Perioperative outcomes and the effects of anesthesia in congenital central hypoventilation patients.

PURPOSE: Patients with congenital central hypoventilation syndrome (CCHS) have autonomic dysfunction and lack ventilatory responses to hypoxemia and hypercarbia and thus are prone to adverse events during general anesthesia. The objective of this study was to describe the perioperative outcomes of patients with CCHS who were undergoing diaphragm pacer (DP) implantation surgeries under general anesthesia. METHODS: A retrospective cohort study was conducted on patients with CCHS who underwent DP implantation surgeries at CHLA between January 2000 and May 2016. Charts were reviewed for demographics, PHOX2B genotype, ventilatory support, comorbidities, anesthesia administered, and perioperative courses. RESULTS: Of 19 patients with CCHS (58% female) mean age at surgeries was 8.6 ± 5.8 years. Seventeen patients were ventilator-dependent during sleep only; two were ventilator dependent 24 h per day. Mean surgery duration was 3.1 ± 0.5 h. Seventeen patients were extubated to PPV via tracheostomy in the OR. Two patients were extubated to NPPV on postoperative day (POD) 1. Mean transition time to home ventilator or NPPV was 3.0 ± 2.2 days, and mean hospital stay was 5.0 ± 2.1 days. One patient premedicated without ventilatory support developed hypoxemia and hypoventilation. Ten patients (52%) had intraoperative events such as bradycardia, hypotension, significant hypoxemia, and bronchospasm. Fifteen patients had postoperative events. Hypoxemia, pneumonia, and atelectasis accounted for most of perioperative complications. One patient experienced seizure on POD 2 due to hypercarbia. CONCLUSION: Patients with CCHS are vulnerable to the cardiorespiratory effects of sedative and anesthetic agents. Therefore, they require vigilant monitoring and optimal ventilatory support in the perioperative period.

Screening for Child Abuse in Children With Isolated Skull Fractures.

OBJECTIVE: Head trauma is the most common cause of death from child abuse, and each encounter for recurrent abuse is associated with greater morbidity. Isolated skull fractures (ISF) are often treated conservatively in the emergency department (ED). We determined patterns of physical abuse screening in a children's hospital ED for children with ISF. METHODS: A retrospective review was performed for children aged 3 years and younger who presented to the ED with ISF from January 1, 2015 to December 31, 2019. Children were stratified by age (<12 mo, ≥12 mo) and witnessed versus unwitnessed injury. Primary outcome was social work (SW) assessment to prescreen for abuse. Secondary outcomes were suspicion for abuse based on Child Protective Services (CPS) referral and subsequent ED encounters within 1 year. RESULTS: Sixty-six ISF patients were identified. Of unwitnessed injury patients aged younger than 12 months (n = 17/22), 88.2% (n = 15/17) underwent SW assessment and 47.1% (n = 8/17) required CPS referral. Of witnessed injury patients aged younger than 12 months (n = 23/44), 60.9% (n = 14/23) underwent SW assessment, with no CPS referrals. Overall, 18.2% (n = 4/22) unwitnessed and 20.5% (n = 9/44) witnessed injury patients returned to our ED: 2 were aged younger than 12 months and had recurrent trauma. CONCLUSIONS: To decrease risk of missed physical abuse, SW consultation should be considered for all ISF patients.

Meeting in the middle: pediatric abdominal wall reconstruction for omphalocele.

BACKGROUND: Omphalocele is a congenital abdominal wall defect with an incidence of 1/4,200 births. Repair timing varies from the neonatal period to the first few years of life. Surgical technique has changed over the last two decades. We sought to establish improved surgical/ventilation protocols for patients with omphaloceles requiring abdominal reconstruction. METHODS: An IRB-approved retrospective review was performed on patients with omphalocele requiring abdominal wall reconstruction by Plastics and/or Pediatric Surgery at a pediatric tertiary-care referral center (January 2006-July 2021). Birth history, comorbidities, surgical details, ventilation data, complications/recurrence were extracted. RESULTS: Of 129 patients screened, seven required Plastic Surgery involvement. Defect size was 102.9 cm2 (range: 24-178.5); five patients required component separation; zero patients received mesh; zero complications/recurrences were recorded. Two patients required postoperative ventilation for 2.5 days, based on increased peak inspiratory pressures at surgery stop versus start time. CONCLUSION: Patients with large defects secondary to omphalocele benefit from collaboration between Pediatric and Plastic Surgery for component separation and primary fascial closure without mesh. Future research should follow patients who mature out of pediatric clinics to evaluate the incidence of hernias in adults with Plastic Surgery-repaired omphaloceles.

Diaphragm Pacing without Tracheostomy in Congenital Central Hypoventilation Syndrome Patients.

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.

Treatment of congenital pulmonary airway malformation induced hydrops fetalis via percutaneous sclerotherapy.

Large type II and III congenital pulmonary airway malformations (CPAMs) can cause pulmonary hypoplasia, non-immune hydrops fetalis and fetal demise. Fetal intervention is indicated if hydrops fetalis develops. In this report, we describe three cases of type II and III CPAMs complicated by hydrops and treated with percutaneous sclerotherapy by ethanolamine injection into the tumor. All 3 cases demonstrated reduction in size of the CPAM and resolution of the hydrops with subsequent delivery at term. We believe that fetal percutaneous sclerotherapy can be used as a minimally invasive palliative strategy to treat CPAM-induced hydrops fetalis. Further studies are needed to delineate the risks of this novel technique.

An unusual cause of diaphragm pacer failure in congenital central hypoventilation syndrome.

Congenital central hypoventilation syndrome is a rare genetic disorder affecting ventilatory response to hypercapnia and/or hypoxemia. We describe a case of diaphragm pacing (DP) failure in a 38-year-old woman with congenital central hypoventilation syndrome who used DP as ventilatory support only during sleep for 24 years. Diagnostic evaluation began with examination of external DP equipment, but adjustment did not elicit adequate diaphragm contractions. Clinical evaluation and transtelephonic monitoring showed absent function of the right pacer and diminished function of the left pacer. The patient had surgical exploration of her internal DP components. The operation revealed that the right pacer receiver had significant circumferential calcium accumulation. After replacement of the receivers in subcutaneous pockets closer to the skin surface, robust diaphragm contractions bilaterally occurred with stimulation. This case suggests DP failure can result from development of calcification and increased distance from the skin surface to the receivers due to weight gain. CITATION: Kwon A, Lodge M, McComb JG, et al. An unusual cause of diaphragm pacer failure in congenital central hypoventilation syndrome. J Clin Sleep Med. 2022;18(3):949-952.

Laparoscopic choledochal cyst excision: lessons learned in our experience.

BACKGROUND: Choledochal cyst (CDC) is a rare biliary disorder. Surgical treatment consists of CDC excision and biliary-enteric reconstruction. Recently, some institutions have reported successful CDC excision by using minimally invasive techniques. In this study, we report our experience with the laparoscopic management of CDC, with a focus on key operative maneuvers that enhance the likelihood of successful excision. METHODS: Following institutional review board approval, we performed a retrospective review of patients who underwent the laparoscopic excision of CDC and Roux-en-Y hepaticojejunostomy. Between October 2003 and November 2007, we performed laparoscopic CDC excision in 9 patients (8 female and 1 male). Median age was 4 years (range, 8 months to 16 years). There were 7 type I and 2 type IV cysts, according to Todani's classification. Average cyst size was 4.4 cm (range, 1.3-8.5). The procedures were performed by utilizing four or five trochars. RESULTS: Six of 9 children presented with preoperative pancreatitis, 1 with abdominal pain, 1 with jaundice, and 1 was found incidentally. Three patients required the conversion to laparotomy due to dense adhesions, secondary to pancreatitis. Six patients underwent successful laparoscopic procedures, 5 had complete cyst excisions, and 1 underwent a proximal excision with distal mucosectomy. Of the 3 patients who required conversion, 2 underwent complete excisions; the other underwent a proximal excision, distal mucosectomy. There were no intraoperative complications. One patient had a postoperative bile leak that required an open hepaticojejunostomy revision. Eight patients had an uneventful recovery. Oral feedings were resumed within an average of 3.4 days (range, 2-9). Average time to discharge was 6.1 days (range, 5-12). Average follow-up time was 18 months (range, 4-48). No further laboratory abnormalities were detected in any of the patients. CONCLUSIONS: Laparoscopic resection of CDC and Roux-en-Y hepaticojejunostomy in children is an excellent treatment option. Preoperative pancreatitis may cause increased technical difficulty, necessitating a conversion. Proximal excision with distal mucosectomy

Combined laparoscopic and thoracoscopic esophagectomy and gastric pull-up in a child.

Severe caustic burns to the esophagus may necessitate esophagectomy with replacement, if stictures form which are not amenable to dilation. Traditionally, the operation is performed via open transabdominal and/or thoracic approaches. Here we describe our minimally invasive approach, combining laparoscopy and thoracoscopy for esophagectomy, stomach tubularization and a cervical esophagogastrotomy. We successfully performed the procedure in a 4-year-old boy. There were no intraoperative complications, and his initial esophagram showed good patency with no leaks. However, one week postoperatively he was noted to have a retained foreign body and a minor anastomotic leak, which was most likely caused by the foreign body. He underwent a neck re-exploration,removal of the foreign body, and repair of the anastomotic leak. His subsequent hospital course was uneventful. He tolerated feedings and was discharged home on an unrestricted diet. The minimally invasive approach to esophagectomy and esophageal replacement is feasible and is an excellent option in select patients.

L1CAM mutation in association with X-linked hydrocephalus and Hirschsprung's disease.

X-linked hydrocephalus (XLH) is characterized by increased intracranial ventricle size and head circumference secondary to aqueduct of Sylvius congenital stenosis. Exceedingly rare is the concurrence of XLH and Hirschsprung's disease (HSCR) with a theoretical incidence of 1 in 125-250 million cases. Herein, we are describing a case of a patient with concurrent XLH and HSCR. The patient was delivered via cesarean section at 37 weeks gestation and underwent uneventful ventriculoperitoneal shunt placement. As a part of a workup for constipation, we performed a rectal biopsy, which was consistent with HSCR. Genetics testing showed hemizygous for R558X hemizygous mutation in the L1CAM gene. A C --> T nucleotide substitution in exon 13 resulted in replacement of an arginine codon with a stop codon, a nonsense mutation. Although it is widely accepted that HSCR represents the failure of early embryonic neural crest cells to migrate properly, the exact mechanism is not known. The association of HSCR with XLH in the presence of L1CAM mutations remains quite interesting because cell adhesion molecules are involved in the proper migration of neural components throughout the body. Additional studies are necessary to fully elucidate the relationship between XLH and HSCR in the presence of L1CAM mutations.

Thoracoscopic repair of neonatal diaphragmatic hernia.

INTRODUCTION: The use of minimally invasive surgery (MIS) in the neonatal population is increasing. Thoracoscopic intervention for congenital diaphragmatic hernia (CDH) is no exception. In this report, we describe our initial experience with thoracoscopic repair of left-sided diaphragmatic defects in neonates. MATERIALS AND METHODS: We performed retrospective chart reviews on all neonates who underwent thoracoscopic repair of CDH between November 2004 and January 2008. Neonates that underwent thoracoscopic repair were physiologically stable with resolved pulmonary hypertension and minimal to moderate ventilatory support. They had no associated cardiac anomalies. RESULTS: We identified 15 neonates with CDH who underwent thoracoscopic repair during the study period. Ten neonates underwent primary repair of the diaphragmatic defect. Five neonates with large defects required closure with a synthetic patch, which was placed thoracoscopically. The average operating room time was 134 minutes. There were no instances of intraoperative respiratory or cardiac instability. Three patients had a recurrence. One recurrence was seen after thoracoscopic patch repair. Two recurrences occurred following primary repair of left diaphragmatic hernias. There were no deaths. Follow-up has been 4-40 months. CONCLUSIONS: Neonatal MIS for CDH should be limited to stable patients. The ideal candidate is the newborn without associated anomalies, not requiring extracorporeal membrane oxygenation, on minimal ventilatory support, and without evidence of pulmonary hypertension. It is technically possible to perform thoracoscopic repair with a patch.

Ethanol-lock technique for persistent bacteremia of long-term intravascular devices in pediatric patients.

OBJECTIVES: To use the ethanol-lock technique (in conjunction with systemic antibiotics) to salvage central lines from removal and to prevent persistence of catheter-related infections among pediatric patients with long-term intravascular devices. DESIGN: Medical records of patients treated with ethanol locks were retrospectively reviewed from June 1, 2004, through June 22, 2005. SETTING: Childrens Hospital Los Angeles, Los Angeles, Calif, a tertiary care pediatric hospital. Patients Forty children with diverse underlying disorders were treated for 51 catheter-related infections using the Childrens Hospital Los Angeles ethanol-lock technique. INTERVENTIONS: Eligible infected central lines were instilled with a dose volume of 0.8 to 1.4 mL of 70% ethanol into the catheter lumen during 12 to 24 hours and then withdrawn. The volume of ethanol used was based on the type of intravascular device. MAIN OUTCOME MEASURES: Clearance of infection and incidence of recurrence. RESULTS: Of the 51 ethanol-lock treatments in 40 children, no catheters were removed because of persistent infection. Eighty-eight percent (45/51) of the treated episodes cleared without recurrence (defined as a relapse within 30 days with the same pathogen). Twelve (75%) of 16 polymicrobial isolates and 33 (94%) of 35 monomicrobial isolates were successfully treated. There were no adverse reactions or adverse effects reported. CONCLUSION: This retrospective study supports the use of the ethanol-lock technique in conjunction with systemic antibiotics as an effective and safe method to retain the use of a previously infected central venous catheter, decrease the need for line removal, and eradicate persistent pathogens in catheter-related infections.

Thoracoscopic placement of phrenic nerve pacers for diaphragm pacing in congenital central hypoventilation syndrome.

PURPOSE: Congenital central hypoventilation syndrome (CCHS), or Ondine's curse, is a rare disorder affecting central respiratory drive. Patients with this disorder fail to ventilate adequately and require lifelong ventilatory support. Diaphragm pacing is a form of ventilatory support which can improve mobility and/or remove the tracheostomy from CCHS patients. Little is known about complications and long-term outcomes of this procedure. METHODS: A single-center retrospective review was performed of CCHS patients undergoing placement of phrenic nerve electrodes for diaphragm pacing between 2000 and 2012. Data abstracted from the medical record included operation duration, ventilation method, number of trocars required, and postoperative and pacing outcomes. RESULTS: Charts of eighteen patients were reviewed. Mean surgical time was 3.3±0.7 hours. In all cases except one, three trocars were utilized for each hemithorax, with no conversions to open procedures. Five patients (27.8%) experienced postoperative complications. The mean ICU stay was 4.3±0.5 days, and the mean hospital stay is 5.7±0.3days. Eleven patients (61.1%) achieved their daily goal pacing times within the follow-up period. CONCLUSIONS: Thoracoscopic placement of phrenic nerve electrodes for diaphragmatic pacing is a safe and effective treatment modality for CCHS. Observed complications were temporary, and the majority of patients were able to achieve pacing goals.

Laparoscopic versus open treatment of congenital duodenal obstruction: multicenter short-term outcomes analysis.

BACKGROUND: Laparoscopic repair of congenital duodenal obstruction has become popularized over the past decade. Comparative data on outcomes, however, are sparse. We hypothesized that laparoscopic repair of congenital duodenal obstruction could be performed with similar outcomes to traditional open repair. PATIENTS AND METHODS: Medical records for all cases of congenital duodenal obstruction from 2005 to 2011 at three academic teaching hospitals were retrospectively reviewed. Patients were excluded from the analysis if they had confounding surgical diseases, did not have duodenoduodenostomy during the first hospital admission, had the repair performed before transfer from a referring hospital, or weighed less than 1.7 kg at the time of surgery. Analysis was performed as intention to treat, with laparoscopic converted to open cases included in the laparoscopic group. RESULTS: Sixty-four cases were included in the analysis (44 open, 20 laparoscopic). Baseline characteristics were similar between the two groups with the exception that the open group, on average, underwent repair later than the laparoscopic group (6 days versus 4 days, respectively). Seven laparoscopic cases were converted to an open procedure (35%), most commonly for difficulty in exposing the decompressed distal duodenum. Laparoscopic repair did take significantly longer than open repair (145 minutes versus 96 minutes, respectively), but clinical outcomes were similar. Complications were rare and were similar between methods of repair. Two patients in the laparoscopic group required subsequent open revision. CONCLUSIONS: Laparoscopic duodenoduodenostomy for congenital duodenal obstruction is a technically challenging procedure with a steep learning curve. Despite a relatively high conversion rate, clinical outcomes remained similar to the traditional open repair in selected patients.

Inversion herniotomy: a laparoscopic technique for female inguinal hernia repair.

PURPOSE: Laparoscopic inversion herniotomy (LIH) is a method of inguinal hernia repair for female patients. In this article, we report our technique and outcomes for this procedure. We propose that LIH is a valid alternative to conventional open herniorraphy. METHODS: Following institutional review board approval, we reviewed the charts of patients who underwent LIH or open inguinal hernia repair (OIH) from 2004 to 2007. LIH was performed with three instrument sites and no groin incision. The diagnosis, operative time, and complications were reviewed. Follow-up ranged from 5 months to 4 years. RESULTS: We performed 79 LIH repairs (15 bilateral, 23 left, and 26 right) in 63 female children. We also performed 76 contemporaneous OIH repairs in girls. The age range was 1 month to 18 years (median, 3.8 years). Operative times for both the unilateral and bilateral LIH repairs were significantly shorter than those for OIH (P = 0.001). There were 2 recurrences after laparoscopic inguinal hernia repair, both within 5 months following repair early in our experience, compared to no hernia recurrences with OIH. The first recurrence was repaired conventionally via an open technique. The second case was repaired laparoscopically. CONCLUSIONS: LIH is an effective method for the repair of pediatric indirect inguinal hernia in female patients. The procedure allows the evaluation of the asymptomatic contralateral side without the necessity of a second long inguinal incision.

State of the practice for pediatric surgery--career satisfaction and concerns. A report from the American Pediatric Surgical Association Task Force on Family Issues.

BACKGROUND: There has been increasing interest and concern raised in the surgical literature regarding changes in the culture of surgical training and practice, and the impact these changes may have on surgeon stress and the appeal of a career in surgery. We surveyed pediatric surgeons and their partners to collect information on career satisfaction and work-family balance. METHODS: The American Pediatric Surgical Association Task Force on Family Issues developed separate survey instruments for both pediatric surgeons and their partners that requested demographic data and information regarding the impact of surgical training and practice on the surgeon's opportunity to be involved with his/her family. RESULTS: We found that 96% of pediatric surgeons were satisfied with their career choice. Of concern was the lack of balance, with little time available for family, noted by both pediatric surgeons and their partners. CONCLUSION: The issues of work-family balance and its impact on surgeon stress and burnout should be addressed in both pediatric surgery training and practice. The American Pediatric Surgical Association is positioned to play a leading role in this effort.

Contemporary management of lipoblastoma.

PURPOSE: Lipoblastoma is a rare, benign, adipose tissue tumor. We report the largest single institution experience managing these uncommon neoplasms. METHODS: We retrospectively reviewed 32 cases of lipoblastoma entered in the pathology database at our institution between January 1991 and August 2005. We conducted a comprehensive literature review of lipoblastoma and summarized the results of the largest series published. RESULTS: Most patients presented with an enlarging, palpable, firm, nontender, mobile mass. The male-to-female ratio was 1.9:1. The anatomical distribution was trunk (n = 12), extremity (n = 12), groin (n = 5), and neck (n = 3). Average age at resection was 2.8 years (range, 2.6 months to 12 years). Thirty-one cases were completely excised, although 1 patient underwent staged partial excision to preserve nerve function. Chromosomal analysis performed in selected patients revealed characteristic aberrations in chromosome 8. Complications included keloid formation (n = 3), wound infection/dehiscence (n = 2), wound seroma (n = 1), and transient brachial plexus neurapraxia (n = 1). Average follow-up was 7.4 months (range, 1 day to 6.5 years); 2 patients were lost to follow-up. There were no recurrences. CONCLUSIONS: A staged approach with meticulous sparing of the neurovascular bundle provides excellent functional outcome for patients with large tumors. Nonmutilating surgical excision is the treatment of choice.