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1.
Technol Health Care ; 31(6): 2155-2164, 2023.
Article in English | MEDLINE | ID: mdl-37302053

ABSTRACT

BACKGROUND: A multi-pod catheter (MPC) is a large drainage catheter that can house multiple smaller retractable (MPC-R) and deployable catheters (MPC-D) within the body. OBJECTIVE: The drainage capabilities and resistance to clogging of a novel MPC have been assessed. METHODS: The drainage capabilities are evaluated by placing the MPC in a bag of either a non-clogging (H2O) or clogging medium. The results are then compared to matched-size single-lumen catheters with either a close (CTC) or open tip (OTC). The means of five test runs were used to measure drainage rate, maximum drained volume (MaxDV), and time to drain the first 200 mL (TTD200). RESULTS: In the non-clogging medium, MPC-D had a slightly higher MaxDV than MPC-R, and higher flow rate than CTC and MPC-R. Moreover, MPC-D needed less TTD200 than MPC-R. In the clogging medium, MPC-D had a higher MaxDV than CTC and OTC, higher flow rate, and faster TTD200 than CTC. However, comparison with MPC-R showed no significant difference. CONCLUSION: The novel catheter may offer superior drainage compared to the single-lumen catheter in a clogging medium, implying various clinical applications, particularly when clogging is a potential risk. Further testing may be required to simulate various clinical scenarios.


Subject(s)
Catheters , Drainage , Humans , Drainage/methods , Equipment Design , Ventriculoperitoneal Shunt , Peritoneal Dialysis
2.
J Laparoendosc Adv Surg Tech A ; 30(2): 228-232, 2020 Feb.
Article in English | MEDLINE | ID: mdl-26953774

ABSTRACT

Introduction: Insecure gastropexy, gastric mucosa overgrowth, granulation tissue formation, and a nonhealing gastrostomy are unwanted consequences encountered in the current minimally invasive gastrostomy tube (GT) placement techniques. Aiming to overcome these problems we have developed a simplified laparoscopic-assisted GT insertion (LAG) procedure using guided transabdominal U-stitches (GTU) gastropexy. Materials and Methods: We retrospectively reviewed all LAG cases performed in our institute using the GTU technique. In brief, a curved clamp is inserted intragastrically through the laparoscopic port and guides a needle across the abdominal and gastric walls to exit, then re-enter back, through the port in an out-in-out fashion creating multiple spaced transabdominal U-stitches that are tied over pledgets. Results: Between March 2008 and January 2015, 31 cases had LAG attempted using GTU. Two cases were converted to open procedures for non-LAG-related reasons. The median age of the remaining 29 cases was 37 (range, 0.3-154.9) months. Of those patients, 20 had fundoplication (LAG-Fundo), whereas the remaining 9 had LAG-only. The mean operative times for LAG-Fundo and LAG-only were 148 ± 57.5 minutes and 41 ± 12.4 minutes, respectively. During a median follow-up of 21 (range, 4-81) months we did not encounter any procedure-related mortality, intraabdominal leaks, or bowel injuries. One patient required redo gastropexy due to unplanned early U-stitch removal, and 7 cases had transient external GT leak, granuloma formation, and/or skin infection. Conclusions: GTU can achieve a simple and secure LAG, avoiding the catastrophic complications of intraabdominal leak without the need of special instruments or enlarging the port's wound. Using a smaller wound and intraabdominally placed mucosa helps in minimizing the risk of wound infection and external leak. Transient complications are expected during the earlier phase of the learning curve.


Subject(s)
Gastropexy/methods , Gastrostomy/methods , Suture Techniques , Child , Child, Preschool , Conversion to Open Surgery , Enteral Nutrition , Female , Fundoplication/methods , Gastropexy/adverse effects , Gastropexy/instrumentation , Gastrostomy/adverse effects , Humans , Infant , Infant, Newborn , Laparoscopy/methods , Male , Operative Time , Postoperative Complications/etiology , Reoperation , Retrospective Studies
3.
Ann Saudi Med ; 37(4): 290-296, 2017.
Article in English | MEDLINE | ID: mdl-28761028

ABSTRACT

BACKGROUND: Faced with growing healthcare demand, the Saudi government is increasingly relying on privatization as a tool to improve patient access to care. Variation in children's access to surgical care between public (PB) and private providers (PV) has not been previously analyzed. OBJECTIVES: To compare access to pediatric surgical services between two coexisting PB and PV. DESIGN: Retrospective comparative study. SETTINGS: A major teaching hospital and the largest PV group in Saudi Arabia. PATIENTS AND METHODS: The outcomes for children who underwent inguinal herniotomy (IH) between May 2010 and December 2014 at both providers were with IH serving as the model. Data collected included patient demographics, insurance coverage, referral pattern and access parameters including time-to-surgery (TTS), surgery wait time (SWT) and duration of symptoms (DOS). MAIN OUTCOME MEASURE(S): TTS, SWT and DOS. RESULTS: Of 574 IH cases, 56 cases of in-hospital referrals were excluded leaving 290 PB and 228 PV cases. PV patients were younger (12.0 vs 16.4 months, P=.043) and more likely to be male (81.6% vs 72.8%, P=.019), expatriates (18% vs 3.4%, P < .001) and insured (47.4% vs 0%, P < .001). The emergency department was more frequently the source for PB referrals (35.2% vs 12.7%, P < .001) while most PV patients were self-referred (72.8% vs 16.7%, P < .001). Access parameters were remarkably better at PV: TTS (21 vs 66 days, P < .001), SWT (4 vs 31 days, P < .001) and DOS (33 vs 114 days, P < .001). CONCLUSION: When coexisting, PV offers significantly better access to pediatric surgical services compared to PB. Diverting public funds to expand children's access to PV can be a valid choice to improve access to care in case when outcomes with the two providers are similar. LIMITATIONS: Although it is the first and largest comparison in the pediatric population, the sample may not represent the whole population since it is confined to a single selected surgical condition.


Subject(s)
Health Services Accessibility/statistics & numerical data , Herniorrhaphy/statistics & numerical data , Hospitals, Private/statistics & numerical data , Hospitals, Public/statistics & numerical data , Pediatrics/statistics & numerical data , Child, Preschool , Female , Hernia, Inguinal/surgery , Hospitals, Teaching , Humans , Infant , Male , Retrospective Studies , Saudi Arabia , Time Factors , Time-to-Treatment/statistics & numerical data , Waiting Lists
5.
World J Surg ; 41(2): 394-401, 2017 Feb.
Article in English | MEDLINE | ID: mdl-27734081

ABSTRACT

BACKGROUND: Privatization is widely perceived as a tool to improve healthcare access; however, its impact on the access of surgical care has not been quantified. We used cholecystectomy as a model to assess the variation in access between coexisting public (PB) and private providers (PVs). METHODS: We performed cross-sectional analysis of patients who underwent cholecystectomy at two major PB and PV groups serving Riyadh, Saudi Arabia. Representative sample sizes were estimated based on 95 % confidence level and ±5 confidence interval (CI). Exclusion criteria were major comorbidities, emergency cholecystectomies, age ≥60 and concurrent non-minor procedures. Data collected were patients' demographics, payer status, and durations of symptoms, diagnosis and hospitalization. RESULTS: Between 2012 and 2104, samples of 330 and 297 were randomly selected from the total of 2164 and 1315 cases performed at PV and PB, respectively. Seventy-eight PV and 73 PB cases were excluded. The distribution of publically funded/insured/self-paid was (3/179/70 PV) and (209/0/4 PB), respectively. Median durations between symptoms and surgery for PV and PB cases were 90 and 365 days (P < 0.001), respectively, while the wait times after ultrasound-based diagnosis were 125 and 11 days (P < 0.001), respectively. Median hospitalization time was significantly shorter in PV compared to PB (1 vs. 2 days, P = 0.001), and same-day admissions were more frequent in PV 94 % than PB 41 % (RR 2.3, CI 1.9-2.7). CONCLUSIONS: When coexist in a competitive environment, PV offers a remarkably better access to cholecystectomies compared to PB. Facilitating access to PV can be an effective strategy to improve patient's access to surgical care.


Subject(s)
Cholecystectomy , Health Services Accessibility , Privatization , Time-to-Treatment/statistics & numerical data , Adult , Cross-Sectional Studies , Economic Competition , Female , Humans , Length of Stay/statistics & numerical data , Male , Saudi Arabia
6.
Ann Saudi Med ; 34(6): 499-502, 2014.
Article in English | MEDLINE | ID: mdl-25971823

ABSTRACT

BACKGROUND: Repair of congenital diaphragmatic hernia (CDH) is ideally delayed until ventilatory parameters are stabilized and patients are switched to conventional ventilation. However, in selected high-risk patients, repair can be performed earlier while they are still on high-frequency oscillatory ventilation (HFOV). DESIGN AND SETTINGS: A retrospective review of all CDH cases treated in our tertiary referral center between 1997 and 2013. METHODS: In 1997, we started repairing selected high-risk CDH cases under HFOV with or without inhaled nitric oxide (iNO). All repairs were performed once the infants' blood gas levels were acceptable. The infants were gradually weaned to conventional ventilation followed by extubation as their ventilatory parameters improved. Their records were reviewed to determine the group-wide outcomes. RESULTS: Between 1997 and 2013, 55 infants with CDH were treated in our institute; of these 12 high-risk cases were repaired under HFOV/iNO combinations and 1 was repaired without iNO. All patients had significant pulmonary hypertension and 8 had herniated livers. The mean age at repair was 9.1 (6.3) days. Two mortalities occurred at the first and tenth postoperative days. Among the remaining 11 survivors, the median ventilation and hospitalization days were 29.5 (11-84) and 45.5 (25-107), respectively, and the median duration under HFOV and conventional ventilation days were 15 (9-40) and 12 (3-47), respectively. CONCLUSION: CDH repair can be performed earlier under HFOV and iNO. The possible advantages are earlier restoration of normal anatomy and earlier start of enteral feeding while minimizing the risk of lung injury.


Subject(s)
Hernias, Diaphragmatic, Congenital/surgery , Herniorrhaphy/methods , High-Frequency Ventilation/methods , Ventilator-Induced Lung Injury/prevention & control , Administration, Inhalation , Bronchodilator Agents/therapeutic use , Cohort Studies , Early Medical Intervention , Female , Humans , Infant, Newborn , Male , Nitric Oxide/therapeutic use , Retrospective Studies
7.
J Pediatr Surg ; 48(1): 203-8, 2013 Jan.
Article in English | MEDLINE | ID: mdl-23331816

ABSTRACT

PURPOSE: Inguinal hernia repair is one of the most common procedures performed by pediatric surgeons. A percutaneous technique could be the next advance in inguinal hernia repair in children. We used a rat model to study the utility of percutaneous inguinal hernia repair (PHR) using 2-octyl-cyanoacrylate (OCT). METHODS: Four-week-old Lewis male rats were randomly divided into three groups: bilateral PHR group (BH) (n=15), unilateral PHR group (UH) (n=12), and sham group (n=14). After inducing a pneumoperitoneum, a 24-gauge cannula was advanced into the patent processus vaginalis (PPV) once air was aspirated. The canal is then obliterated by injecting 0.2 ml of 2-octyl-cyanoacrylate. Mating and herniography were performed at postoperative weeks 2 and 6, respectively. All rats were sacrificed at week 12. RESULTS: Herniography revealed complete closure of PPV in (25/30) BH, (12/12) UH, and (0/28) of the sham group. All OCT-treated sides were found obliterated at the post-mortem gross examination. Histological analysis of the inguinal region revealed patent vase in all rats. However, mild to moderate foreign body reactions and fat necrosis were noticed at the injected sites. All rats demonstrated fertility at mating. CONCLUSION: Percutaneous obliteration of PPV was feasible and safe in rats and potentially less invasive than the current techniques used in children. However, demonstrating long-term effectiveness, the need for pneumoperitoneum and the risk of OCT intraperitoneal spillage remain as challenges to overcome.


Subject(s)
Cyanoacrylates/therapeutic use , Hernia, Inguinal/surgery , Herniorrhaphy/methods , Minimally Invasive Surgical Procedures/methods , Tissue Adhesives/therapeutic use , Animals , Disease Models, Animal , Feasibility Studies , Male , Random Allocation , Rats , Treatment Outcome
8.
Ann Saudi Med ; 32(5): 502-6, 2012.
Article in English | MEDLINE | ID: mdl-22871620

ABSTRACT

BACKGROUND AND OBJECTIVES: Seating position in motor vehicle collisions (MVC) plays a major role in determining the injury pattern in mainly restrained children. However, compliance with child seating and restraint laws is still suboptimal. The role of seating position in predicting injury patterns among unrestrained children has not been previously studied. DESIGN AND SETTING: Retrospective review based on the trauma registry of a level I trauma center in Riyadh, Saudi Arabia. Data collection was restricted to unrestrained children involved in MVC. PATIENTS AND METHODS: Between July 2001 and March 2010, 274 records were identified. Detailed information about the collision, child seating position and the use of restraints was cross-verified using parental phone interviews. RESULTS: Of the 274 identified records, cross-verification was possible for 89 (32.4%) unrestrained children, 64 boys and 25 girls, with a mean (SD) age of 83 (40) months. Of these children, 41 (46.1%) were front seated (FS), and 48 (53.9%) were back seated (BS). There were higher rates of rollover (52.1% vs 24.4%, P=.02), ejection (41.7% vs 22%, P=.05), and occupant death ratio (14.8 vs 4, P=.04) among BS children. However, the two groups did not differ in pediatric trauma scores, Glascow coma scale score, or age distribution. FS children were more likely to present with isolated head, neck or facial injuries (HNFI) (51.2% vs 25%, P=.01), whereas BS children were more likely to suffer long bone or pelvic fractures (LPF) (60.4% vs 36.6%, P=.025). CONCLUSION: Injury pattern can vary according to seating position among unrestrained children presenting at trauma centers after MVC. While FS children are more likely to present with HNFI, BS children more often sustain LPF. BS children had similar trauma severity compared with FS children despite the higher-impact nature of their MVCs. While highlighting the value of proper restraints use and seating position, these results can be valuable in the initial assessment of traumatized children involved in MVC.


Subject(s)
Accidents, Traffic , Posture , Wounds and Injuries/epidemiology , Child , Female , Humans , Injury Severity Score , Male , Retrospective Studies , Trauma Centers
9.
J Pediatr Surg ; 47(5): 952-5, 2012 May.
Article in English | MEDLINE | ID: mdl-22595580

ABSTRACT

INTRODUCTION: Variation in access to care has a significant impact on the disease management process and outcomes. Variable access to care might have similar effects on the management of Hirschsprung disease (HD). However, such variation has not been highlighted. MATERIALS AND METHODS: All patients referred to 3 academic centers (ACs) with HD were reviewed. Patient presentations, referral patterns, timing, and type of surgical intervention were compared between patients born in AC and those born in non-AC. Babies born with major congenital anomalies (MCAs) or total colonic HD were excluded. RESULTS: Between 1998 and 2011, 129 patients were identified. After excluding 30 patients, 99 were split into 20 inborn patients (AC) and 79 outborn patients. Outborn patients more often presented with constipation (95% vs 65%, P = .001), whereas inborn patients presented with feeding intolerance or vomiting (75% vs 39%, P = .004). Outborn patients were diagnosed and had their pull-through (PT) at an older median age (in days) of 186 (1-2621) vs 4.5 (1-451) (P = .001) and 345 (11-2757) vs 92 (3-928) (P = .001), respectively. Moreover, inborn patients were more likely to undergo primary PT (75% vs 46%, P = .02) and avoid bowel diversion (95% vs 66%, P = .02). CONCLUSION: Variation in access to care can have a significant impact on the quality of care delivery in HD. Limited access to AC is associated with staged PT in addition to delay in the diagnosis and management.


Subject(s)
Academic Medical Centers/standards , Digestive System Surgical Procedures/standards , Health Services Accessibility , Healthcare Disparities , Hirschsprung Disease , Hospitals, Community/standards , Academic Medical Centers/statistics & numerical data , Age Factors , Child , Child, Preschool , Colon/surgery , Colostomy , Delayed Diagnosis , Digestive System Surgical Procedures/statistics & numerical data , Female , Hirschsprung Disease/diagnosis , Hirschsprung Disease/surgery , Hospitals, Community/statistics & numerical data , Humans , Infant , Infant, Newborn , Male , Outcome and Process Assessment, Health Care , Quality of Health Care , Referral and Consultation/statistics & numerical data , Retrospective Studies , Saudi Arabia
10.
J Pediatr Surg ; 47(1): 258-63, 2012 Jan.
Article in English | MEDLINE | ID: mdl-22244427

ABSTRACT

BACKGROUND: Minimally invasive (MI) congenital diaphragmatic hernia (CDH) repair can be challenging. Placing rib-anchoring stitches without creating skin incisions and closing wider defects are some of the difficulties. In Bochdaleck hernia repair, maintaining visceral reduction and minimizing pneumothorax use are additional obstacles. We describe the use of hollow-needle snares (HNS) and transthoracic traction stitches (TTS) to overcome these challenges. METHODS: Hollow-needle snares is assembled by passing a prolene stitch through a hollow needle creating a retractable snare, which is used to extract the placed anchoring stitches by passing it over the ribs but through the same stitch's skin entrance site. In Bochdaleck hernia, the early placement of TTS using HNS can facilitate visceral reduction, patch lay down, and tension-free closure of possible residual V-shaped defects. RESULTS: Between July 2009 and April 2011, we performed 10 consecutive MI CDH repairs for 9 patients, including 7 Bochdaleck and 3 Morgagni hernias. The median age was 8 days (range, 3-172 days), and the mean operative time was 148.5 ± 37.8 minutes for Bochdaleck hernia repairs. For Morgagni hernia, the median age was 18.3 months (range, 10.5-37 months), and the mean operative time was 100 ± 26.5 minutes. All cases were completed without conversion. One patient had a hernia recurrence and was repaired similarly, whereas the others had uneventful recovery at a median follow-up of 5.8 months (range, 1.1-23.7 months). CONCLUSION: Hollow-needle snare and TTS are simple and available tools that can facilitate MI repair of CDH. This initial experience demonstrates the technique's effectiveness and its excellent cosmetic outcomes.


Subject(s)
Hernias, Diaphragmatic, Congenital , Herniorrhaphy/methods , Sutures , Thoracoscopy , Female , Hernia, Diaphragmatic/surgery , Humans , Infant , Infant, Newborn , Male , Needles , Retrospective Studies
11.
J Laparoendosc Adv Surg Tech A ; 22(1): 97-101, 2012.
Article in English | MEDLINE | ID: mdl-22082006

ABSTRACT

INTRODUCTION: Inguinal herniotomy in children is still dominated by conventional open inguinal herniotomy (COIH) as laparoscopic techniques have yet to demonstrate clear advantages. A technical modification that minimizes the incision of COIH in selected children can offer another minimally invasive alternative. A comparative analysis of safety, efficacy, and parental attitudes between mini-scar inguinal herniotomy (MSIH) and COIH was performed. MATERIALS AND METHODS: All inguinal herniotomy cases performed between January 2008 and April 2010 were reviewed. Patients who were younger than 6 months, presented with complicated hernias, or had an associated hydrocele were excluded. In the MSIH group the final scar length was prospectively measured and then retrospectively compared with a matched group of COIH. Parents in both groups were then interviewed using a standardized questionnaire to inquire about operative outcomes, their satisfaction level, and perception of the incision size. RESULTS: Of the 145 patients identified, 113 (79%) had completed the parental phone interview at a mean interval of 275±212 days. Forty (35%) underwent MSIH with a mean final incision length of 7.7±2 mm, and 73 (65%) underwent COIH. The two groups were similar in age, sex, and hernias' sides. Postoperative complication including recurrence rates did not differ between MSIH and COIH (2.5% versus 6.8%, P=.4). However, parents in the MSIH group were more likely to notice that the scar was smaller than what they have expected (odds ratio, 4.9; 95% confidence interval, 2.1-11.9) and were more likely to be very satisfied (odds ratio, 10.8; 95% confidence interval, 3.1-38). CONCLUSION: The safety and efficacy of MSIH are comparable to those of COIH. However, in the MSIH group, parents are more likely to notice the smaller scar, which might improve their satisfaction.


Subject(s)
Hernia, Inguinal/surgery , Minimally Invasive Surgical Procedures/methods , Child , Cicatrix/prevention & control , Female , Humans , Learning Curve , Male , Parents , Patient Satisfaction , Recurrence , Retrospective Studies , Treatment Outcome
12.
Ann Saudi Med ; 31(6): 609-12, 2011.
Article in English | MEDLINE | ID: mdl-22048507

ABSTRACT

BACKGROUND AND OBJECTIVES: Hypertrophic pyloric stenosis (HPS) is a common cause of gastric outlet obstruction (GOO) in infants. Prolonged GOO is believed to result in acid and electrolyte disturbances, gastric atony, and delayed postoperative recovery. We studied the impact of prolonged vomiting as an indicator of GOO symptoms on the post-operative outcomes in HPS. DESIGN AND SETTING: A retrospective chart review of all patients who underwent pyloromyotomy at a tertiary care center between February 1997 and February 2009. PATIENTS AND METHODS: The duration of pre-operative vomiting was correlated with presenting electrolytes and acid-base balances, postoperative time to full feed, postoperative morbidity and duration of hospitalization. RESULTS: Forty-seven patients were identified. At presentation, the median (range) for duration of symptoms was 14 (3-60) days, and surgeries were performed at 2 (0-6) days after admission. Apart from one case of postoperative wound infection, all patients had an unremarkable recovery. The unusually prolonged duration of vomiting in our cohort did not correlate with the mean (SD) preoperative chloride level of 93.9 (8.8) mEq/L, mean (SD) pH level of 7.5 (0.9), mean postoperative time to full feeding of 31 (15.1) hours, or mean duration of hospitalization of 5.1 (2.2) days. CONCLUSION: Duration of vomiting in HPS at presentation does not seem to have a significant impact on the postoperative outcomes.


Subject(s)
Acid-Base Imbalance , Digestive System Surgical Procedures/adverse effects , Gastric Outlet Obstruction , Postoperative Complications/blood , Pyloric Stenosis, Hypertrophic , Vomiting , Acid-Base Imbalance/blood , Acid-Base Imbalance/etiology , Chlorides/blood , Digestive System Surgical Procedures/methods , Female , Gastric Outlet Obstruction/etiology , Gastric Outlet Obstruction/physiopathology , Gastric Outlet Obstruction/surgery , Humans , Infant , Length of Stay , Male , Prognosis , Pyloric Stenosis, Hypertrophic/complications , Pyloric Stenosis, Hypertrophic/physiopathology , Pyloric Stenosis, Hypertrophic/surgery , Recovery of Function , Statistics as Topic , Time Factors , Treatment Outcome , Vomiting/blood , Vomiting/etiology , Vomiting/physiopathology
13.
Saudi J Gastroenterol ; 17(5): 363-6, 2011.
Article in English | MEDLINE | ID: mdl-21912067

ABSTRACT

The presence of ductal disruption in pancreatic trauma is a major indicator of severity leading to higher morbidities and prolonged hospital stay. However, the adoption of early interventional approach in selected cases of documented grade III pancreatic trauma could result in shorter hospitalization and early recovery. We are describing our approach of early presentation-tailored interventions in managing two consecutive children diagnosed with grade III pancreatic injuries, which constitute the two main ends of the presentations' spectrum. For the early presenter a spleen preserving distal pancreatectomy was performed, while for the late presenter with large symptomatic pseudocyst endoscopic drainage was attempted. Both early and late presenting children had quick and uneventful recoveries leading to 5 and 6 days of hospitalization, respectively. Both cases continued to be asymptomatic at 4 and 12 months post procedure. In the pseudocyst case, the gastro-cystostomy stents were removed after 10 weeks, and 2.5 months later a completely healed pancreas was demonstrated by magnetic resonance cholangio-pancreatography. Unlike other abdominal solid organ injuries in children, adopting early presentation-tailored intervention can be associated with quicker recovery and short hospitalization for grade III pancreatic injuries. While the series is still small, achieving such remarkable outcomes in two consecutive cases is possible and could set a new trend in managing these injuries in children.


Subject(s)
Abdominal Injuries/surgery , Drainage/methods , Early Medical Intervention , Pancreas/injuries , Wounds, Nonpenetrating/surgery , Abdominal Injuries/diagnosis , Child , Child, Preschool , Cholangiopancreatography, Endoscopic Retrograde , Early Medical Intervention/trends , Female , Follow-Up Studies , Hospitalization/trends , Humans , Male , Pancreas/surgery , Time Factors , Tomography, X-Ray Computed , Wounds, Nonpenetrating/diagnosis
14.
J Pediatr Surg ; 45(9): 1896-9, 2010 Sep.
Article in English | MEDLINE | ID: mdl-20850641

ABSTRACT

Breast tumors are very rare in infants. We describe a 4-month-old female infant who presented with a firm and painless right breast mass. It was first noticed at the age of 1 month and then gradually increased in size. Further physical examination and imaging studies revealed other subcutaneous masses and lytic bone lesions. True-cut biopsy from the breast lesion was consistent with infantile myofibromatosis. Infantile myofibromatosis is a group of uncommon mesenchymal tumors that tend to occur in infancy and regress spontaneously, as demonstrated in our case. Surgical excision of such lesion might have led to permanent loss of breast tissue. The report discusses the clinical, radiological, and pathologic features, in addition to the previously described treatment options for this condition.


Subject(s)
Breast Diseases/diagnosis , Breast/pathology , Myofibromatosis/diagnosis , Biopsy, Needle , Female , Humans , Infant , Magnetic Resonance Imaging , Remission, Spontaneous
15.
Am J Med Genet A ; 146A(14): 1875-9, 2008 Jul 15.
Article in English | MEDLINE | ID: mdl-18553553

ABSTRACT

Johanson-Blizzard syndrome (JBS) is a rare autosomal recessive condition characterized by pathognomonic facies and a constellation of other features most notably exocrine pancreatic insufficiency, oligodontia, growth retardation, hearing loss, mental retardation, scalp defects, hypothyroidism and imperforate anus. We report on an infant with classical JBS who also has unusually severe neonatal cholestatic liver disease that progressed to liver fibrosis and portal hypertension. Sequencing of UBR1 revealed a previously unreported homozygous missense mutation in a consensus splice acceptor site (IVS12-1G > A). This report is the first to document severe liver involvement in JBS and raises the possibility that this could be a rare but genuine association.


Subject(s)
Abnormalities, Multiple/genetics , Liver/abnormalities , Point Mutation , Ubiquitin-Protein Ligases/genetics , Animals , Base Sequence , Consanguinity , Craniofacial Abnormalities/genetics , DNA/genetics , Disease Models, Animal , Exocrine Pancreatic Insufficiency/genetics , Female , Fetal Growth Retardation/genetics , Genes, Recessive , Hearing Disorders/genetics , Humans , Hypothyroidism/genetics , Infant , Infant, Newborn , Liver/pathology , Male , Mice , Mice, Knockout , Pregnancy , Syndrome , Ubiquitin-Protein Ligases/deficiency
16.
J Pediatr Surg ; 42(1): 203-6, 2007 Jan.
Article in English | MEDLINE | ID: mdl-17208566

ABSTRACT

PURPOSE: Prophylactic total thyroidectomy is now recommended after having confirmed RET mutations in children of parents with multiple endocrine neoplasia type 2 or familial medullary thyroid carcinoma. We reviewed our experience to determine the incidence of medullary thyroid carcinoma with respect to age at surgery, the location of the mutation, and its association with Hirschsprung's disease (HD). METHODS: A retrospective review from 1996 to 2005 revealed 20 children with genetic screening for multiple endocrine neoplasia type 2A or familial medullary thyroid carcinoma who underwent a prophylactic total thyroidectomy with parathyroid gland preservation. RESULTS: The median age of the 20 patients (9 boys and 11 girls) included in this study was 8.2 years (range, 3.7-16.9 years) at the time of their surgery. Final pathology revealed normal thyroid tissue (n = 3; median age, 5.9 years), C-cell hyperplasia (n = 13; median age, 10 years), or medullary thyroid carcinoma (n = 4; median age, 8 years). Four children, all with mutations in C620, had a previous diagnosis of HD. At a median follow-up of 3.7 years (range, 1 month to 8.4 years), all patients were well and cancer free. CONCLUSIONS: There is no correlation between histologic findings and median age at surgery. Hirschsprung's disease was found in 50% of the patients with the RET mutation in C620. In children of C620 parents, symptoms of HD should be actively sought, and if such are found, rectal biopsies should be performed even if mutation results are not yet available. Based on the age of the earliest cancer and the safety of total thyroidectomy, children should promptly undergo surgery after genetic screening and before their fifth year of life.


Subject(s)
Carcinoma, Medullary/epidemiology , Hirschsprung Disease/epidemiology , Multiple Endocrine Neoplasia Type 2a/epidemiology , Proto-Oncogene Proteins c-ret/genetics , Thyroid Neoplasms/epidemiology , Adolescent , Age Factors , Carcinoma, Medullary/surgery , Child , Child, Preschool , Female , Genetic Testing , Hirschsprung Disease/genetics , Humans , Incidence , Male , Multiple Endocrine Neoplasia Type 2a/surgery , Mutation , Prevalence , Retrospective Studies , Thyroid Neoplasms/surgery , Thyroidectomy
17.
J Pediatr Surg ; 41(5): e9-11, 2006 May.
Article in English | MEDLINE | ID: mdl-16677874

ABSTRACT

Congenital pulmonary arteriovenous malformations (AVMs) are rare lesions, usually asymptomatic. We report on the case of a baby who was thought to have a congenital cystic adenomatoid malformation of the left upper lobe based on prenatal and postnatal imaging. Final pathology revealed a congenital pulmonary AVM. Neither the child nor her family have any evidence of hereditary hemorrhagic telangiectasia. To our knowledge, this is the first reported case of a pulmonary AVM mimicking a congenital cystic adenomatoid malformation.


Subject(s)
Arteriovenous Malformations/diagnosis , Cystic Adenomatoid Malformation of Lung, Congenital/diagnosis , Pulmonary Artery/abnormalities , Pulmonary Veins/abnormalities , Diagnosis, Differential , Female , Humans , Infant, Newborn
18.
J Pediatr Surg ; 41(5): 940-2, 2006 May.
Article in English | MEDLINE | ID: mdl-16677888

ABSTRACT

BACKGROUND/PURPOSE: Wound management in children has traditionally consisted of daily dressings. Although vacuum-assisted closure (VAC) is well described in the adult literature, there are few reports about children. We reviewed our experience with VAC. METHODS: A retrospective review from 2003 to 2005 revealed that 16 children underwent VAC. Variables analyzed included demographics, diagnosis, duration and characteristics of VAC, wound closure, recurrence, complications, and cost analysis. RESULTS: Sixteen children received VAC therapy at an average age of 12.1 years (range, 1 month-18 years). Indications included tissue loss after pilonidal sinus excision (n = 8, primary = 5, recurrent = 3) after wound dehiscence of the abdomen (3), the sternum (2), the back (1), the leg (1), and after chronic postoperative perineal fistula. Average length of VAC use was 23 days, with an average pressure of 104 mm Hg. Wound closure occurred in 15 of 16 patients. Patients with primary pilonidal disease obtained wound closure by 45 days, whereas those with recurrent disease required 72 days. Children with wound dehiscence healed by 28 days. Recurrent sinuses developed in all 3 patients with known recurrent pilonidal disease. Pain in 1 patient required cessation of VAC therapy after 7 days. Follow-up after wound closure averaged 8 months. CONCLUSIONS: Vacuum-assisted closure is well tolerated in our pediatric population and offers many advantages including fewer dressing changes and an earlier return to daily activities.


Subject(s)
Soft Tissue Injuries/therapy , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Retrospective Studies , Vacuum , Wound Healing
19.
J Pediatr Surg ; 41(5): 975-9, 2006 May.
Article in English | MEDLINE | ID: mdl-16677896

ABSTRACT

PURPOSE: The aim of this study was to report the experience and efficacy of Ethibloc sclerotherapy as treatment of lymphangiomas. METHODS: Between 1992 and 2004, 63 patients had Ethibloc sclerotherapy for lymphangiomas at our institution. Computed tomographic scan or magnetic resonance imaging and clinical evaluation determined efficacy of the treatment. Results were classified as excellent (> or =95% decrease in lesion volume), satisfactory (> or =50% decrease and asymptomatic), or poor (<50% decrease or symptomatic). RESULTS: Sixty-three patients with 67 lesions underwent sclerotherapy with a median of 2 treatments per patient. Thirty-five involved the neck; 10, the head and face; and 22, the thorax or limb. Thirteen were predominantly microcystic; 28, macrocystic; and 26, mixed. Of the 63 patients, 6 underwent sclerotherapy for postsurgical residual lesions. Results were classified by type: of the 54 macrocystic/mixed cases, 26 (49%) had excellent, 19 (35%) had good, and 9 (16%) had poor results; in the 13 predominantly microcystic lesions, 3 (23%) had excellent, 7 (54%) had good, and 3 (23%) poor results. Five patients (7.7%) required surgery for complications; 2, for scar revision; 2, for persistent drainage; and 1, for a salivary fistula. Infection occurred in 4 patients (6.2%) after sclerotherapy. Follow-up averaged 3.5 years (range, 12 months to 12 years). CONCLUSION: Ethibloc sclerotherapy is a safe and effective alternative to surgical excision of macrocystic lymphangiomas and can be used for postsurgical recurrences.


Subject(s)
Diatrizoate/therapeutic use , Fatty Acids/therapeutic use , Lymphangioma/therapy , Propylene Glycols/therapeutic use , Sclerosing Solutions/therapeutic use , Sclerotherapy , Zein/therapeutic use , Child , Child, Preschool , Drug Combinations , Female , Follow-Up Studies , Humans , Infant , Male
20.
J Pediatr Surg ; 41(5): 1010-3, 2006 May.
Article in English | MEDLINE | ID: mdl-16677902

ABSTRACT

BACKGROUND: In-hospital observation of 24 to 48 hours has been the standard practice after successful enema reduction (ER) of ileocolic intussusceptions, but this practice has not been validated. We evaluated retrospectively the safety of short-term emergency department observation. METHODS: Between April 2000 and October 2004, 121 patients presented to the emergency department with ileocolic intussusception, and all had ER attempts. RESULTS: Ninety-six patients had successful reduction, 25 were excluded for failed reduction or unconfirmed diagnosis, and another 16 needed observation anyway for high white blood count or persistent postreduction pain. Of the remaining 80 patients, the mean time from symptoms to reduction was 45.9 hours (4 hours to 10 days). All patients, except one, were admitted for observation for a mean period of 1.6 days (8 hours to 6.5 days). No complications were associated with air ER; however, 6 (7.5%) patients had reintussusception during the observation period and 5 (6.3%) recurred after discharge. The mean intervals for recurrence postreduction were 17.8 hours and 14.5 months with no mortality or morbidity in either. CONCLUSIONS: Short-term emergency department observation could be a safe practice in more than 90% of the selected cases, recurrence of intussusception outside the hospital is not associated with unfavorable outcome, and routine admission is not warranted.


Subject(s)
Enema , Hospitalization , Ileal Diseases/therapy , Intussusception/therapy , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Remission Induction , Retrospective Studies
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