Anorectal malformation and. Neonates

BACKGROUND: This study describes the presentation and initial management of anorectal malformation (ARM); evaluating the frequency, causes and consequences of late diagnosis. METHODS: A prospective, population cohort study was undertaken for newly diagnosed ARMs in the UK and Ireland from 01/10/2015 and 30/09/2016. Follow-up was completed at one year. Data are presented as n (%), appropriate statistical methods used. Factors associated with late diagnosis; defined as: detection of ARM either following discharge or more than 72 h after birth were assessed with univariable logistic regression...

BACKGROUND: Anorectal malformations (ARMs) are the most common congenital anomaly of the digestive tract. And colostomy should be performed as the first-stage procedure in neonates diagnosed with intermediate- or high-type ARMs. However, the most classic Pe˜na's colostomy still has some disadvantages such as complicated operation procedure, susceptibility to infection, a greater possibility of postoperative incision dehiscence, difficulty of nursing and large surgical trauma and incision scarring when closing the stoma...

OBJECTIVES: Multicystic dysplastic kidney (MCDK) is defined as the presence of multiple noncommunicating cysts of various sizes, detected sonographically, without evidence of functioning renal parenchyma on dimercaptosuccinic acid renal scan. It has an incidence of 1:4000 live births. They are more commonly diagnosed in boys, usually on the left side, but may also be bilateral. There is the presence of primitive ducts surrounded by fibromuscular connective tissue. These are because of the disturbed connection of the ureteric bud with renal blastema and abnormal division at the stage of metanephros, resulting in an abnormal metanephros differentiation...

BACKGROUND: Urinary tract drainage is necessary to facilitate the maneuver in laparoscopic-assisted anorectoplasty (LAARP). Male patients with Anorectal malformations (ARM) are often encounter difficult catheterization during surgery. OBJECTIVE: We pioneered the urinary catheterization through the urachus in ARM patients and evaluated the efficacy. METHOD: Fourteen ARM patients undergoing urinary catheterization through urachus between September 2015 and September 2023 were reviewed...

Congenital pouch colon (CPC) is highly uncommon congenital anorectal malformation where a distended pouch-like structure replaces either some part of the colon or the entire colon and communicates to the genitourinary tract through a fistula. Diagnosis of CPC is usually made after birth when neonate/infant presents with abdominal distension and absence of anal opening. Making antenatal diagnosis of CPC is difficult because of the lack of specific and verifiable signs on sonography. Hence, only a few cases of antenatal diagnosis of CPC have been reported...

Penile agenesis is complete absence of the penis in an otherwise normal phenotypic and genotypic male at birth that results from failure of development of the genital tubercle. It is an extremely rare anomaly that may be associated with anomalies in other organ systems, the extent and severity of which may affect the prognosis. The management is challenging and may have far reaching implications for the individual and family. While gender reassignment with bilateral orchidectomy and feminising genitoplasty has been carried out for most patients, significant psychosexual and social issues related to the male identity may occur due to foetal or postnatal sex steroid imprinting...

OBJECTIVE: To analyze the clinical characteristics and types of congenital heart defect (CHD) in mild congenital anorectal malformation (CARM), namely the rectoperineal and rectovestibular fistulas. METHODS: The retrospective study of 183 patients with mild CARM was conducted with assessments of demographic information, color Doppler echocardiography results, and follow-up data. We performed an analysis of the clinical characteristics of CHD, grouping them based on sex and type of mild CARM...

INTRODUCTION: Enhanced Recovery After Surgery (ERAS) guidelines are bundled evidence-informed recommendations implemented to improve quality and safety of perioperative care. This study aims to determine feasibility of NICU implementation of an ERAS Guideline for Intestinal Resection, describing clinical outcomes and adherence to recommendations following light-touch implementation. METHODS: Infants <28 days undergoing laparotomy for intestinal resection in a closed-NICU were prospectively enrolled...

OBJECTIVE: Congenital heart diseases (CHD) are the most frequently associated anomalies with anorectal malformations (ARM). Nevertheless, many specific aspects of CHD in ARM patients have yet to be studied. The aims of this study were to evaluate the prevalence and distribution of CHD in neonates-infants with ARM, and to explore whether the severity of ARM, and the presence of VACTERL association, had an impact on CHD rate, severity, and timing at first cardiac surgery. STUDY DESIGN: All consecutive newborn-infants with ARM managed in our tertiary center (January-1999; December-2021) were collected from a prospective database and retrospectively analyzed...

Gray platelet syndrome is a rare hereditary autosomal recessive condition distinguished by a mild to moderate propensity toward bleeding, moderate reduction in platelet count, and a significant decrease or complete absence of platelet alpha granules. VACTERL association is a condition of specific birth defects affecting multiple organ systems, with an unknown etiology. The acronym stands for vertebral anomalies (V), anal anomalies (A), cardiac anomalies (C), tracheoesophageal fistula (TE), renal anomalies or radial bone anomalies (R), and limb defects (L)...

BACKGROUND: In recent years, the number of neonatal surgeries has been on the rise despite the decline in the number of births, and we examined the actual trends and problems at Tottori University Hospital located in the Sanin region. METHODS: Medical records were retrospectively searched for patients who underwent major surgery during the neonatal period (within 30 days of age) at the Tottori University Hospital over the past 10 years (Jan. 2011 to Dec. 2020). RESULTS: Sixty-five cases were included...

Fetal inguinal hernia (FIH) is a rare event, and only a few cases were published in the medical literature. In the present study, we aimed to characterize the sonographic features, clinical presentation, management, outcomes, and differential diagnoses of FIH. Accordingly, we reviewed all 17 cases of FIH published in the medical literature, including one new case evaluated by our group. All 17 cases (100%) were male, and FIH presented as a scrotal mass with a mean diameter of 38±9.5 mm. The right side was dominant (62%)...

PURPOSE: Recent series of newborn Oesophageal Atresia (OA) repair continue to report widespread use of chest drains, gastrostomy, routine contrast studies and parenteral nutrition (PN) despite evidence suggesting these are superfluous. We report outcomes using a minimally interventional approach to post-operative recovery. METHODS: Ethically approved (15/WA/0153), single-centre, retrospective case-note review of consecutive infants with OA 2000-2022. Infants with OA and distal trache-oesophageal fistula undergoing primary oesophageal anastomosis at initial surgery were included (including those with comorbidities such as duodenal atresia, anorectal malformation and cardiac lesions)...

Anorectal malformations (ARMs) are managed classically in three stages - colostomy at birth, anorectal pull-through after 2-3 months, and stoma closure. Single-stage pull-through has been contemplated in neonatal age aimed to reduce the number of procedures, better long-term continence, the better psycho-social status of the child, and reduced cost of treatment, especially in resource-strained countries. We conducted a systematic review comparing neonatal single-stage pull-through with stage pull-through and did a meta-analysis for the outcome and complications...

PURPOSE: Perioperative and early post-operative outcomes of Primary Posterior sagittal anorectoplasty (P-PSARP) were evaluated. METHOD: Retrospective analysis of cases who underwent P-PSARP from 2004 to 2019 was done. Perioperative care, management, complications, voluntary bowel movement, soiling and constipation, graded by Krickenbeck criteria were studied. RESULTS: One hundred fifty six patients (134 girls) underwent P-PSARP at median age of 5 months (3 months to 14 years) in girls and 5(1-10) days in 21 boys...

BACKGROUND: Neonatal intestinal obstruction is a challenging issue, especially in developing countries. There is an apparent difference in the etiology, complications, and mortality of intestinal obstruction in neonates in different countries. OBJECTIVES: We aimed to describe the causes, early postoperative outcomes, and predictors of morbidities in neonates with intestinal obstruction in a tertiary neonatal intensive care unit (NICU) in Iran. DESIGN & METHODS: We conducted a retrospective study on neonates who were admitted with intestinal obstruction requiring surgery in the NICU of Boo-Ali Sina Hospital in northern Iran during 2018 to 2022...

BACKGROUND: In patients with anorectal malformation (ARM), a divided descending colostomy is associated with high complication rates, including wound infection, dehiscence, and prolapse, and it places a significant burden on caregivers. To decrease the chances of such complications, we modified our approach for colostomy fashioning by keeping an intact skin bridge between the colostomy and mucous fistula. The objective was to compare the rate of complications among patients diagnosed with ARM who underwent a modified divided descending colostomy and classical descending colostomy...

Anorectal malformations (ARMs) are common congenital anomalies in neonates. Colonic perforation is very rare in ARMs. Delay in diagnosis of neonate with ARM results in colonic perforations and life-threatening morbidity. Colonic perforation due to ARM may not be completely avoided; however, early diagnosis and management are essential in assuring better outcomes.

Urethral duplication is a rare anomaly with varying clinical manifestations. About 300 cases have been reported till date and are often associated with genitourinary (GU) and gastrointestinal (GI) tract anomalies. A 6-day-old neonate presented with an absent anal opening and a tiny perineal fistula having slight meconium staining. He did well after anoplasty. Low anorectal malformation (ARM) has been reported as the GI anomaly associated with urethral duplication. However, an ectopic scrotum as the principal GU tract anomaly associated with urethral duplication is being reported for the first time in literature with probable embryologic explanation...

Anorectal malformation is a disease with different subtypes and anatomical and functional multisystemic involvement that requires a unique approach in each age group. Anomalies associated with vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities (VACTERL) association require detailed investigation and management. Beginning from the neonatal period, treatment is carried out with different surgical procedures. The clinical course of these patients may be associated with medical problems, accompanying congenital anomalies, perioperative management, or late sequelae...