Anus, imperforate

Megacolon, bowel obstruction, imperforate anus, or CHF Do not use sodium phosphate and sodium biphosphate in these patients hypernatremic dehydration may occur. 

Additional cases of aplasia cutis congenita in thiona-mide-exposed infants associated with other congenital abnormalities, such as choanal atresia, esophageal atresia, imperforate anus, and cardiovascular defects, were also reviewed. This pattern of abnormalities has previously led to the term methimazole [thiamazole] embryopathy (95). 

Gastrointestinal Duodenal atresia Agenesis of appendix and cecum Mai rotation Imperforate anus... 

Misra D, Mushtaq I, Drake DP et al (1996) Associated urologic anomalies in low imperforate anus are capable of causing significant morbidity a 15-year experience. Urology 48 281-283... 

Periodic screening US is reconunended in children with characteristics that are known to be associated with renal benign or malignant tumors (aniridia, hemihypertrophy, Drash syndrome, Beck-with-Wiedemann syndrome, tuberous sclerosis). In patients with malformation known to be associated with renal abnormality (the VATER association, imperforate anus, internal genital anomalies, Fanconi anemia), one postnatal US examination is recommended. However, abnormal external ears, unique umbilical artery, hypospadias, and undescended testis have not been proven to be associated with renal malformation and do not represent indications for renal screening (Currarino et al. 1993). 

Other indications are rarer. Imaging of urethral malformation or trauma remains based on the voiding part of VCU. Patients with cloacal anomalies, ambiguous genitalia, or imperforate anus can be explored. On the other hand, the role of VCU in investigating pelvic or bladder tumors has decreased since the advent of cystoscopy and MRI. 

The most common associated abnormality is that of VUR (Fig. 4.6b) (Kramer and Kelalis 1984). Therefore, VCU should be performed in all of these patients. Skeletal anomalies, imperforate anus, and cardiovascular anomalies have an increased incidence (Abeshouse and Bhisitkul 1959). Patients with solitary crossed ectopy have a higher incidence of genital abnormalities, probably related to the renal agenesis (Kakei et al. 1976). 

Cloacal malformation (Lebowitz 1997) exclusively occurs in the female phenotype and should not be confused with cloacal exstrophy. Cloacal malformation represents the most severe degree of imperforate anus (Hendren 1998). Prenatal diagnosis is difficult because a septated fluid-filled cavity can be mistaken for a normal bladder. However, echogenic... 

McLorie GA, Sheldon CA, Fleisher M, Churchill BM (1987) The genitourinary system in patients with imperforate anus. J Pediatr Surg 22 1100-1104 Merguerian PA, McLorie GA (1992) Disorders of the female genitalia In Kelalis PP, King LR, Belman AB (eds) Clinical pediatric urology, 3rd edn. Saunders, Philadelphia, PP 1084-1105... 

Metis JC, Kotkin L, Kasper S, Shyr Y, Adams MC, Brock JW (1997) Genital malformations and coexistent urinary tract or spinal anomalies in patients with imperforate anus. J Urol 158 1298-1300... 

Retterer E (1890) Sur I origine et T volution de la region ano-g nitale des mammiftres. J Anat Physiol 26 126-216 Rich MA, Brock WA, Pena A (1988) Spectrum of genitourinary malformations in patients with imperforate anus. Pediatr Surg Int 3 110-113... 

The term imperforate anus includes all kinds of anorectal malformations from covering of the anus by a thin skin membrane to high anorectal atresia - with or without a fistula, into the urethra or bladder - and to cloacal anomalies in females. It is well known that a high proportion of patients with imperforate anus have this maldevelopment in association with anomalies of one or several other... 

Duhamel (1961) reported that the most frequent malformations associated with imperforate anus are just those that one finds constantly in the... 

Urinary Problems Associated with Imperforate Anus 167... 

Fig. 8.2. Distribution of associated vertebral anomalies in patients with imperforate anus (n=33)...

Thus, the high incidence of vesicoureteral reflux in patients with imperforate anus may partly be... 

Fig. 8.4. IVU. Sacral agenesis in a patient with hyperreflexive neurogenic bladder and imperforate anus. Vesicoureteral reflux grade 2...

Recently, Boemers et al. (1999) have published detailed guidelines for the diagnostic screening and initial management of babies with imperforate anus. These recommendations are based on the complexity of the malformation and malfunction pattern in patients with imperforate anus and their impact on morbidity and mortality, which calls for a pre- and postoperative evaluation of the vertebral spine and the urogenital tract. 

---