Perineal Groove: An Anorectal Malformation Network, Consortium Study.

OBJECTIVE: To review the Anorectal Malformation Network experience with perineal groove (PG) focusing on its clinical characteristics and management. STUDY DESIGN: Data on patients with PG managed at 10 participating Anorectal Malformation Network centers in 1999-2019 were collected retrospectively by questionnaire. RESULTS: The cohort included 66 patients (65 females) of median age 1.4 months at diagnosis. The leading referral diagnosis was anal fissure (n = 20 [30.3%]): 23 patients (34.8%) had anorectal malformations. Expectant management was practiced in 47 patients (71.2%). Eight (17%) were eventually operated for local complications. The median time to surgery was 14 months (range, 3.0-48.6 months), and the median age at surgery was 18.3 months (range, 4.8-58.0 months). In the 35 patients available for follow-up of the remaining 39 managed expectantly, 23 (65.7%) showed complete or near-complete self-epithelization by a mean age 15.3 months (range, 1-72 months) and 4 (11.4%) showed partial self-epithelization by a mean age 21 months (range, 3-48 months). Eight patients showed no resolution (5 were followed for ≤3 months). Nineteen patients (28.7%) were primarily treated with surgery. In total, 27 patients were operated. Dehiscence occurred in 3 of 27 operated patients (11.1%). CONCLUSIONS: PG seems to be an underestimated anomaly, frequently associated with anorectal malformations. Most cases heal spontaneously; therefore, expectant management is recommended. When associated with anorectal malformations requiring reconstruction, PG should be excised in conjunction with the anorectoplasty.

[A neonate with soft tissue protruding through the umbilicus]. / Een neonaat met weefsel uit de navel.

A 6-week-old neonate presented with soft tissue protruding through the umbilicus due to a persisting ductus omphalo-entericus. This remnant was surgically removed the next day.

Inguinal hernia in girls: A retrospective analysis of over 1000 patients.

BACKGROUND: In girls with inguinal hernia, timing of surgical repair to prevent ovarian strangulation and screening for Androgen Insensitivity Syndrome (AIS) remain controversial. This study assesses the incidence of ovarian strangulation and AIS, and its associated risk factors. METHODS: Electronic patient records were used to study girls aged 0-15 years who underwent inguinal hernia repair between 2000 and 2017. Patients with incomplete data were excluded. Risk factors were identified using logistic regression. RESULTS: This study includes 1084 girls (median (IQR) age: 133.5 (14-281) weeks) who underwent 1132 hernia repairs (1015 unilateral, 117 bilateral) within a median (IQR) time interval of 12 (6-23) days following diagnosis. Hernia sac intraoperatively contained ovary in 235 (21.7%) patients, ovary was strangulated in 14 (6%). Risk factors for ovarian strangulation were younger gestational age (OR 0.49), higher birthweight (OR 32.18), and first presentation at the emergency department (OR 13.07). However data were partly missing. Ectopic testis was found in seven (0.6%) patients. Metachronous contralateral inguinal hernia and ipsilateral recurrence developed in 6.1% and 0.3%, respectively. CONCLUSIONS: Ovarian hernia was diagnosed in 21.7%, and ovary was strangulated in 6%. No definite conclusions can be drawn regarding risk factors for strangulation and timing of surgery in girls with irreducible ovarian hernia. LEVEL OF EVIDENCE: Level III.

Bronchoscopy and Fogarty Balloon Insertion of Distal Tracheo-Oesophageal Fistula for Oesophageal Atresia Repair With Video Illustration.

BACKGROUND/PURPOSE: During repair of esophageal atresia with distal tracheo-esophageal fistula, air leakage through the fistula during mechanical ventilation can cause respiratory demise. METHODS: From February 2012 until November 2014, all patients with esophageal atresia and distal tracheo-esophageal fistula were subjected to preoperative tracheobronchoscopy. Relatively distal fistulas were cannulated with a Fogarty catheter and blocked by insufflation (video illustration). Relatively proximal distal fistulas were sealed by precise placement of a cuffed ventilation tube. RESULTS: Nine of 12 patients received Fogarty balloon placement. The fistula of the remaining 3 patients were sealed by careful tube placement. No complications related to tracheobronchoscopy or Fogarty placement were noted. All procedures were uneventful. CONCLUSIONS: Preoperative tracheobronchoscopy to evaluate the usefulness of Fogarty balloon insertion or correct tube placement for distal tracheo-esophageal fistula is a safe and easy to perform procedure that can avoid complications in type C esophageal atresia repair.

Outcome of isolated gastroschisis; an international study, systematic review and meta-analysis.

OBJECTIVE: To determine outcome of children born with isolated gastroschisis (no extra-gastrointestinal congenital abnormalities). STUDY DESIGN: International cohort study and meta-analysis. PRIMARY OUTCOME: time to full enteral feeding (TFEF); secondary outcomes: Duration of mechanical ventilation, length of stay (LOS), mortality and differences in outcome between simple and complex gastroschisis (complex; born with bowel atresia, volvulus, perforation or necrosis). To compare the cohort study results with literature three databases were searched. Studies were eligible for inclusion if cases were born in developed countries with isolated gastroschisis after 1990, number of cases >20 and TFEF was reported. RESULTS: The cohort study included 204 liveborn cases of isolated gastroschisis. The TFEF, median duration of ventilation and LOS was, 26days (range 6-515), 2days (range 0-90) and 33days (range 11-515), respectively. Overall mortality was 10.8%. TFEF and LOS were significantly longer (P<0.0001) and mortality was fourfold higher in the complex group. Seventeen studies, amongst the current study, were included for further meta-analysis comprising a total of 1652 patients. Mean TFEF was 35.3±4.4days, length of ventilation was 5.5±2.0days, LOS was 46.4±5.2days and mortality risk was 0.06 [0.04-0.07 95%CI]. Outcome of simple and complex gastroschisis was described in five studies. TFEF, ventilation time, LOS were significant longer and mortality rate was 3.64 [1.95-6.83 95%CI] times higher in complex cases. CONCLUSIONS: These results give a good indication of the expected TFEF, ventilation time and LOS and mortality risk in children born with isolated gastroschisis, although ranges remain wide. This study shows the importance of dividing gastroschisis into simple and complex for the prediction of outcome.

Early versus late reconstruction of cloacal malformations: the effects on postoperative complications and long-term colorectal outcome.

INTRODUCTION: Patients with a cloacal malformation generally undergo reconstructive surgery within the first years of life. However, the ideal age for surgery has rarely been mentioned. The aim of this study was to report differences in outcome between early (<6 months) and late repair of cloacal malformations. METHODS: Charts of patients with a cloacal malformation treated in 5 pediatric surgical centers between 1985 and 2009 were retrospectively studied for associated anomalies, postoperative complications, and colorectal and urological outcome. RESULTS: Forty-two patients were eligible for this study, giving a mean exposure of less than 1 patient yearly per center. Forty-five percent of the patients had a short common channel (>3 cm), and 14% had a long common channel. Length of common channel was missing in 41% of the patients. Median age of the cloacal reconstruction was 9 months (range 1-121 months). Twelve patients (29%) underwent an early surgical repair (within the first 6 months of age; median 3 months), and 30 (71%) patients underwent a late repair (after 6 months of age; median 14 months). Eighteen postoperative complications (<30 days) had been documented in 15 patients (35%), with significant more perineal wound dehiscences in patients with an early repair (42% vs. 10%, p=0.031). There were no differences in complication rate between patients with short and long common channels. Mean follow-up was 142 months (range 15-289). At the last follow-up, 10 patients (24%) had voluntary bowel movements. Fourteen patients (33%) had complaints of soiling, 25 (60%) were constipated, with no differences between the early and late repair groups. Patients in the late repair group as well as the group of patients with a short common channel were more frequently able to void spontaneously. CONCLUSIONS: Postoperative complications are common in patients with cloacal malformations. Early repair is associated with more wound dehiscences, however, without affecting long-term functional outcome. All centers had limited annual exposure of less than 1 patient. In these clinical settings, ideal age of cloacal reconstruction seems to be between 6 and 12 months. In general, centralized care for these complex malformations may be the crucial factor for reducing postoperative complications and better long-term outcome.

Urogenital function after cloacal reconstruction, two techniques evaluated.

OBJECTIVE: Current surgical techniques for cloacal reconstruction are posterior sagittal anorecto vagino urethroplasty (PSARVUP) and posterior sagittal anorectoplasty (PSARP) with total urogenital mobilization (TUM). The aim of this study was to explore the results of reconstructive cloaca surgery in the Netherlands and evaluate urogenital function after PSARVUP and TUM. PATIENTS AND METHODS: Medical records from five pediatric surgical departments in the Netherlands were studied for patients with cloacal malformations treated between 1985 and 2009. Forty-two patients were eligible, and patients with short common channels were categorized into PSARVUP and TUM groups. Groups were compared using Fisher's exact test. RESULTS: Median age at time of surgery was 9 months (range 1-121). In 24 patients (57%) a PSARVUP was done, in 18 patients (43%) TUM. Median follow-up was 142 months (range 15-289). At follow-up spontaneous voiding was seen in 29 patients (69%). Clean intermittent catheterization (CIC) was needed in 14 patients (33%); a urinary diversion was created in 10 patients (24%). In total 32 patients (76%) were dry with no involuntary loss of urine per urethra. Recurrent urinary tract infections were seen in 23 patients (55%). When comparing PSARVUP and TUM groups in our series of patients with short common channels, there were no differences in urological outcome. Normal menstruation was present in 11 of the 20 patients who reached puberty (55%). CONCLUSION: Urogenital functional outcome after reconstructive surgery for cloacal malformations was similar in PSARVUP and TUM groups in patients with short common channels. A thorough urological follow-up is needed to establish the long-term bladder function and urinary incontinence results to prevent long-term risks of recurrent UTI. Albeit without differences between PSARVUP and TUM groups, 45% of the patients present with abnormal or absent menstruations. Gynecological follow-up is mandatory in all patients with cloacal malformations 6 months after the first sign of puberty.

[The bubble-blowing neonate: a red flag for oesophageal atresia]. / Een pasgeborene die bellen blaast: alarmsignaal voor oesofagusatresie.

When a newborn develops feeding difficulties, is blowing bubbles or has excessive saliva, it is important to consider the diagnosis of oesophageal atresia. Prenatal detection of oesophageal atresia is difficult. Postnatal bubble blowing, resulting from the inability to swallow excessive oropharyngeal secretions, is pathognomonic. These symptoms should trigger the midwife or physician to consider this diagnosis. We present three cases to illustrate the difficulties of early recognition and the consequences of a late detection of this condition. The first and third cases show that a delayed diagnosis of oesophageal atresia can lead to dangerous situations. Our second case illustrates that not every poorly drinking neonate has oesophageal atresia, and that this diagnosis may be rejected if the physician is able to pass a nasogastric tube. In every neonate who is unable to drink, we advise attempting to pass a nasogastric tube and taking a chest x-ray to distinguish between oesophageal atresia and other causes of feeding problems.

[Dysphagia after introduction of solid food: typical presentation of congenital oesophageal stenosis]. / Dysfagie na introductie van vast voedsel: typische presentatie van congenitale slokdarmstenose.

BACKGROUND: Congenital oesophageal stenosis is a rare cause of food passage symptoms in infants. It has a typical presentation with symptoms of dysphagia of solid food, starting at the time of introducing supplementary feeding. CASE DESCRIPTION: We present a 6-month-old girl, who started spitting and coughing and had a slower growth rate after the introduction of solid food. Using upper gastrointestinal tract radiography, oesophagogastroscopy and histopathological examination, a congenital oesophageal stenosis due to tracheobronchial remnants was demonstrated. The stenosis was surgically removed. This case description is typical for congenital oesophageal stenosis. CONCLUSION: Early recognition of the typical presentation of congenital oesophageal stenosis can prevent unnecessary investigation and delay in treatment. Surgical resection of the stenotic oesophageal segment usually results in full recovery.

Cervical cystic swelling in an adolescent: unusual association of a cervical mature teratoma with vertebral anomalies and a history of gastric duplication cyst.

A 14-year-old girl presented with a cervical cystic swelling in association with deformity of cervical vertebrae. As a child, she had been treated for gastric duplication. Pathologic examination of the resected cervical swelling revealed a mature teratoma. We discuss possible embryologic associations, which could explain the unusual combination of a mature teratoma with vertebral anomalies and gastric duplication.

[Internally situated sacrococcygeal teratomas, often invisible but not harmless]. / Inwendig gelegen sacrococcygeale teratomen. Vaak onzichtbaar, maar niet onschuldig.

A sacrococcygeal teratoma (SCT) may be completely concealed within the pelvis and therefore not be visible externally. Symptoms of such an SCT can be very aspecific. We present 4 patients with an intrapelvic SCT. The first patient, a newborn girl, had an antenatally-diagnosed cystic mass in her pelvis, which was a benign SCT. After removal she suffered from neurogenic bladder dysfunction. The second patient was a 2-year-old boy who after two incomplete resections of a benign intrapelvic SCT, developed a malignant SCT. The other 2 patients, 2 girls aged 4 and 15 years, both presented with constipation and abdominal pain and were diagnosed as having an SCT. Early detection and radical excision, including removal of the os coccygis, are necessary to prevent development of malignancy. Long-term functional sequelae can occur after excision.

Shoulder dislocation in a very-low-birth-weight infant: case report and review of the literature.

Shoulder dislocation in a preterm infant is very rare. We report a case of a preterm infant, with gestational age of 25 2/7 weeks and birth weight of 910 g, with anterior shoulder dislocation after breech delivery. The infant was successfully treated with closed reduction. In general, long-term outcome of shoulder dislocation in these infants is good. However, prompt recognition and treatment of the dislocated shoulder are essential to maintain normal shoulder girdle function and to prevent functional disability.