巨指症9例治疗体会

目的 介绍巨指症的手术治疗与疗效。方法 对9例巨指症病人分别采用巨指修剪术及神经松解术、巨指截指术、巨指部分切除与足趾移植再造术。结果 术后经0．5－15年（平均5年9个月）的随访，2例巨拇指修剪术术后外形仍显臃肿，功能不理想，其余7例术后功能与外形均满意。结论 根据巨指发生的指别与指数，巨指增生的程度，尤其是骨、关节以及神经增生程度，选择最佳的手术方案。     

A gastric duplication cyst with an aberrant pancreatic ductal system: report of a case

We report an extremely rare case of a gastric duplication cyst together with an aberrant pancreatic ductal system, which communicated with the stomach rather than the pancreatic ductal system with no evidence of pancreatitis. A 46-year-old woman developed severe abdominal pain after a 10-year history of occasional mild abdominal pain. Upper gastrointestinal barium radiography showed a rigidity of the stomach wall, and gastroscopy revealed a fistula orifice at a greater curvature of the gastric body. Subsequent endoscopic suction of mucous secretion from within the fistula provided immediate pain relief. Abdominal computed tomography and ultrasonography showed a cystic mass contiguous with the stomach wall. Surgical exploration revealed an uncommon anomaly of a gastric duplication cyst with the aberrant pancreatic lobe. The patient made an uneventful recovery and remains well 4 years after surgery. We also herein review ten other similar cases of this uncommon congenital anomaly reported in the literature. Received: August 20, 2001 / Accepted: January 8, 2002     

Preliminary experience with the combined use of recombinant bone morphogenetic protein and bisphosphonates in the treatment of congenital pseudarthrosis of the tibia

Purpose  

Congenital pseudarthrosis of the tibia (CPT) is a rare but serious disorder in children. No single approach has clearly emerged as superior in terms of operative procedure, fixation, optimal time for surgery or adjunctive pharmaceutical intervention. CPT is frequently associated with neurofibromatosis type 1 (NF1), a condition featuring deficient bone anabolism and excessive catabolism. We have therefore combined the use of bone morphogenetic proteins (BMP) with bisphosphonates (BP) as an adjunct to surgical intervention.     

Neonatal tracheal changes following in utero fetoscopic balloon tracheal occlusion in severe congenital diaphragmatic hernia

Objective

To report postnatal tracheal changes after in utero fetoscopic balloon tracheal occlusion in severe congenital diaphragmatic hernia (CDH).

Design

Case series.

Setting

Tertiary care center, CDH National Reference Center.

Patients

Seven consecutive newborn infants with severe CDH who underwent fetoscopic balloon tracheal occlusion.

Interventions

Flexible laryngotracheoscopy and histological aspect observed at necropsy in 2 nonsurvivors.

Results

All infants displayed elongation and relaxation of the posterior tracheal wall, intermittently obstructing the lumen during tidal breathing. Whereas the cartilage displayed adequate rigidity, the pars membranacea appeared both flaccid and loose. Tracheal widening (tracheomegaly) was seen in all cases. Histology (n = 2) pointed to structural modifications throughout the pars membranacea, that is, loss of epithelial folding and of longitudinal elastic network and focal muscular disruption. The cartilage displayed no visible or histologic changes. The above tracheal changes were not symptomatic, except for a barking cough during increased respiratory efforts.

Conclusions

Tracheal widening and intermittent collapse of the posterior wall of the trachea during tidal breathing was found in 7 consecutive newborns who underwent fetoscopic balloon tracheal occlusion, causing mild clinical symptoms. Endoscopic tracheal assessment might provide useful information in children with CDH, in particular, when they underwent in utero fetoscopic balloon tracheal occlusion.     

The paradoxical effect of medical insurance on delivery of surgical care for infants with congenital anomalies

Objective

Caring for neonates with major congenital anomalies has significant financial implications for the treating institution, which can be positive or negative depending on whether the patient has insurance. We hypothesized that insured affected neonates born in non-children's hospitals would be more likely to be treated on site, whereas uninsured neonates would be more likely to be transferred.

Patients and Methods

We used the Kids' Inpatient Database to study neonates with congenital anomalies who were born in US non-children's hospitals. We performed bivariate analysis using the χ² test and adjusted for covariates with multiple logistic regression.

Results

Uninsured patients were 2.57 (95% confidence interval, 1.83-3.62) times more likely to be transferred compared with patients with private insurance or Medicaid, after adjusting for patient and hospital characteristics. This trend increased over time between 1997 and 2006.

Conclusions

The current reimbursement structure in the United States incentivizes non-children's hospitals to retain insured patients with congenital anomalies and transfer uninsured patients with these same anomalies. This places a disproportionate financial burden on children's hospitals while paradoxically causing insured infants to be cared for at hospitals that may not be best equipped to provide complex care.     

Congenital and acquired mesocolic hernias presenting with small bowel obstruction in childhood and adolescence

Objective

The objective was to present a case series of pediatric patients presenting with small bowel obstruction secondary to both congenital and acquired internal mesocolic hernias, and the use of imaging technology in the management of this condition.

Methods

A retrospective review of patients treated at the Yale-New Haven Children's Hospital for small bowel obstruction from 1998 to 2008 (n = 6) who presented with acute small bowel obstruction secondary to internal mesocolic hernias was performed.

Results

We present 6 patients with small bowel obstruction caused by congenital (n = 4) and acquired (n = 2) mesocolic hernias after previous surgery. The median age at presentation was 13 years. Small bowel obstruction with a mesocolic hernia was identified by preoperative abdominal computerized tomography in 3 patients (50%) and at operation in the others. The mean length of stay was 6 days, with no recurrent episodes in the follow-up period.

Conclusion

Small bowel obstruction secondary to mesocolic hernias, although rare, may be considered in the differential diagnosis of patients with history of malrotation or abdominal wall defects owing to their association with congenital mesenteric anomalies. This condition requires special attention from the clinician because of its catastrophic consequences. Imaging studies are an important asset because of the difficulty in making an accurate clinical diagnosis and the rarity of internal hernias.     

Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma?

Background

The relationship between congenital cystic adenomatoid malformation (CCAM) and pleuropulmonary blastoma (PPB), whether causal, correlational, or coincidental, remains controversial. There is a lack of consensus as to the optimal treatment of patients with asymptomatic CCAM.

Method

We reviewed all cases of CCAM and PPB seen at our institution from 1999 to 2008. Institutional Research Ethics Board approval was obtained. The incidence of CCAM and PPB, respectively, was calculated based on birth numbers during the study period.

Results

Seventy-four CCAMs were resected over the study period in 129 children diagnosed with CCAM. Five PPBs were diagnosed during the study period. Three of the 5 PPB cases were initially diagnosed as CCAMs. These PPBs were not clinically or radiologically distinguishable from CCAMs. In our referral area, the incidence of CCAM was 1 in 12,000; and the incidence of PPB was 1 in 250,000 live births. The mortality rate for PPB in this cohort was 20%.

Conclusion

Asymptomatic cystic lung malformations represent a therapeutic dilemma. In this cohort, the incidence of PPB among apparently benign lung lesions was 4%. No clinical or radiological markers differentiated benign CCAMs from PPBs. Our experience provides further justification for resection of all CCAMs. This should be discussed with parents until CCAMs and PPBs can be clearly distinguished preoperatively.     

Survival disparities in newborns with congenital diaphragmatic hernia: a national perspective

Purpose

The aim of the study was to examine national outcomes for congenital diaphragmatic hernia (CDH).

Methods

We analyzed the Kids' Inpatient Database for patients admitted at less than 8 days of age.

Results

Overall, 2774 hospitalizations were identified. Most patients were white and had private insurance. Most patients were treated at urban (96%), teaching (75%), and not identified as children's hospital (NIACH) (50%). Birth was the most common admission source at NIACH (91%) and children's unit in general hospital (CUGH) (59%), compared to hospital transfer at children's general hospital (CGH) (81%). Most CDH were repaired through the abdomen (81%), and 25% required extracorporeal membrane oxygenation (ECMO). Most NIACH patients were transferred to another hospital, whereas most at CGH and CUGH were discharged home. Survival to discharge was 66% after excluding hospital transfers. Univariate analysis revealed higher survival for males, birth weight (BW) of 3 kg or more, whites, patients with private insurance, and those in the highest median household income quartile. Survival was 86% after CDH repair but 46% for ECMO. Multivariate analysis identified black race (hazard ratio [HR], 1.536; P = .03) and other race (HR, 1.515; P = .03) as independent predictors of mortality.

Conclusions

Hospital survival for CDH is related to sex, BW, race, and socioeconomic status. Blacks and other non-Hispanic minorities have higher mortality rates.     

Evaluation of perfusion modes on vital organ recovery and thyroid hormone homeostasis in pediatric patients undergoing cardiopulmonary bypass

The objectives of this study were: (i) to evaluate the effects of perfusion modes (pulsatile vs. nonpulsatile) on vital organs recovery and (ii) to investigate the influences of two different perfusion modes on the homeostasis of thyroid hormones in pediatric patients undergoing cardiopulmonary bypass (CPB) procedures. Two hundred and eighty‐nine consecutive pediatric patients undergoing open heart surgery for repair of congenital heart disease were prospectively entered into the study and were randomly assigned to two groups: the pulsatile perfusion group (Group P, n = 208) and the nonpulsatile perfusion group (Group NP, n = 81). All patients received identical surgical, perfusional, and postoperative care. Study parameters included total drainage, mean urine output in the intensive care unit (ICU), intubation time, duration of ICU and hospital stay, the need for inotropic support, pre‐ and postoperative enzyme levels (ALT [alanine aminotransaminase] and AST [aspartate aminotransaminase]), c‐reactive protein, lactate, albumin, blood count (leukocytes, hematocrit, platelets), creatinine levels, and thyroid hormones (thyroid stimulating hormone [TSH], FT₃[free triiodothyronine], FT₄[free thyroxine]). All patients survived the perioperative and postoperative periods. There were no statistically significant differences in either preoperative or operative parameters between the two groups. Group P, compared to Group NP, required significantly less inotropic support, had a shorter intubation period, higher urine output in ICU, and shorter duration of ICU and hospital stay. Lower lactate levels and higher albumin levels were observed in Group P and there were no significant differences in creatinine, enzyme levels, blood counts, or drainage amounts between two groups. TSH, Total T₃, Total T₄, and FT₃, FT₄ levels were markedly reduced versus their preoperative values in both groups. FT₃ and FT₄ levels were reduced significantly further in the nonpulsatile group both during CPB and at 72 h postoperation. The results of this study confirm our opinion that pulsatile perfusion leads to better vital organ recovery and clinical outcomes in the early postoperative period as compared to nonpulsatile perfusion in pediatric patients undergoing CPB cardiac surgery. The plasma concentrations of thyroid hormones are dramatically reduced during and after CPB, but pulsatile perfusion seems to have a protective effect of thyroid hormone homeostasis compared to nonpulsatile perfusion.     

Height outcome of patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Objective:To retrospectively investigate the height outcome of patients with congenital adrenal hyperplasia(CAH)due to 21-hydroxylase deficiency(21-OHD).Methods:The 135 CAH patients with 21-OHD diagnosed in our hospital from Jan 1980 to Oct 2006 were retrospectively analyzed.The investigated parameters included final height(FH),FH standard deviation score(FH SDS),target height SDS(TH SDS),difference between TH and FH(TH-FH),FH SDS-TH SDS,the age of onset of sexual development,and the difference between bone age and chronological age(BA-CA)when patients got the FH.Results:Among the 135 patients,female/male=108/27.Mean FH was(156.8±5.4)cm(n=14)and(150.8±6.8)cm(n=76)for males and females,respectively.Mean FH SDS was(-0.6±0.8)(n=13)and(0.2±1.2)(n=54)for males and females,respectively.Sexual development began at(5.2±1.7)years old(y/o)(n=13)and(7.9±3.2)y/o(n=43)in males and females,respectively.Conclusions:The FH of CAH patients with 21-OHD was lower than that of the normal range.Effect of the disease on the height growth in male patients was more severe than that in females.All patients began sexual development much earlier than the normal age-matched group.Male patients began their sexual development even earlier.