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1.
Semin Pediatr Surg ; 32(1): 151259, 2023 Feb.
Article in English | MEDLINE | ID: mdl-36739693

ABSTRACT

Robotic colorectal surgery allows adult and pediatric surgeons to overcome the technical limitations of laparoscopic surgery. It also provides improved ergonomics in the field of surgery. Robotic surgery has several advantages in colorectal operations that require complex minimally invasive skills including anorectal malformations, Hirschsprung disease, and inflammatory bowel disease. In this section, we discuss the key aspects of colorectal surgery where robotic instrumentation seems ideal.


Subject(s)
Colorectal Neoplasms , Colorectal Surgery , Digestive System Surgical Procedures , Inflammatory Bowel Diseases , Laparoscopy , Robotic Surgical Procedures , Adult , Humans , Child , Colorectal Neoplasms/surgery
2.
J Pediatr Surg ; 57(1): 135-140, 2022 Jan.
Article in English | MEDLINE | ID: mdl-34670678

ABSTRACT

PURPOSE: Intercostal Nerve Cryoablation (INC) has significantly improved pain control following the Nuss repair of pectus excavatum (PE). This study sought to evaluate patients undergoing the Nuss repair with INC compared to the Nuss repair with an ERAS protocol, INC, and intercostal nerve blocks (INB). METHODS: In June 2020, a new protocol was implemented involving surgery, anesthesia, nursing, physical therapy, and child life with the goal of safe same day discharge for patients undergoing the Nuss repair. They were compared to a control group who underwent the Nuss repair with INC alone in 2017-2019. The primary outcome measure was hospital length of stay (LOS) in hours, secondary outcomes were number of patients discharged on postoperative day (POD) 0, and returns to the emergency department (ED), urgent care (UC), and operating room (OR). RESULTS: The characteristics between the groups were the same (Table 1). The mean LOS was 11.8 h in the INB group versus 58.2 h in the INC group, p < 0.01. 10 of 15 patients in the INB group went home on POD 0 (average of 5.5 h postop), versus 0 patients in the INC only group, p < 0.01. Five patients in the INB stayed overnight. Two patients stayed owing to anxiety, one owing to urinary retention, one owing to nausea, and one owing to drowsiness. None stayed for pain control. Four patients in the INC group returned to the ED for pain control, versus 0 in the INB group, and 1 patient in the INB returned to UC for constipation. CONCLUSIONS: The majority of patients undergoing the Nuss repair of PE with a multidisciplinary regimen of pre and postoperative nursing education, precise intraoperative anesthesia care, performance of direct vision INB and INC, as well as careful surgery can go home on the day of surgery without adverse outcomes or unanticipated returns to the hospital. LEVEL-OF-EVIDENCE: Level II.


Subject(s)
Anesthesia, Conduction , Funnel Chest , Child , Funnel Chest/surgery , Humans , Pain, Postoperative/etiology , Patient Discharge , Retrospective Studies , Treatment Outcome
3.
J Pediatr Surg ; 57(9): 34-38, 2022 Sep.
Article in English | MEDLINE | ID: mdl-33678403

ABSTRACT

PURPOSE: The use of intercostal nerve cryoablation (INC) has been an effective modality for treating pain in patients undergoing pectus excavatum (PE) repair. This study sought to evaluate if PE patients undergoing Nuss procedures with INC and intercostal nerve block (INB) could safely be discharged the same day of surgery. METHODS: A prospective study with IRB approval of 15 consecutive patients undergoing PE Nuss repair with INC, INB, and an enhanced recovery after surgery (ERAS) protocol was conducted. The primary outcome measure was hospital length of stay (LOS) in hours. Secondary variables included same day discharge, postoperative complications, emergency department (ED) visits, urgent care (UC) visits, opioid use, and return to the operating room (OR). RESULTS: LOS averaged 11.9 h amongst 15 patients. Ten patients (66.7%) went home on postoperative day (POD) 0, and the rest went home on POD 1. No patients stayed in the hospital due to pain. Reasons for failure to discharge included urinary retention, drowsiness, vomiting, and anxiety, but not pain. No patients were readmitted to the ED. One patient visited UC for constipation. One patient had bar migration requiring return to the OR for revision. Ten (66.7%) patients did not use opioids after discharge. CONCLUSIONS: Same day discharge is feasible and safe in PE patients undergoing Nuss procedure with INC and INB. INC with INB can adequately control pain without significant complications. Same day discharge can be safely considered for PE patients undergoing Nuss procedure with INC with INB. TYPE OF STUDY: Prognosis study LEVEL-OF-EVIDENCE RATING: Level II.


Subject(s)
Funnel Chest , Funnel Chest/surgery , Humans , Pain, Postoperative/etiology , Pain, Postoperative/therapy , Patient Discharge , Prospective Studies , Retrospective Studies
4.
Pediatr Surg Int ; 37(1): 67-75, 2021 Jan.
Article in English | MEDLINE | ID: mdl-33210165

ABSTRACT

PURPOSE: The use of intercostal nerve cryoablation (INC) is becoming increasingly common in patients undergoing pectus excavatum (PE) repair. This study sought to evaluate the use of INC compared to traditional use of thoracic epidural (TE). METHODS: A retrospective review of 79 patients undergoing PE repair with either INC or TE from May 2009 to December 2019 was conducted. The operations were performed by four surgeons who worked together at four different hospitals and have the same standardized practice. The primary outcome measure was hospital length of stay (LOS). Secondary variables included surgical time, total operating room time, operating room time cost, total hospital cost, inpatient opioid use, long-term opioid use after discharge, and postoperative complications. RESULTS: LOS decreased to 2.5 days in the INC group compared to 5 days in the TE group (p < 0.0001). Surgical time was increased in the INC group, but there was no difference in total OR time. The INC group experienced significantly lower hospital costs. Total hospital opioid administration was significantly lower in INC group, and there was a significant decrease in long-term opioid use in the INC group. CONCLUSIONS: INC is a newer modality that decreases LOS, controls pain, and results in overall cost savings. We recommend that INC be included in the current practice for postoperative pain control in PE patients undergoing Nuss procedure.


Subject(s)
Analgesics, Opioid/administration & dosage , Cryosurgery/methods , Funnel Chest/surgery , Length of Stay/statistics & numerical data , Pain, Postoperative/drug therapy , Adolescent , Adult , Child , Female , Humans , Intercostal Nerves/surgery , Male , Operative Time , Postoperative Complications , Retrospective Studies , Young Adult
5.
J Laparoendosc Adv Surg Tech A ; 30(12): 1257-1262, 2020 Dec.
Article in English | MEDLINE | ID: mdl-33202165

ABSTRACT

Introduction: Cloaca malformation repair strategy is strongly dictated by common channel and urethral lengths. Mid to long common channel cloacas are challenging and often require laparotomy for dissection of pelvic structures. The balance of common channel and urethral lengths often dictates the approach for reconstruction. Laparoscopy has been utilized for rectal dissection but not for management of the urogenital (UG) structures. We hypothesized that laparoscopy could be applied to UG separation in reconstruction of cloaca malformations. Methods: Records were reviewed for 9 children with cloaca who underwent laparoscopic rectal mobilization and UG separation. Clinical parameters reviewed included demographics, relevant anatomic lengths, operative duration, transfusion requirements, and perioperative complications. Results: Repair was perfomed at a median (interquartile range) age of 12 (7, 15) months. Common channel length as measured by cystoscopy was 3.5 (3.3, 4.5) cm. There were no intraoperative complications. Transfusion requirements were minimal. Postoperative length of stay was 6 (5, 11) days. One patient developed a urethral web and 2 developed vaginal stenosis. One patient later underwent a laparotomy for obstruction due to a twisted rectal pull-through. Conclusions: Laparoscopic rectal mobilization and UG separation in long common channel cloaca are safe and well tolerated. Laparoscopy affords full evaluation of Mullerian structures and enables separation of the common UG wall, which may ultimately enhance long-term urinary continence.


Subject(s)
Cloaca/surgery , Laparoscopy/methods , Rectum/surgery , Urethra/surgery , Urologic Surgical Procedures/methods , Female , Humans , Infant , Male , Retrospective Studies
6.
Perm J ; 232019.
Article in English | MEDLINE | ID: mdl-31926567

ABSTRACT

BACKGROUND: Morgagni hernias are rare, with a reported incidence of 2% to 5% of congenital diaphragmatic hernias. OBJECTIVES: To review a laparoscopic technique to repair Morgagni hernias in pediatric patients. METHODS: Retrospective chart review of pediatric patients who underwent minimally invasive repair of a Morgagni hernia from November 2009 to September 2017 within a defined population. RESULTS: During an 8-year period, 15 patients with Morgagni hernias were identified. Four patients with Morgagni hernias were excluded because they had open repairs. Eleven Morgagni hernias were repaired through a completely minimally invasive approach. Three repairs were completed using a soft-tissue patch (Gore-Tex patch, W L Gore & Associates Inc, Flagstaff, AZ). All minimally invasive repairs were completed with transfascial sutures using an endoscopic suturing device (Endo Close, Covidien/Medtronic, Fridley, MN) and 2-0 nonabsorbable synthetic sutures with extracorporeal knot tying. Median follow-up was 40 months (range = 2.6 months to 7.3 years). No patients had postoperative pectus excavatum defects. There were no recurrences. CONCLUSION: Morgagni hernias are amenable to minimally invasive repair with this simple technique. With large defects, synthetic patches should be used. Recurrences are rare, and morbidity is low.


Subject(s)
Hernias, Diaphragmatic, Congenital/surgery , Minimally Invasive Surgical Procedures/methods , Suture Techniques , Child, Preschool , Humans , Infant , Infant, Newborn
7.
J Pediatr Surg ; 53(12): 2488-2490, 2018 Dec.
Article in English | MEDLINE | ID: mdl-30270119

ABSTRACT

BACKGROUND: Reconstruction of complex chest wall deformities is a surgical challenge. A new technique can improve long-term outcomes and result in high patient satisfaction. METHODS: A multicenter study was conducted on pediatric patients undergoing complex chest wall reconstruction between September 2015 and January 2018. The evolution of the technique using open reduction and internal fixation (ORIF) with SternaLock® and RibFix® to repair chest wall deformities is described. RESULTS: Seventeen patients underwent complex chest wall reconstruction with ORIF. Eight patients had severe or recurrent pectus excavatum, five patients had pectus carinatum, and four patients had complex chest wall fractures or other anomalies causing significant chronic pain. Up to three SternaLock® plates and four RibFix® plates were used for each procedure. Median length of hospital stay after surgery was four days. Median follow-up time was 12 months (range 2-30). There were no postsurgical complications. There was 100% patient satisfaction in postoperative recovery and cosmesis. CONCLUSION: ORIF using SternaLock® and RibFix® is an effective method of reconstructing complex chest wall deformities. This technique improves physical stability without the requirement of a subsequent surgery and enhances overall patient satisfaction. High volume centers should integrate this novel approach for challenging chest wall reconstruction. TYPE OF STUDY: Treatment study: case series. LEVEL OF EVIDENCE: Level IV.


Subject(s)
Musculoskeletal Abnormalities/surgery , Orthopedic Procedures/methods , Plastic Surgery Procedures/methods , Thoracic Surgical Procedures/methods , Thoracic Wall/surgery , Adolescent , Adult , Child , Female , Humans , Length of Stay/statistics & numerical data , Male , Patient Satisfaction/statistics & numerical data , Retrospective Studies , Thoracic Wall/abnormalities , Treatment Outcome , Young Adult
8.
J Pediatr Surg ; 53(9): 1811-1814, 2018 Sep.
Article in English | MEDLINE | ID: mdl-29246399

ABSTRACT

INTRODUCTION: The ability to use detailed, accurate current procedural terminology (CPT) codes is a key component of effective research. We examined the effectiveness of CPT codes to accurately reflect care in patients undergoing surgery for necrotizing enterocolitis (NEC). METHODS: A multicenter retrospective analysis of operations on patients with NEC was conducted across 4 institutions between 2011 and 2016. Correlation between operative dictation and CPT coding was analyzed. RESULTS: A total of 124 patients with NEC diagnosis undergoing exploratory abdominal operations were identified. NEC was improperly diagnosed in 25 patients, who were excluded from further analysis. Of the 99 patients reviewed, the initial exploratory abdominal operation was coded inaccurately in 58 cases (59%). Within these, 15 (26%) had multiple coding errors such that the nature of the original operation was not discernable from the applied codes. Inaccurate codes often did not describe the presence of a mucous fistula (n=27, 44%), ostomy (n=24, 39%), or extra segments of bowel resected (n=9, 16%). The length of bowel resected is not currently described by any CPT codes. CONCLUSION: CPT coding for abdominal operations does not sufficiently reflect complexity of pediatric surgeries. This study highlights the significance of this inadequacy and its implications in future database studies in the era of electronic medical records. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Clinical research study.


Subject(s)
Current Procedural Terminology , Electronic Health Records , Enterocolitis, Necrotizing/classification , Enterocolitis, Necrotizing/surgery , Child , Databases, Factual , Female , Humans , Infant, Newborn , Male , Retrospective Studies
10.
Respiration ; 89(6): 534-8, 2015.
Article in English | MEDLINE | ID: mdl-25924848

ABSTRACT

BACKGROUND: Congenital central hypoventilation syndrome (CCHS) is a rare disorder affecting central control of breathing. Thus, patients require lifelong assisted ventilation. Diaphragm pacing (DP) may permit decannulation in those who are ventilator dependent only during sleep. OBJECTIVE: The purpose of this study is to determine if patients with CCHS can be successfully ventilated by DP without tracheostomy. METHODS: We reviewed the records of 18 CCHS patients (mean age 19.5 ± 10.1 years; 44% female) who were ventilated by DP only during sleep. RESULTS: Prior to diaphragm pacer implantation surgery, 14 CCHS patients had been using home portable positive pressure ventilation (PPV) via tracheostomy, 1 had been on PPV via endotracheal tube, and 3 had been using noninvasive PPV (NPPV). Of the patients with tracheostomy prior to DP (n = 15), 11 (73%) were decannulated and ventilated successfully by DP without tracheostomy. Of all the patients reviewed (n = 18), 13 (72%) were successfully ventilated by DP without tracheostomy. Obesity prevented successful DP without tracheostomy in 1 patient, and upper airway obstruction prevented success in another patient. Snoring and/or obstructive apneas were present in some patients, but they were improved by diaphragm pacer changes, adenotonsillectomy, and/or use of nasal steroids. CONCLUSIONS: DP without tracheostomy can be successfully achieved in patients with CCHS. Snoring and obstructive apneas, when present, can be managed by diaphragm pacer changes and medical therapies. Obesity can pose a challenge to successful DP.


Subject(s)
Diaphragm , Electric Stimulation Therapy/methods , Hypoventilation/congenital , Sleep Apnea, Central/therapy , Adolescent , Adult , Child , Cohort Studies , Female , Humans , Hypoventilation/complications , Hypoventilation/therapy , Male , Noninvasive Ventilation , Obesity/complications , Positive-Pressure Respiration , Retrospective Studies , Sleep Apnea, Central/complications , Sleep Apnea, Obstructive/complications , Tracheostomy , Treatment Outcome , Young Adult
11.
J Laparoendosc Adv Surg Tech A ; 24(5): 359-61, 2014 May.
Article in English | MEDLINE | ID: mdl-24410634

ABSTRACT

Recurrent gastroesophageal reflux is a common complication after fundoplication procedures. We report our experience with laparoscopic redo Nissen fundoplications in pediatric patients with a history of open antireflux procedure. The medical records of all patients with a history of open antireflux procedure who underwent a subsequent laparoscopic redo Nissen fundoplication were reviewed. One hundred eighty laparoscopic Nissen fundoplications were performed between September 2004 and September 2012; 23 were redo procedures. Twelve patients had a history of prior open fundoplication. Average time between operations was 113.7±64 months. Seven patients presented with emesis, 4 with aspiration pneumonia, and 1 with clinical reflux. Eight had a history of cerebral palsy and/or seizure disorder. Laparoscopic revision was completed in 100% of the patients, with no intraoperative complications. Average operative time was 177.5±86 minutes. Seven patients were able to resume feeds on postoperative Day 1. Median length of stay was 3 days. Median follow-up was 21 months. One patient required a redo antireflux procedure 8 months later for persistent dysphagia. Thus laparoscopic revision Nissen fundoplication after a prior open antireflux procedure is feasible and safe.


Subject(s)
Fundoplication/methods , Gastroesophageal Reflux/surgery , Laparoscopy/methods , Adolescent , Adult , Child , Child, Preschool , Esophagus/surgery , Feasibility Studies , Female , Follow-Up Studies , Gastroesophageal Reflux/complications , Gastroesophageal Reflux/diagnostic imaging , Hernia, Hiatal/complications , Hernia, Hiatal/diagnostic imaging , Humans , Length of Stay , Male , Operative Time , Radiography , Reoperation , Retrospective Studies , Young Adult
12.
J Laparoendosc Adv Surg Tech A ; 24(5): 362-5, 2014 May.
Article in English | MEDLINE | ID: mdl-24195783

ABSTRACT

BACKGROUND/PURPOSE: Choledocholithiasis is uncommon in the pediatric population. Techniques for common bile duct stone extraction are still not well established. This article aims to describe safe and applicable techniques for pediatric common bile duct exploration. MATERIALS AND METHODS: This was a retrospective review of a prospectively maintained database of two pediatric surgeons for patients undergoing laparoscopic common bile duct exploration at two tertiary-care centers from April 2008 to March 2012. RESULTS: For 39 patients under 15 years of age undergoing laparoscopic cholecystectomy, 10 cholangiograms were performed, and 8 were found to have filling defects. Seven patients underwent successful laparoscopic common bile duct exploration with documentation of stone clearance, and 1 patient was sent for postoperative endoscopic retrograde cholangiopancreatography with stone extraction. Eleven patients had cholecystectomy performed by single-incision laparoscopic surgery, but none of these had cholangiograms or common bile duct explorations. Various methods of stone clearance were used, including the use of saline flush, balloon catheters, nitinol stone extractors, and the aide of glucagon. Depending on patient size, a choledochosope or a ureteroscope was used. There were no complications and no conversions to open surgery. CONCLUSIONS: Laparoscopic common bile duct exploration is feasible in the pediatric population, using many of the instruments readily available in the standard operating room. With an armamentarium of tools and techniques, the method of stone extraction can be tailored to the patient and situation.


Subject(s)
Cholecystectomy, Laparoscopic/methods , Gallstones/surgery , Adolescent , Child , Child, Preschool , Cholangiography , Female , Gallstones/diagnosis , Humans , Infant , Length of Stay , Male , Monitoring, Intraoperative , Operative Time , Retrospective Studies
13.
Perm J ; 17(1): 11-4, 2013.
Article in English | MEDLINE | ID: mdl-23596362

ABSTRACT

CONTEXT: Increasing popularity of strong magnets as toys has led to their ingestion by children, putting them at risk of potentially harmful gastrointestinal tract injuries. OBJECTIVES: To heighten physician awareness of the potential complications of magnetic foreign body ingestion, and to provide an updated algorithm for management of a patient who is suspected to have ingested magnets. DESIGN: A retrospective review of magnet ingestions treated over a two-year period at our institutions in the Southern California Permanente Medical Group. Data including patient demographics, clinical information, radiologic images, and surgical records were used to propose a management strategy. RESULTS: Five patients, aged 15 months to 18 years, presented with abdominal symptoms after magnet ingestion. Four of the 5 patients suffered serious complications, including bowel necrosis, perforation, fistula formation, and obstruction. All patients were successfully treated with laparoscopic-assisted exploration with or without endoscopy. Total days in the hospital averaged 5.2 days (range = 3 to 9 days). Average time to discharge following surgery was 4 days (range = 2 to 7 days). Ex vivo experimentation with toy magnetic beads were performed to reveal characteristics of the magnetic toys. CONCLUSIONS: Physicians should have a heightened sense of caution when treating a patient in whom magnetic foreign body ingestion is suspected, because of the potential gastrointestinal complications. An updated management strategy is proposed that both prevents delays in surgical care and avoids unnecessary surgical exploration.


Subject(s)
Foreign Bodies/etiology , Gastrointestinal Diseases/etiology , Magnets , Play and Playthings , Adolescent , Algorithms , Child , Child, Preschool , Disease Management , Eating , Female , Foreign Bodies/surgery , Gastrointestinal Diseases/surgery , Humans , Infant , Male , Retrospective Studies
14.
J Pediatr Surg ; 48(3): e37-9, 2013 Mar.
Article in English | MEDLINE | ID: mdl-23480947

ABSTRACT

The hepatic portoenterostomy (HPE) is the accepted initial operation for biliary reconstruction for biliary atresia, but in a select group of patients with patent distal extrahepatic bile ducts (PDEBD), a hepatic portocholecystostomy (HPC) may also be considered. A10 year old boy presented with sudden onset of jaundice following a successful HPC at 2 months of age. Radiographic evaluation revealed excretion into a distended gallbladder with distal biliary obstruction. He underwent a successful Roux-en-Y cholecystojejunostomy and remains jaundice-free two years later. Cholecystojejunostomy is an effective salvage operation for patients who develop late distal biliary obstruction after an HPC.


Subject(s)
Biliary Atresia/surgery , Cholecystostomy/methods , Jejunostomy/methods , Child , Humans , Liver/surgery , Male , Remission Induction , Time Factors , Treatment Failure
15.
J Pediatr Surg ; 47(9): 1754-6, 2012 Sep.
Article in English | MEDLINE | ID: mdl-22974618

ABSTRACT

In children, pyogenic granulomas are most commonly cutaneous benign vascular lesions but can also present in the gastrointestinal tract. When they occur in the intestine, they can cause acute or chronic gastrointestinal bleeding. We present an unusual case of rectal pyogenic granuloma and our management strategy.


Subject(s)
Granuloma, Pyogenic/surgery , Intestinal Mucosa/surgery , Rectal Diseases/surgery , Rectum/surgery , Adolescent , Anal Canal , Granuloma, Pyogenic/diagnosis , Humans , Male , Rectal Diseases/diagnosis
16.
Perm J ; 16(3): 25-7, 2012.
Article in English | MEDLINE | ID: mdl-23012595

ABSTRACT

BACKGROUND: Ultrasound guidelines for hypertrophic pyloric stenosis (HPS) have fixed minimum measurements and do not account for variation in patient weight or age. We sought to determine if preoperative pyloric measurements correlated with weight and age in patients with surgically proven HPS. METHODS: A retrospective analysis was conducted of 189 patients with HPS treated at a single institution over a 5-year period (2005 to 2010). Pearson correlation and linear regression analyses were used to determine if there were statistically significant associations between these combinations of factors: age and pyloric muscle thickness, weight and pyloric muscle thickness, age and pyloric length, and weight and pyloric length. RESULTS: Patients' mean age was 4.6 weeks (range, 1 to 17 weeks). Their mean weight was 3.9 kg (range, 2.5 to 8.0 kg). Mean pyloric muscle thickness was 0.42 cm (range, 0.18 to 0.86 cm), and mean pyloric length was 1.89 cm (range, 0.8 to 2.8 cm). Pearson correlation coefficient analysis showed a significant relationship between age and muscle thickness (r = 0.35, p < 0.001) as well as weight and muscle thickness (r = 0.24, p = 0.001). No significant relationship existed between pyloric length and age or weight. Linear regression analysis demonstrated similar results. CONCLUSION: In patients with HPS, pyloric muscle thickness was directly related to age and weight. Practitioners should be aware that smaller and younger infants with a clinical diagnosis of HPS may still truly have HPS even though the minimum diagnostic criterion for muscle thickness or length is not found on ultrasound.


Subject(s)
Age Factors , Body Weight , Muscles/diagnostic imaging , Pyloric Stenosis, Hypertrophic/diagnostic imaging , Pylorus/diagnostic imaging , Guidelines as Topic , Humans , Hypertrophy , Infant , Infant, Newborn , Linear Models , Retrospective Studies , Ultrasonography
17.
Pediatr Surg Int ; 28(4): 435-8, 2012 Apr.
Article in English | MEDLINE | ID: mdl-22212493

ABSTRACT

We present a case of an adolescent with lower gastrointestinal bleeding caused by a colorectal venous malformation (VM) with concomitant portal hypertension. After an episode of massive gastrointestinal bleeding, we performed an extended right hemicolectomy and resection of the VM and selective portosystemic shunt. Here, we present the case and review the literature regarding portal hypertension and gastrointestinal vascular malformations. Additionally, we discuss the physiologic and hemodynamic effects of gastrointestinal vascular malformations on the portal system.


Subject(s)
Arteriovenous Malformations/complications , Colon/blood supply , Gastrointestinal Hemorrhage/etiology , Hypertension, Portal/complications , Adolescent , Arteriovenous Malformations/surgery , Colon/surgery , Humans , Male
18.
J Pediatr Surg ; 45(11): e39-42, 2010 Nov.
Article in English | MEDLINE | ID: mdl-21034928

ABSTRACT

Prune belly syndrome (PBS), megacystis-microcolon-intestinal hypoperistalsis (MMIH), and omphalocele-exstrophy of the bladder-imperforate anus-spine abnormalities complex (OEIS) are rare congenital malformations of the newborn that lead to incomplete formation of the gastrointestinal and genitourinary tract systems. To date, incomplete mesodermal development is identified as the cause for all these complex genetic syndromes even if the etiology is still unknown. We present an original case sharing characteristics common to PBS, MMIH, and OEIS complex, without a clear inclination toward any particular one. This case hints toward a common pathway in the creation of the 3 syndromes. We hypothesize that they are a spectrum of malformations based on the time frame when the mesoderm fails to create a normal interaction between infraumbilical mesoderm, urorectal septum, lumbosacral somites in the formation of the abdominal wall and the genitourinary and lower gastrointestinal tracts.


Subject(s)
Abnormalities, Multiple/diagnosis , Cloaca/abnormalities , Hernia, Umbilical/diagnosis , Prune Belly Syndrome/diagnosis , Urachus/abnormalities , Abnormalities, Multiple/surgery , Diagnosis, Differential , Digestive System Surgical Procedures/methods , Female , Follow-Up Studies , Gynecologic Surgical Procedures/methods , Hernia, Umbilical/surgery , Humans , Infant, Newborn , Magnetic Resonance Imaging , Prune Belly Syndrome/surgery , Plastic Surgery Procedures/methods , Urography
19.
J Pediatr Surg ; 44(12): 2327-9, 2009 Dec.
Article in English | MEDLINE | ID: mdl-20006019

ABSTRACT

PURPOSE: Inguinal hernias in infants and children may not be evident during visits to the pediatric surgeon's office. Preoperative photographic confirmation of the hernia may be helpful and accurate. This study retrospectively assesses the preoperative examination, photographic evidence, operative findings, and complications of these patients. METHODS: For a 30-month period, children with a history of a possible inguinal bulge were assessed by clinical examination. If no bulge was detected, the parents were given the option of sending a photograph to the surgeon. Patients underwent surgery based solely upon the history and definitive image. RESULTS: For 30 months, 25 children were evaluated for inguinal pathology but had no hernia on clinical examination. Based solely upon the history and the digital image, 23 of these patients underwent surgery. All of the patients who were diagnosed with a hernia by history and imaging alone had an operatively confirmed hernia. The remaining 2 patients have not developed a hernia with continued observation. CONCLUSION: Photographic images are a reliable way to document the presence and laterality of inguinal hernias. Reliance upon digital transmission of images avoids the need for repeat office visits, saving considerable physician and parental time and expense.


Subject(s)
Hernia, Inguinal/diagnosis , Parents , Photography/methods , Physical Examination/methods , Cell Phone/statistics & numerical data , Child , Child, Preschool , Diagnosis, Differential , Electronic Mail/statistics & numerical data , Female , Functional Laterality , Groin/pathology , Hernia, Inguinal/pathology , Hernia, Inguinal/surgery , Humans , Infant , Laparoscopy/methods , Male , Medical History Taking , Office Visits , Preoperative Care , Retrospective Studies
20.
J Pediatr Surg ; 43(12): 2213-5, 2008 Dec.
Article in English | MEDLINE | ID: mdl-19040937

ABSTRACT

BACKGROUND: Prenatal closure of the umbilical ring in gastroschisis may result in an amorphous, nonviable appearing extracorporeal tissue that is resected during the repair. However, it is unclear whether such remnant intestine is truly nonviable. METHODS AND RESULTS: We examined the outcomes of patients when this tissue is preserved. We identified 8 patients who presented with a closing gastroschisis and a mass of tissue connected by a vascular pedicle. Four patients underwent abdominal exploration with resection of the mass and gastroschisis closure. Histologic examination revealed normal intestinal wall architecture. All patients in this group developed short bowel syndrome, requiring long-term parenteral nutrition. Conversely, 4 patients underwent abdominal exploration with internalization of the remnant tissue, a maneuver referred to as "parking," along with either silo placement, or primary closure of the gastroschisis. At re-exploration, 3 of 4 patients were found to have viable intestine, and bowel continuity was reestablished. The mean parenteral nutrition requirement for this group was significantly shorter than the resected group. CONCLUSION: In this series, we show that this amorphous tissue, when preserved, may exhibit normal intestinal architecture and absorptive function. Therefore, such remnant tissue should be preserved as it may significantly increase bowel length and minimize parenteral nutrition requirement.


Subject(s)
Gastroschisis/surgery , Intestine, Small/pathology , Postoperative Complications/prevention & control , Short Bowel Syndrome/prevention & control , Anastomosis, Surgical , Anthropometry , Atrophy , Enteral Nutrition , Female , Gastroschisis/complications , Gastroschisis/embryology , Gastroschisis/pathology , Humans , Infant, Newborn , Intestinal Atresia/etiology , Intestine, Small/surgery , Length of Stay/statistics & numerical data , Male , Negative-Pressure Wound Therapy , Parenteral Nutrition, Total , Reoperation , Retrospective Studies , Time Factors
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