Long-term results of 10 conjoined twin separations

Background/Purpose

From 1978 to 2000, 10 pairs of conjoined twins were successfully separated with 14 surviving twins. Six were separated emergently because of the death or impending death of their respective twins. All of the remaining 4 sets required at least one emergency operation on at least one twin before subsequent separations.

Methods

A 6-item questionnaire was developed to interview this unique cohort. The question design was open ended to maximize opportunity for patient/family response. Families were administered the same questionnaire in face-to-face interviews. The physician-author asked questions related to physical health and disability, whereas the social worker-author asked questions related to developmental, educational, psychosocial, and family functioning.

Results

The first 4 surviving twins have graduated from college. The 1981 twin completed high school. The remaining 9 twins are in classes appropriate for their age. All 14 twins have required operations subsequent to initial discharge, particularly to correct urological, orthopedic, neurosurgical, as well as pediatric surgical problems.

Conclusions

In utero diagnosis of conjoined twins by ultrasonography compels physicians and parents early in the pregnancy to make decisions concerning possible surgical separation and the alternative of pregnancy termination. The case reports presented offer additional information for physicians and parents to make informed decisions at a crucial time by illustrating long-term physical and psychological outcomes of 14 surgical survivors.     

Epidemiological and clinical analysis of a consecutive series of conjoined twins in Spain

Purpose

The aim of the study was to analyze the frequency and certain epidemiological characteristics of a consecutive series of conjoined twins born in Spain.

Material and Methods

We used data from the Spanish Collaborative Study of Congenital Malformations for the period April 1976 to 2006. Because the Spanish law permitting voluntary termination of pregnancies (TOP) when the fetus presented malformations was effective by the end of 1985, we analyzed the data in 4 periods, 2 before 1986 and 2 after. During the first period (1976-1979) only live births were recorded, whereas both still and live births were included in the other three (1980-1985, 1986-1995, and 1996-2006). In the present study, the cases were classified as symmetrical (16 pairs) and asymmetrical (1 pair) conjoined twins. Each pair of conjoined twins was considered as only one case for calculations, regardless of the type of union.

Results

Among a total of 2,281,604 consecutive births between 1980 and 2006, there were a total of 15 cases of symmetrical conjoined twins giving a frequency of 0.70 per 100,000 (1/152,107), whereas there was only 1 stillborn asymmetrical conjoined twin pair (0.04/100,000). Among the 13,418 consecutive stillborns surveyed, 6 cases of conjoined twins were identified (either symmetrical or asymmetrical) giving a frequency of 44.72 per 100,000, and 11 pairs were identified among the 2,425,583 total live births surveyed during the first period 1976 to 1979, a frequency of 0.45 per 100,000. Thus, the frequency among stillborn infants is 99.34 times higher than that observed among live births. However, the frequency for the total births (3 last periods) showed a decreasing trend from 1.47 per 100,000 birth in the first period (1980-1985) when TOP was illegal, to a value of 0.09 per 100,000 in the last period, more than 16-fold lower, probably because of the TOP of affected fetuses. Therefore, we consider that the frequencies observed in the period 1980 to 1985 are the basal values in our population. The most frequent type observed was thoracopagus, with an overall prevalence at birth of 0.44 per 100,000 (1/228,160) from 1980 to 2006, representing 58.82% of the total population of symmetric conjoined twin pairs. Diprosopus pairs were the next most common group (11.76%). Most of the cases were females (4 males/11 females), and although this appeared to be mainly because of the thoracopagus pairs (males-females, 2:8), in such a small number of cases, it is not possible to determine the ratios for the other groups. Gestational age was significantly shorter than in control twins for each type studied.

Conclusions

We conclude that it is incorrect to consider that all types of conjoined twins have the same epidemiological characteristics, such as the frequency at birth. The differences observed may be related with the distinct embryo-fetal mortality of each type of conjoined twins in different populations, and the sex ratio, among others.     

Is replacement of albumin in major abdominal surgery useful?

Study Objective

To evaluate retrospectively serum albumin concentrations as well as morbidity and mortalitiy of abdominal surgical patients who -if hypoalbuminemic- did not receive human albumin solutions versus those who did receive such solutions.

Design

Retrospective observational study.

Setting

Academic community hospital.

Measurements

The records of 76 consecutive patients who had undergone elective abdominal surgery were reviewed. Preoperative and postoperative serum albumin concentrations up to the seventh day after surgery were recorded. Morbidity and mortality were followed until the time of discharge from the hospital.

Results

38 patients who received albumin replacement if they were hypoalbuminemic versus 38 patients who did not receive albumin replacement showed no significant difference in cumulative (P < 0.52) or individual postoperative complications (infections P < 0.35, cardiovascular complications P < 1.0, organ failure P < 0.67, thromboembolic incidents P < 0.26), and mortality (P < 0.47).

Conclusions

Postoperative serum albumin concentration had no correlation with postoperative morbidity. There is no justification for perioperative albumin replacement in abdominal cancer surgical patients.     

Serum levels of interleukin-9 during acute rejection in liver transplantation

Introduction

Interleukin-9 (IL-9) has been cast as a player in autoimmunity, but its role in liver transplantation remains to be clarified. The aim of our study was to investigate the time course of IL-9 serum levels during hepatic allograft rejection.

Methods

IL-9 serum levels were determined in 34 healthy subjects and 50 hepatic transplant recipients. The patients were divided into two groups: group I was composed of 15 patients with acute rejection episodes, and group II, 35 patients free of this problem. Samples were collected on days 1 and 7 after liver transplantation and on the day of liver biopsy.

Results

The concentrations of IL-9 were similar in the rejection and nonrejection groups over the entire postoperative period. The whole transplant group, including those with stable graft function, showed higher IL-9 serum levels than the controls at all times after liver transplantation.

Conclusions

These preliminary results suggest a lack of participation of IL-9 in human liver allograft rejection.     

Thirteen cases of isolated tubal torsions associated with hydrosalpinx in children and adolescents, proposal for conservative management: retrospective review and literature survey

Background/Purpose

Isolated tubal torsion associated with hydrosalpinx is a rare pathology. Our goal was to analyze the clinical and imaging features and discuss the different treatment options available.

Methods

We retrospectively reviewed all the cases of adnexal torsion treated in our department of pediatric surgery over a 10-year period. We searched 2 electronic databases (Medline and Sciencedirect) and targeted reports published during the same period using the key words tubal torsion and hydrosalpinx.

Results

A total of 13 cases, 6 from our hospital and 7 in the medical literature, were identified and analyzed. In 9 (69%) of 13 cases (n = 9/13), torsion and hydrosalpinx occurred on the left fallopian tube. Salpingectomy was performed in 11 of the patients. The resected tubes showed the persistence of ciliated cells associated with signs of moderate ischemic infarction in 50% (n = 3/6) of the cases.

Conclusions

Isolated tubal torsion associated with hydrosalpinx is too often misdiagnosed and treated by salpingectomy regardless of the negative impact on the future reproductive potential of our young patients. As is commonly advocated for ovarian salvage in adnexal torsions, tubal conservation should be favored when possible.     

Surgical separation of shared liver with cotton tourniquets in conjoined twins: simple and effective hemostasis

Objective

Surgical separation of the fused liver is extremely risky and sometimes life-threatening in conjoined twins because of the potential risks of hypovolemia and hemorrhagic shock.

Methods

Three pairs of symmetrical conjoined twins sharing fused livers were successfully separated by using a simple but effective local blockade measure without disturbing the portal circulation.

Results

The volume of intraoperative blood loss was minimal, and no major complications occurred. All the separated babies survived the procedure and remained healthy, both physically and mentally, after discharge. Two babies died of pneumonia associated with their preexisting cardiac defects.

Conclusions

Cotton tourniquets temporally and securely blocked the local blood supply to the narrow gap dissecting interface with minimal interference with the remaining segments, in addition to orienting the transection of the fused liver and minimizing blood loss from the liver dissection.     

First Year Renal Function as a Predictor of Kidney Allograft Outcome

Background

Several factors are known to have detrimental effects on kidney allograft function in the first year posttransplantation, which has been reported to be an important factor influencing long-term graft survival.

Objectives

The objectives of this study were to evaluate risk factors for lower estimated glomerular filtration rate (eGFR) at 3 and 12 months posttransplantation and analyze the influence of first year allograft function on graft and patient survivals.

Patients

We performed a retrospective review of the clinical data from 433 cadaveric donor kidney transplantations in adults performed in our unit from May 1989 to May 2007.

Results

Donor female gender and nontraumatic cause of death, panel-reactive antibody (PRA) titer ≥50%, acute rejection episodes, and delayed graft function (DGF) were significant risk factors for a decreased eGFR at one year posttransplantation. Recipient and donor age showed negative correlations with eGFR at 3 and 12 months. A logistic regression model showed acute rejection episodes, DGF, donor age ≥55 years, donor female gender, and nontraumatic cause of donor death to be independent adverse risk factors for eGFR <60 mL/min at 3 and 12 months. Lower eGFRs at 3 and 12 months were associated with poorer allograft survival when data were censored for death with a functioning graft and patient survival. Multivariate analysis revealed that PRA titer ≥50%, acute rejection episodes, and eGFR <30mL/min at 12 months had adverse effects on allograft survival.

Conclusion

Several factors influence kidney allograft function in the first year after transplantation. Kidney allograft function at 12 months predicted long-term graft survival.     

Tinnitus in the elderly: Profile, correlates, and impact in the Nigerian Study of Ageing

Objective

To determine the prevalence and correlates of tinnitus among community elderly and its impact on their quality of life.

Study Design

Longitudinal cohort.

Setting

Yoruba-speaking communities in Nigeria.

Subjects and Methods

Face-to-face interviews of 1302 subjects 65 years or older selected by the use of a multistage stratified sampling of households. Subjects were assessed for subjective tinnitus, chronic health conditions, functional impairment, and quality of life by use of the brief version of the World Health Organization quality of life instrument.

Results

Tinnitus was reported in 184 (110 female and 74 male subjects), giving a prevalence of 14.1 percent (SE = 0.49). Gender, age, economic status, educational level, residence, smoking, and alcohol consumption were not significantly associated with tinnitus. Univariate analysis revealed a history of recurrent otitis media (odds ratio [OR] = 4.5, 95% confidence interval [95% CI] 3.1-6.6, P = 0.01), head injury (OR 3.4, 95% CI 2.1-5.6, P = 0.01), rhinosinusitis (OR 2.4, 95% CI 1.5-4.0, P = 0.01), dizziness (OR 2.1, 95% CI 1.4-3.1, P = 0.01), and hypertension (OR 1.7, 95% CI 1.0-2.7, P = 0.05) as significant correlates. However, in multivariate analysis, only a history of otitis media and of head injury remained significant. Compared with those without, persons with tinnitus had a more negative perception of their overall health and a poorer quality of life as well as twofold likelihood to experience impairment in both activities of daily living and instrumental activities of daily living.

Conclusion

Tinnitus is common among elderly Nigerians and is associated with treatable health conditions, such as otitis media, rhinosinusitis, head injury, and hypertension. Its association with functional impairment and reduced quality of life highlights the need for inclusion in any comprehensive health care for the elderly.     

Preoperative prostate-specific antigen isoform p2PSA and its derivatives, %p2PSA and prostate health index, predict pathologic outcomes in patients undergoing radical prostatectomy for prostate cancer

Background

Currently available predictive models fail to assist clinical decision making in prostate cancer (PCa) patients who are possible candidates for radical prostatectomy (RP). New biomarkers would be welcome.

Objective

Test the hypothesis that prostate-specific antigen (PSA) isoform p2PSA and its derivates, percentage of p2PSA to free PSA (%p2PSA) and the Prostate Health Index (PHI), predict PCa characteristics at final pathology after RP.

Design, setting, and participants

An observational prospective study was performed in 350 consecutive men diagnosed with clinically localised PCa who underwent RP.

Measurements

We determined the predictive accuracy of serum total PSA (tPSA), free PSA (fPSA), fPSA-to-tPSA ratio (%fPSA), p2PSA, %p2PSA, and PHI. The primary end point was to determine the accuracy of these biomarkers in predicting the presence of pT3 disease, pathologic Gleason sum ≥7, Gleason sum upgrading, and tumour volume <0.5 ml.

Intervention

Open retropubic and robot-assisted laparoscopic RP was performed. Pelvic lymphadenectomy was performed according to baseline oncologic parameters and the surgeon's judgement.

Results and limitations

The %p2PSA and PHI levels were significantly higher in patients with pT3 disease, pathologic Gleason sum ≥7, and Gleason sum upgrading (all p values <0.001). Conversely, %p2PSA and PHI levels were significantly lower in patients with tumour volume <0.5 ml (p < 0.001). By univariate analysis, both %p2PSA and PHI were accurate predictors of pT3 disease, pathologic Gleason sum ≥7, Gleason sum upgrading, and tumour volume <0.5 ml. By multivariate analyses, the inclusion of both %p2PSA and PHI significantly increased the predictive accuracy of a base multivariate model (excluding the tumour volume prediction for both variables, and Gleason sum upgrading for the model including %p2PSA) that included patient age, tPSA, fPSA, f/tPSA, clinical stage, and biopsy Gleason sum.

Conclusions

We found that p2PSA and its derivatives are predictors of PCa characteristics at final pathology after RP and are more accurate than currently available markers.     

The role of obesity in kidney transplantation outcome

Background

The number of obese kidney transplant candidates has been growing. However, there are conflicting results regarding to the effect of obesity on kidney transplantation outcome. The aim of this study was to investigate the association between the body mass index (BMI) and graft survival by using continuous versus categoric BMI values as an independent risk factor in renal transplantation.

Methods

We retrospectively reviewed 376 kidney transplant recipients to evaluate graft and patient survivals between normal-weight, overweight, and obese patients at the time of transplantation, considering BMI as a categoric variable.

Results

Obese patients were more likely to be male and older than normal-weight recipients (P = .021; P = .002; respectively). Graft loss was significantly higher among obese compared with nonobese recipients. Obese patients displayed significantly lower survival compared with nonobese subjects at 1 year (76.9% vs 35.3%; P = .024) and 3 years (46.2% vs 11.8%; P = .035).

Conclusions

Obesity may represent an independent risk factor for graft loss and patient death. Careful patient selection with pretransplantation weight reduction is mandatory to reduce the rate of early posttransplantation complications and to improve long-term outcomes.     

Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children

Purpose

The authors report their experience with doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations (LMs) in children.

Methods

A retrospective chart review was used to collect data on 11 patients treated with doxycycline sclerotherapy for LMs of the head and neck at our institution since 2003. Radiographic imaging allowed classification of patient LM as macrocystic, microcystic, or mixed according to previously published guidelines. Only patients with macrocystic or mixed lesions were offered doxycycline sclerotherapy. Radiographic imaging and physical examination were used to determine efficacy of treatment. After each treatment, the clinical and radiographic response was characterized as excellent (≥95% decrease in lesion size), satisfactory (≥50% decrease in volume and asymptomatic), or poor (<50% decrease in volume or symptomatic).

Results

Eleven patients underwent a total of 23 sclerotherapies with an average of 2 treatments per patient (range, 1-4). All 7 patients with macrocystic lesions achieved complete clinical resolution with an average radiographic resolution of 93%. The 4 patients with mixed lesions achieved only partial clinical resolution and an average of 73% radiographic resolution. No patient experienced any adverse effects related to the treatment. At a median follow-up of 8 months, 2 patients (18%) experienced lesion recurrence in the setting of concomitant infection.

Conclusion

Doxycycline sclerotherapy is safe and effective as a primary treatment modality for macrocystic and mixed LMs of the head and neck in the pediatric population.     

The use of intraabdominal tissue expanders as a primary strategy for closure of giant omphaloceles

Background

Giant omphaloceles present a unique challenge to pediatric surgeons because of the difficulty in obtaining timely, tension-free closure of tissues over the defect. Reports of the use of tissue expanders in the subcutaneous space, intramuscular space, or intraabdominal cavity have illustrated the usefulness of this technique to provide biologic closure of abdominal wall defects. However, these reports have focused on use of tissue expanders as a second-line treatment after other options, such as silastic silos or attempted primary closure, have failed.

Methods

We report 2 cases in which intraabdominal tissue expanders were used as a primary strategy to obtain closure of giant omphalocele defects.

Case Reports

The first patient was a baby boy born at 36 weeks by date who was prenatally diagnosed with a giant omphalocele. An intraabdominal tissue expander was placed at 2 weeks of age. The tissue expander was removed and his abdomen was primarily closed at 8 weeks of age. The second patient was born at 25 weeks gestation as part of a twin gestation with severe intrauterine growth retardation (600 g birth weight). Bedside reduction was not attempted because of severe pulmonary hypertension and significant loss of abdominal domain because of herniated liver and bowel. At 8 months of age, she underwent laparoscopically assisted placement of an intraabdominal tissue expander. At 9 months of age, the tissue expander was removed, all abdominal viscera were reduced, and the defect was closed using only an 8 × 8-cm piece of AlloDerm (LifeCell, Branchburg, NJ). Both children are currently at home and doing well.

Conclusions

We believe that early use of intraabdominal tissue expanders provides a more expedient method of obtaining closure of the defect in giant omphaloceles.     

A prospective, randomized trial for use of prednisolone in patients with facial nerve paralysis after parotidectomy

Background

A high rate of facial nerve paralysis (FNP) develops after parotid surgery, but there have been few clinical trials on treatments to improve recovery times. This study evaluated the efficacy of prednisolone in these patients.

Methods

A total of 123 patients who underwent parotidectomy without injury to the facial nerve were assessed prospectively for postoperative FNP. These patients were assigned randomly to treatment with prednisolone or placebo for 10 days. FNP was evaluated by House-Brackmann grades. Recovery rates and adverse effects were monitored regularly for 6 months after surgery.

Results

Of the 123 patients, 45 had postoperative FNP. All FNPs were grades II to IV, mostly on 1 or 2 facial areas. The incidence of FNP was increased significantly with malignant pathology, increased parotidectomy extent, and neck dissection (P < .01). Of 44 evaluable patients, 43 showed full recovery of FNP after 6 months, whereas 1 patient had permanent FNP. Recovery rates were equal in both groups, and there was no evidence of major adverse effects.

Conclusions

Prednisolone was ineffective in early recovery from postparotidectomy FNP.     

Prevention of seroma formation after mastectomy and axillary dissection by lymph vessel ligation and dead space closure: a randomized trial

Introduction

We aimed to reduce the incidence of seroma formation by altering surgical technique.

Methods

Two hundred one breast cancer patients were randomly divided into 2 arms: arm 1 was operated on using an altered surgical technique, which is to ligate all of the tissue connecting axillary vein bundles to the specimen, to suture the anterior edge of the latissimus dorsi to the chest wall, and to fix the skin flap to the underlying muscle by subcutaneous sutures; arm 2 was operated on using the conventional technique.

Results

The drainage volume, in the initial 3 days, for patients in arm 1 was significantly less than that for patients in arm 2 (P < .01). The duration of drainage in arm 1 was shorter than that in arm 2 (P < .01). The incidence of seroma formation in arm 1 (2%) was significantly less than that in arm 2 (14%) (P < .01).

Conclusion

The modified operating technique is an effective approach to reducing the incidence of seroma formation after mastectomy and axillary dissection.     

Hereditary pancreatitis in children: surgical implications with special regard to genetic background

Purpose

Hereditary pancreatitis (HP) is the primary etiology of chronic pancreatitis during childhood, progressing through recurrent episodes of acute pancreatitis and finally leading to pancreatic insufficiencies. Hereditary pancreatitis is because of mutations of the cationic trypsinogen (PRSS1) gene. Some other genes, such as SPINK1 or CFTR, have been associated with familial idiopathic chronic pancreatitis. The aim of our study was to clearly define diagnostic and therapeutic strategies for HP patients, through an analysis of our study group and a review of the literature.

Methods

All children admitted from 1995 to 2007 with a final diagnosis of hereditary pancreatitis were restrospectively included in the study. We analyzed all medical records with special attention given to cases involving genetic screening (PRSS1, SPINK1, and CFTR genes).

Results

Ten children were included. Eight had HP with PRSS1 mutation, 2 of them without a familial history of chronic pancreatitis. The 2 others patients had SPINK1 mutations. Three HP patients were operated on for acute complications of pancreatitis and are well with a mean follow-up of 5.5 years. No patient had pancreatic insufficiencies or weight loss.

Conclusions

Hereditary pancreatitis is associated with severe pancreatitis, with a greater risk of developing pancreatic cancer. It must therefore be diagnosed correctly and treated to prevent its considerable complications.     

Severe dysplasia in children with familial adenomatous polyposis: rare or simply overlooked?

Background/Purpose

Because severe dysplasia and carcinoma in children with familial adenomatous polyposis (FAP) younger than 18 years is rare, earlier surgical intervention is not common. The purpose of this study is to report the prevalence of dysplasia and carcinoma among children with FAP in our institution.

Methods

From 1998 through 2004, children 18 years or younger with FAP that underwent total proctocolectomy at a large children's hospital were retrospectively reviewed.

Results

Eleven children underwent surgery for FAP. The mean age at surgery was 13 ± 3.2 years. Approximately half of the patients who underwent preoperative endoscopy had evidence of dysplasia. Nine (82%) patients had dysplasia on preoperative biopsy and/or operative specimen, and 3 (27%) of these patients had severe dysplasia or carcinoma in situ. Four of the 5 patients without symptoms had evidence of dysplasia. No patients had invasive carcinoma.

Conclusion

We observed a higher incidence of severe colonic dysplasia in young children with FAP compared with the current literature. A significant number of patients with dysplasia at surgery had no symptoms and no evidence of dysplasia on preoperative endoscopic biopsies. These data suggest that earlier intervention in children with FAP may be beneficial and should be investigated further.     

Surgical treatment for anomalous arrangement of the pancreaticobiliary duct with nondilatation of the common bile duct

Background/purpose

For anomalous arrangement of the pancreaticobiliary duct (AAPBD) with nondilatation of the common bile duct (CBD), the optimal surgical procedure remains controversial. The authors investigated which procedure would be most effective for AAPBD with nondilatation of the CBD.

Methods

The authors encountered 60 children with AAPBD in our institution between 1979 and 2002. Six of the 60 were classified as the nondilated type (CBD diameter; less than 8 mm), whereas the other 54 were classified as the dilated type (CBD diameter; more than 9 mm). Amylase levels in serum, CBD, and gallbladder were examined. Cellular activity of the resected gallbladder was examined for the incidence of hyperplasia and Ki-67 labeling index (Ki-67 LI).

Results

The amylase level in the nondilated type was elevated as in the dilated type. Epithelial hyperplasia of the gallbladder was present in 4 of the 6 with the nondilated type (67%). 10 of the 20 with the dilated type (50%), and none of the 6 controls (0%). The Ki-67 LI of the dilated type was significantly higher than that of control.

Conclusions

A free reflux of pancreatic juice into the biliary system was found regardless of dilatation, and cellular proliferative activity of the gallbladder mucosa was increased in both the nondilated and dilated type. Therefore, excision of the extrahepatic bile duct including cholecystectomy is recommended for AAPBD with nondilatation of the CBD.     

Repair of Chiari III malformation using cranioplasty and an occipital rotation flap: technical note and review of literature

Background

Chiari III malformation (CM3) is rare among Chiari malformations (I-IV). Its definition has been expanded to include caudal medullary displacement and hindbrain herniation into encephaloceles in lower occipital and high cervical regions. Prognosis is recorded as dismal, with respect to survival and functional outcome.

Methods

We describe the presentation, radiologic evaluation, and repair of this malformation using methyl-methacrylate cranioplasty and an occipital scalp rotation flap for closure. Outcome after surgery is addressed.

Results

Adequate closure of the defect and protection of underlying structures was achieved without undue stress at incision site.

Conclusions

This method of closure can be considered in cases of large occipital and cervical encephaloceles with poor skin cover and added osseous anomalies around the foramen magnum.