Incomplete pentalogy of Cantrell in a Border terrier puppy

Abstract

A 4-month-old, 6.4-kg, male entire, Border terrier was referred for investigation of exercise intolerance and a suspected congenital midline defect. Physical examination revealed a sternal and a supraumbilical abdominal defect. Thoracic radiography and CT confirmed a congenital sternal cleft, a large supraumbilical diastasis rectus and a pleuroperitoneal diaphragmatic hernia. No congenital cardiac defect was detected during echocardiographic examination. These findings were consistent with an incomplete Cantrell’s pentalogy. Surgical reconstruction involved diaphragmatic reconstruction, abdominal muscle herniorrhaphy and novel closure of a sternal cleft with crimped monofilament nylon leader line. The dog had grown since surgery to a mature adult size and did not exhibit exercise intolerance 18 months postoperatively. Cantrell’s pentalogy is a collection of congenital midline defects that are rare in the dog and cat but can be successfully treated surgically, especially if diagnosed at a young age and if not involving intracardiac defects.

Combined pentalogy of Cantrell with ectrodactyly and surgical implant‐free repair of a sternal cleft and supraumbilical hernia in an adult cat

“…27 Caudal sternal clefts in 4-and 5-month-old puppies with incomplete PoC and as an isolated defect in a 2-month-old kitten were closed using direct primary approximation of the sternal halves using crimped monofilament nylon lines, stainless steel monofilament wire and absorbable monofilament sutures, respectively. 6,7,20 A limiting factor may be where direct primary closure causes reduced abdominal and/or thoracic domain with significantly increased intracompartmental pressure, as this is poorly tolerated in the presence of severe cardiac malformations in people with PoC. 18 In our patient, the sternal cleft could be significantly reduced but deformity of the 'arms' meant that this caused potentially clinically significant ventral thoracic wall abnormality and myocardial compression.…”

“…PoC is a congenital syndrome seen sporadically in people 1 and extremely rarely in veterinary medicine. [5][6][7][8] If all five anomalies that define the complete form of PoC are not present, then incomplete PoC exists, with the class determined by the number of abnormalities present. 4 The combination and severity of the defects found are sometimes incompatible with life with neonates delivered stillborn, while others require early surgical correction.…”

“…PoC has been described only in the last decade in smallanimal medicine [5][6][7] ; this could suggest previous underreporting of recognised cases or failure to recognise this rare syndrome among veterinary patients. It may also be that severely affected animals die or are euthanased without veterinary intervention.…”

“…During the first few months of life, the sternal cleft can be easily closed due to the elasticity and relative expandability of young cartilage compared to the adult bony chest wall 27 . Caudal sternal clefts in 4‐ and 5‐month‐old puppies with incomplete PoC and as an isolated defect in a 2‐month‐old kitten were closed using direct primary approximation of the sternal halves using crimped monofilament nylon lines, stainless steel monofilament wire and absorbable monofilament sutures, respectively 6,7,20 . A limiting factor may be where direct primary closure causes reduced abdominal and/or thoracic domain with significantly increased intra‐compartmental pressure, as this is poorly tolerated in the presence of severe cardiac malformations in people with PoC 18 .…”

“…4 Complete or Class 1 PoC is where all five defects are present, incomplete can be Class 2 where four defects are present or Class 3 where three defects exist. In veterinary medicine, incomplete PoC (Class 3) has been reported in four occasions: an adult cat, 5 two puppies 6,7 and a calf. 8 To our knowledge, a case of complete PoC has not been reported in veterinary medicine to date.…”