Developmental Posterior Enteric Remnants and Spinal Malformations: The Split Notochord Syndrome

Bentley, J.F.R.; Smith, J. Richard

doi:10.1136/adc.35.179.76

Cited by 412 publications

(167 citation statements)

References 20 publications

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“…Saunders suggested that this canal is formed within a localized duplication or cleft of the notochord, through which the endoderm (or primitive gut) herniates to adhere to the dorsal ectoderm [21]. Bentley and Smith [6] supported this theory. Beardmore and Wigglesworth suggested that adhesions between ectoderm and endoderm, possibly with an endomesenchymal tract formed around these adhesions (as suggested by Pang and Dias [27]), create an accessory neuroenteric canal, bisecting the developing notochord [28].…”

Section: Discussionmentioning

confidence: 58%

“…More subtle anomalies, such as spina bifida occulta, may be indicated only by the presence of hypertrichosis, a nevus, angioma, or a sacral dimple. Associated anomalies are diverse, and those present in our case as mentioned by others [6,8,9,[11][12][13][14][15][16][17][18][19][20][21][22] are listed in Table 1. Bentley and Smith stated that malformations of the heart and the great vessels have also been associated with abnormal spinal development, but a ready explanation is not at hand [6].…”

Section: Discussionmentioning

confidence: 94%

“…According to Bentley and Smith [6], visceral malformations can be classified into fistulae, sinuses, posterior enteric diverticula, and posterior enteric cysts. Complicated anomalies may be easy to detect, even antenatally [7].…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

A case of split notochord syndrome: a child with a neuroenteric fistula presenting with meningitis

Ramshorst

Lequin

Mancini

et al. 2006

Journal of Pediatric Surgery

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The authors describe a case of split notochord syndrome with a neuroenteric fistula in a newborn presenting with meningitis. Associated anomalies included agenesis of the corpus callosum, short colon, malrotation, epispadias, and an abnormally high bifurcation of the abdominal aorta and inferior vena cava. The embryological mechanisms and etiologic theories are discussed in short. D 2006 Elsevier Inc. All rights reserved.Split notochord syndrome (SNS) describes congenital spinal anomalies, associated with anomalies of the vertebrae, central nervous system, and gastrointestinal tract. The anomalies arise from a persistent connection between endoderm and dorsal ectoderm. Depending on its size, site, and point in time, abnormal splitting of the notochord can result in a wide variety of malformations and clinical symptomatology. Case reportA 4-day-old boy was referred to our hospital on suspicion of meningitis with secondary dehydration and for evaluation of vertebral anomalies. He was born at 37 weeks of gestation through normal vaginal delivery as the third child of a 26-year-old mother. Birth weight was 3010 g. Physical examination revealed normal tonus, minor facial dysmorphic signs (eg, broad mouth and dysmorphic, normally placed ears with a thin helix), left-sided congenital hip dysplasia, sacral dimple, and rocker bottom feet. The penis showed a mild epispadias, whereas anus, scrotum, and testes were normal. The mother had smoked during pregnancy and had not used supplementary folic acid. Besides local treatment of a vaginal yeast infection, no other medication had been used during pregnancy. The day before, the patient had been brought in at the referring hospital with a 2-day history of poor feeding and fever. He was jaundiced and showed a tachycardia at a body temperature of 398C. Neurologic examination revealed high tonus and a bulging anterior fontanel. The abdomen was distended. Abdominal x-ray showed a spinal cleft of the vertebrae of Th 12 and L1 (Fig. 1). The kidneys were normal on ultrasound. Blood tests showed elevated C-reactive protein (109 mg/L) and white blood cell count (25,500 cells per cubic millimeter). No lumbar puncture was attempted because of the vertebral anomalies. Blood cultures showed growth of Escherichia coli bacteria, after which, antibiotic treatment was switched from amoxicillin/ cefotaxim to meropenem.

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Section: Discussionmentioning

confidence: 58%

Section: Discussionmentioning

confidence: 94%

See 1 more Smart Citation

A case of split notochord syndrome: a child with a neuroenteric fistula presenting with meningitis

Ramshorst

Lequin

Mancini

et al. 2006

Journal of Pediatric Surgery

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show abstract

“…According to the split notochord theory by Bentley and Smith [6], abnormal adherence of endoderm (which differentiates into the foregut) and ectoderm (which forms the neural tube) and herniation of the same through a sagittally split notochord dorsally may lead to a neurenteric fistula. If the fistula is not closed by subsequent differential growth of nearby tissues, a variety of abnormalities including a duplication cyst may result.…”

Section: Discussionmentioning

confidence: 99%

Unusual Cystic Hypopharyngeal Mass in a Child with Obstructive Symptoms

Roy¹,

Rupa²,

Deepthi³

et al. 2011

Indian J Otolaryngol Head Neck Surg

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We present a rare case of a cystic posterior pharyngeal wall mass in a 5 year old child in whom prior surgery without biopsy led to considerable delay in both diagnosis and definitive treatment. The child presented with recurrent aspiration pneumonia, dysphagia and obstructed breathing from birth. Transoral wide excisional biopsy established the diagnosis of foregut duplication cyst. Only seven reported cases have been described in the English language literature till date.

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“…The duplicated bowel is rarely associated with vertebral anomalies, known as split notochord syndrome (Bentley and Smith 1960). This syndrome is a group of abnormalities caused by abnormal splitting or deviation of the notochord leading to a persistent connection between the gut and dorsal skin (Bentley and Smith 1960).…”

mentioning

confidence: 99%

Split Notochord Syndrome: Ileal Duplication Causing Intermittent Episodes of Vomiting

Tsuchida

Takahashi

Noguchi

et al. 2006

Tohoku J. Exp. Med.

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Developmental Posterior Enteric Remnants and Spinal Malformations: The Split Notochord Syndrome

Cited by 412 publications

References 20 publications

A case of split notochord syndrome: a child with a neuroenteric fistula presenting with meningitis

A case of split notochord syndrome: a child with a neuroenteric fistula presenting with meningitis

Unusual Cystic Hypopharyngeal Mass in a Child with Obstructive Symptoms

Split Notochord Syndrome: Ileal Duplication Causing Intermittent Episodes of Vomiting

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