Short and Long-Term Outcomes in Hirschsprung Disease: Are the Syndrome-Associated Patients Really Doing Worse?

INTRODUCTION: Given the lack of data to help caregivers in the follow-up of Hirschsprung disease (HD), this study aimed to compare the functional outcomes of isolated Hirschsprung disease (I-HD) to syndrome-associated Hirschsprung disease (SA-HD) at 1, 3, 5, and 10 years. METHODS: A retrospective chart review of patients diagnosed with HD between January 1990 and May 2021 at our pediatric center was performed to collect data on patient characteristics, investigations, and treatments. Ninety-five patients were identified, of whom 76 were included in the study. SA-HD is defined as a syndrome known to be associated with HD or cognitive impairment. RESULTS: Patient characteristics were comparable between groups ( P > 0.05). There were 52 patients with I-HD and 24 with SA-HD. The patients median age was 9 days at diagnosis and 1.5 month at surgery. SA-HD patients became bowel continent at a significantly older age (mean age 8.43 vs 4.94 years, P = 0.0471) and received more bowel continence medications. At 5 years, SA-HD patients requiring ≥2 medications for bowel continence represented 54.5% versus 11.1% of I-HD patients ( P = 0.009). Lastly, SA-HD patients had urinary incontinence at a significantly older age ( P = 0.0136, 5 years). CONCLUSION: Clinicians should be aware that SA-HD patients are more prone to bladder dysfunction and became bowel continent at an older age than I-HD patients. They need more and prolonged bowel management medications, and other important complications need to be addressed in patient care. These results should prompt a longer follow-up period for these patients, especially in SA-HD.

Treatment and Outcomes of Congenital Ovarian Cysts A Study by the Canadian Consortium for Research in Pediatric Surgery (CanCORPS).

OBJECTIVE: We conducted a multicenter study to assess treatments and outcomes in a national cohort of infants with congenital ovarian cysts. SUMMARY BACKGROUND DATA: Wide variability exists in the treatment of congenital ovarian cysts. The effects of various treatment strategies on outcomes, specifically ovarian preservation, are not known. METHODS: Female infants diagnosed with congenital intra-abdominal cysts between 2013 and 2017 at 10 Canadian pediatric surgical centers were retrospectively evaluated. Sonographic characteristics, median time to cyst resolution, incidence of ovarian preservation, and predictors of surgery were evaluated. Subgroup analyses were performed in patients with complex cysts and cysts ≥40 mm in diameter. RESULTS: The study population included 189 neonates. Median gestational age at diagnosis and median maximal prenatal cyst diameter were 33 weeks and 40 mm, respectively. Cysts resolved spontaneously in 117 patients (62%), 14 (7%) prenatally, and the remainder at a median age of 124 days. Intervention occurred in 61 patients (32%), including prenatal aspiration (2, 3%), ovary sparing resection (14, 23%), or oophorectomy (45, 74%). Surgery occurred at a median age of 7.4weeks. Independent predictors of surgery included postnatal cyst diameter ≥40 mm [odds ratio (OR) 6.19, 95% confidence interval (CI) 1.66-35.9] and sonographic complex cyst character (OR 63.6, 95% CI 10.9-1232). There was no significant difference in the odds of ovarian preservation (OR 3.06, 95% CI 0.86 -13.2) between patients who underwent early surgery (n = 22) and those initially observed for at least 3 months (n = 131). CONCLUSIONS: Most congenital ovarian cysts are asymptomatic and spontaneously resolve. Early surgical intervention does not increase ovarian preservation.

Differentiating congenital ovarian cysts from other abdominal cystic lesions in female infants: A study by the Canadian Consortium for Research in Pediatric Surgery (CanCORPS).

PURPOSE: The origin of congenital abdominal cysts in the female fetus often dictates management. While most arise from the ovary and are often managed non-operatively, some are non-ovarian and are frequently removed. We analyzed a national sample of female infants with congenital abdominal cysts to elucidate prenatal and postnatal factors associated with the diagnosis of a non-ovarian cyst. METHODS: A retrospective cohort study of female infants who were prenatally diagnosed with abdominal cysts between 2013 and 2017 at 10 Canadian pediatric surgical centres was performed. Clinical characteristics, pre- and postnatal sonographic findings, and cyst trajectories were compared between patients with proven ovarian etiology and those with cysts arising from other organs. RESULTS: Of 185 infants with prenatally diagnosed abdominal cysts, 22 (12%) were non-ovarian, five of which had clear non-ovarian organ of origin on prenatal ultrasound. Comparison of the other 17 cysts with 163 congenital ovarian cysts showed the following factors to be associated with a non-ovarian origin: earlier gestational age at diagnosis (23.5 vs 33.5 weeks, p <0.001), smaller diameter on first prenatal ultrasound (15.8 vs. 39.7 mm, p <0.001), change in sonographic character from simple to complex (87% vs 22%, p <0.001), and postnatal sonographic characteristics of complex cyst (87% vs. 48%, p = 0.004). CONCLUSION: Clear organ of origin, diagnosis earlier in gestation, smaller initial prenatal cyst diameter, and sonographic cyst character change differentiate congenital non-ovarian cysts from their ovarian counterparts. These characteristics may be used to guide diagnosis and management.

Hereditary multiple intestinal atresia: thirty years later.

BACKGROUND: Hereditary multiple intestinal atresia (HMIA) is an unusual form of intestinal atresia with a presumed autosomal recessive mode of inheritance. The aim of this study was to review the authors' experience with this disease, 30 years after its first description. METHODS: All cases of HMIA treated at the authors' institution were reviewed with a particular focus on presence of close consanguinity in the families, prenatal diagnosis, radiologic and surgical findings, pathology report, and outcome. RESULTS: Sixteen cases were identified. Two patients were siblings (1 newborn and 1 aborted foetus) and close consanguinity was proven in 1 other case. Bowel obstruction was suspected on prenatal ultrasound scan in 6 patients, but HMIA could not be diagnosed specifically. Radiologic, surgical, and pathologic findings were compatible with the standard description of this disease in the literature. All the patients died. Mean survival time was 50 days. CONCLUSIONS: Thirty years after its first description, HMIA remains a disease without reliable prenatal diagnosis nor effective surgical therapy. An autosomal recessive mode of inheritance is suspected. Until accurate in utero diagnosis becomes available, children with HMIA should be oriented toward palliative care.

Pediatric spontaneous pneumothorax.

The aim of this study was to review pediatric spontaneous pneumothorax and describe diagnostic and therapeutic approaches and to review our institutional experience with spontaneous pneumothorax. A medline search using keywords pneumothorax and pediatric in both French and English from 1988 through May 2002 was performed, and pertinent papers regarding the natural history, diagnosis, imaging, and therapy were reviewed. A 10-year chart review of spontaneous pneumothorax recording data on demographics, diagnostic imaging, therapeutic approach, and outcomes was performed. Only 4 case series and one review article were produced by the medline search involving exclusively pediatric spontaneous pneumothrax. One hundred ninety-six other articles involved adult series with a minority of patients under 18 years of age. A subset of articles from this adult-based group were examined to assist in formulating recommendations for diagnosis, imaging, treatment, and surgical management of pediatric spontaneous pneumothorax. Few articles on this topic report randomized studies in the adult literature and none in the pediatric literature. The chart review yielded 53 cases of spontaneous pneumothorax in 40 patients with important data presented in the current review. Although few articles are able to provide best-evidence information, the diagnosis, imaging, treatment, and surgical management of patients with primary spontaneous pneumothorax is achieved with good results. Stronger data specific to pediatric patients are needed.

Laparoscopic appendectomy in children: A favorable alternative in simple and complicated appendicitis.

BACKGROUND/PURPOSE: The laparoscopic treatment of pediatric appendicitis remains controversial, particularly in complicated cases (gangrene and perforation). This study evaluates outcomes of open (OA) and laparoscopic appendectomy (LA). METHODS: The 391 cases of pediatric appendectomy performed between January 1998 and January 2001 were reviewed for age, sex, weight, type and length of intervention, operative description, antimicrobial therapy, analgesia, complications, length of hospitalization, and histopathology. RESULTS: A total of 126 patients were operated on by laparoscopy, 262 by laparotomy, and there were 3 conversions (LA + OA). LA patients were older (11.9 v 9.6 years; P <.001) and more frequently girls (57.1% v 38.2%; P =.0004). LA took longer to perform (45.7 v 40.6 minutes; P =.0014). Operatively, 24.6% of LAs were described as complicated compared with 22.5% in OA. Narcotic use was equivalent in both groups (1.16 v 1.29 days; P =.434), as was the incidence of complications, either operative (1.6% v 0.4%; P =.20) or postoperative (10.3% v 8.02%; P =.32). Hospitalization was shorter in LA (2.38 v 2.94 days; P =.0131). Histopathology was negative in 21.4% of LAs compared with 13% of OAs (P =.032). CONCLUSIONS: Laparoscopic appendectomy does not increase the incidence of complications, even with gangrenous or perforated appendicitis. The length of intervention is prolonged by 5 minutes on average. This technique allows for a shorter hospitalization.