (p = 0.003) and multivariate (p = 0.010) analysis. No cases of testicular atrophy were documented at 2 (SD = 2.7) years.

Conclusion

Children ≤3 years of age in our cohort safely underwent LH with similar pain scores, complications, and recurrence as OH. Parents and caregivers report high satisfaction with both techniques.

     

Hematopoietic stem cell transplantation for children with <Emphasis Type="Italic">β</Emphasis>-thalassemia major: multicenter experience in China

Background

β-Thalassemia major (β-TM) has become a public health problem in mainland China. Hematopoietic stem cell transplantation (HSCT) has remained the only cure for β-TM in mainland China since 1998.

Methods

This multicenter retrospective study provides a comprehensive review of the outcomes of 50 pediatric patients with β-TM who received HSCT between 1998 and 2009 at five centers in mainland China. Both related (n = 35) and unrelated donors (n = 15) with complete human leukocyte antigen matches were included. The stem cell sources included bone marrow (BM), peripheral blood stem cells, umbilical cord blood (UCB) and a combination of BM and UCB or a combination of BM and peripheral blood stem cells from a single sibling donor.

Results

The probabilities of 5-year overall survival (OS) and thalassemia-free survival (TFS) after the first HSCT were 83.1 and 67.3%, respectively. Graft failure (GF) occurred in 17 patients. Univariate analyses showed that umbilical cord blood transplantation (UCBT) was one of the potential risk factors for decreased OS (P = 0.051), and that UCBT (P = 0.002) was potentially related to TFS. GF incidence was distinct between the UCBT and non-UCBT groups (P = 0.004). Four cases of UCB-BM combined transplantation led to decreased risks of mortality and recurrence. In the UCBT group, related donor transplantation produced more favorable results than unrelated donor transplantation in OS (P = 0.009) but not in TFS (P = 0.217).

Conclusions

GF was the primary cause of UCBT failure. Though UCBT from related donors was not favorable, the combined transplantation of UCB and BM could improve the prognosis of UCBT.

     

Relation between amount of bile ducts in portal canal and outcomes in biliary atresia

Background

There is no good prognostic indicator for biliary atresia (BA). We reviewed liver biopsies taken during the initial procedure to find a prognostic marker.

Methods

Thirty-two BA cases underwent Kasai operation from 1976 to 2009. We compared two groups at 1, 3, and 9 years. Group A required liver transplantation or died. Group B survived with their native liver. Biopsies were analyzed for liver fibrosis, portal-central vein bridging (P–C bridging), ductal plate malformation (DPM) and the number of the bile ducts in portal canal/measured surface area of the portal canal (BDP ratio). Statistical comparisons of the multiple data were evaluated by Mann–Whitney U test, Student’s t test and Pearson’s Chi-square test. Regression analysis with P < 0.05 was considered significant.

Results

BDP ratios (/mm²) were 2.4 ± 1.5 in Group A1 (n = 9) vs 4.6 ± 2.4 in Group B1 (n = 23) (P = 0.01); 2.6 ± 1.4 in Group A3 (n = 14) vs 5.1 ± 2.5 in Group B3 (n = 18) (P < 0.01), 3.0 ± 2.2 in Group A9 (n = 15) vs 4.9 ± 2.5 in Group B9 (n = 15) (P < 0.05). There was no significant difference in any other finding.

Conclusion

The BDP ratio is a sound prognostic indicator in BA.