A rare genetic disease characterized by co-occurrence of sick sinus syndrome (manifesting as sinus bradycardia, often requiring pacemaker implantation) and chronic intestinal pseudo-obstruction (which may be of myogenic or neurogenic origin and usually requires total parenteral nutrition), with an age of onset within the first four decades of life.
CAID disrupts the normal rhythm of the heartbeat ; affected individuals have a heart rhythm abnormality called sick sinus syndrome. The disorder also impairs the rhythmic muscle contractions that propel food through the intestines (peristalsis), causing a digestive condition called intestinal pseudo-obstruction. The heart and digestive issues develop at the same time, usually by age 20. Sick sinus syndrome (also known as sinus node dysfunction) is an abnormality of the sinoatrial (SA) node, which is an area of specialized cells in the heart that functions as a natural pacemaker. ... These signals travel from the SA node to the rest of the heart, signaling the heart (cardiac) muscle to contract and pump blood. In people with sick sinus syndrome, the SA node does not function normally, which usually causes the heartbeat to be too slow (bradycardia), although occasionally the heartbeat is too fast (tachycardia) or rapidly switches from being too fast to being too slow (tachycardia-bradycardia syndrome).
Cardiac features included sick sinus syndrome (SSS), manifested as sinus bradycardia in all 17 patients, 4 of whom exhibited junctional escape rhythms; atrial dysrhythmias in 6 patients, consisting of atrial flutter in 5 and atrial fibrillation in 1; and valve anomalies in 7 patients, involving the aortic valve in 4, the mitral valve in 2, and the pulmonary valve in 1. ... INHERITANCE - Autosomal recessive GROWTH Weight - Low weight Other - Failure to thrive CARDIOVASCULAR Heart - Sick sinus syndrome - Bradycardia - Junctional escape rhythm (in some patients) - Atrial flutter (in some patients) - Atrial fibrillation (rare) - Left atrial dilation - Sclerotic aortic valve (in some patients) - Bicuspid aortic valve (in some patients) - Mitral regurgitation, mild (in some patients) - Pulmonary valve stenosis (in some patients) ABDOMEN Gastrointestinal - Intestinal pseudoobstruction, neurogenic and myogenic - Hypoplastic ganglia in enteric nervous system - Mislocalization of ganglia in circular and longitudinal cell layers - Mislocalization of Cajal cells in circular and longitudinal cell layers - Abundant T cells in smooth muscle layers - Extensive fibrosis in smooth muscle layers - Thinning of smooth muscle layers - Fragmentation of smooth muscle fiber architecture SKIN, NAILS, & HAIR Skin - Hyperpigmented skin lesions (in some patients) LABORATORY ABNORMALITIES - C-band karyotype shows 'railroad track' appearance MISCELLANEOUS - Onset of symptoms in first or second decade of life - Almost all patients require total parenteral nutrition - Many patients require cardiac pacemakers MOLECULAR BASIS - Caused by mutation in the shugoshin-like-1 gene (SGOL1, 609168.0001 ) ▲ Close
A rare syndromic genetic deafness characterized by profound congenital bilateral sensorineural deafness, developmental delay, moderate intellectual disability, generalized delay in bone maturation, short stature, epiphyseal dysplasia particularly of the capital femoral epiphyses, and mild dysmorphic facial features such as prominent forehead and small, pointed chin.
Clinical Features Balikova et al. (2008) studied a large family with autosomal dominant inheritance of microtia and nasolacrimal duct imperforation associated with variable degrees of eye coloboma. Cytogenetics High resolution cytogenetic analysis in the family reported by Balikova et al. (2008) revealed an abnormal karyotype 46,XY,add(4)(pter). The cytogenetically visible alteration at 4p16-p15 consisted of 5 copies of a copy number variable region, encompassing a low copy repeat (LCR)-rich sequence. Balikova et al. (2008) demonstrated that the amplicon of approximately 750 kb occurred in exact tandem copies. This was thought to be the first example of an amplified copy number variant (CNV) associated with a mendelian disorder.
Craniodigital syndrome - intellectual deficit is characterised by syndactyly of the fingers and toes, characteristic facies (`startled' facial expression with a small pointed nose, micrognathia, long dark eyelashes and prominent eyebrows) and intellectual deficit.
Hirschsprung disease-type D brachydactyly syndrome is characterized by Hirschsprung disease and absence or hypoplasia of the nails and distal phalanges of the thumbs and great toes (type D brachydactyly).
Reynolds et al. (1983) presented a family in which 2 brothers and 2 of their maternal uncles had Hirschsprung disease and absence or hypoplasia of the nails and distal phalanges of the thumbs and great toes. Obligate heterozygotes showed no abnormality. The authors concluded that the pedigree was 'consistent with X-linked recessive inheritance but autosomal dominant with incomplete penetrance in females or multifactorial causation could not be ruled out.' Limbs - Absent/hypoplastic thumb and great toe distal phalanges - Brachydactyly type D GI - Hirschsprung disease Inheritance - ? X-linked ▲ Close
A rare X-linked syndromic intellectual disability characterized by congenital and permanent vocal cord paralysis causing severe congenital laryngeal stridor, associated with intellectual disability in male patients.
Morelli et al. (1980, 1982) described 5 members of a family with autosomal dominant inheritance of Gerhardt syndrome. The severity of the disorder was variable: 2 died of neonatal asphyxia, one had partial paralysis with mild symptoms, and 3 required surgery.
Plott (1964) described 3 brothers with permanent congenital laryngeal abductor paralysis and mental deficiency. A fourth male sib suspected of having been affected died perinatally. Dysgenesis of the nucleus ambiguus was considered likely. Watters and Fitch (1973) presented a pedigree which made X-linked recessive inheritance likely. Two brothers were affected together with a first cousin once removed connected through females. Opitz (1977) made the useful point that brain damage resulting from respiratory distress may dominate the picture so that X-linked mental retardation (see 309530) may be suspected.
A form of endogenous Cushing syndrome (CS) caused by abnormal production of ACTH due, in 80% of cases, to adrenocorticotropic hormone (ACTH) oversecretion by a pituitary adenoma (Cushing disease, CD) and in 20% of cases to ectopic ACTH secretion (CS due to EAS) by an extrapituitary tumor (in 50% of cases originating in the lungs or less commonly in the thymus, pancreas, adrenal gland or thyroid) or very rarely due to a tumor secreting both ACTH and corticotrophin-releasing hormone (CRH).
A number sign (#) is used with this entry because of evidence that glycosylphosphatidylinositol biosynthesis defect-15 (GPIBD15) is caused by homozygous or compound heterozygous mutation in the GPAA1 gene (603048) on chromosome 8q24. Description GPIBD15 is an autosomal recessive disorder characterized by delayed psychomotor development, variable intellectual disability, hypotonia, early-onset seizures in most patients, and cerebellar atrophy, resulting in cerebellar signs including gait ataxia and dysarthria. The disorder is caused by a defect in glycosylphosphatidylinositol (GPI) biosynthesis (summary by Nguyen et al., 2017). For a discussion of genetic heterogeneity of GPI biosynthesis defects, see GPIBD1 (610293). Clinical Features Nguyen et al. (2017) reported 10 patients from 5 unrelated families with GPIBD15.
Corneal intraepithelial dyskeratosis-palmoplantar hyperkeratosis-laryngeal dyskeratosis syndrome is a rare, genetic, corneal dystrophy disorder characterized by corneal opacification and dyskeratosis (which may cause visual impairment), associated with systemic features including palmoplantar hyperkeratosis, laryngeal dyskeratosis, pruritic hyperkeratotic scars, chronic rhintis, dyshidrosis and/or nail thickening.
A number sign (#) is used with this entry because of evidence that multiple self-healing palmoplantar carcinoma (MSPC) is caused by heterozygous mutation in the NLRP1 gene (606636) on chromosome 17p13. Description Multiple self-healing palmoplantar carcinoma (MSPC) is characterized by recurrent keratoacanthomas in palmoplantar skin as well as in conjunctival and corneal epithelia. In addition, patients experience a high susceptibility to malignant squamous cell carcinoma (summary by Zhong et al., 2016). Clinical Features Soler et al. (2013) described a Caucasian French mother and son who had corneal intraepithelial dyskeratosis and palmoplantar hyperkeratosis. At 27 years of age, the mother presented with unilateral keratopathy with neovascularization and complete corneal opacification.
Colobomatous microphthalmia-rhizomelic dysplasia syndrome is a rare, genetic developmental defect during embryogenesis characterized by a range of developmental eye anomalies (including anophthalmia, microphthalmia, colobomas, microcornea, corectopia, cataract) and symmetric limb rhizomelia with short stature and contractures of large joints.
A number sign (#) is used with this entry because of evidence that microphthalmia and/or coloboma, with or without rhizomelic skeletal dysplasia, is caused by heterozygous mutation in the MAB21L2 gene (604357) on chromosome 4q31. One family with a homozygous mutation has also been reported. Clinical Features Rainger et al. (2014) described 8 patients from 5 unrelated families with mutations in the MAB21L2 gene who had microphthalmia with coloboma or clinical anophthalmia, with or without rhizomelic skeletal dysplasia. In 1 family (family 1463), there were 9 affected individuals spanning 3 generations; all but 1 had isolated microphthalmia and coloboma. The 13-year-old male proband also exhibited skeletal involvement, with bowing of both legs noted in infancy; he had joint contractures of the knees and hips bilaterally and bilateral hypoplastic femoral condyles and pes cavus as well as hypospadias and wasting of the calf muscles. A 10-year-old Norwegian girl (family 676) and an unrelated 24-year-old man (family 4480), both with clinical anophthalmia, had severe bilateral rhizomelia as well as contractures of all large joints; additional findings in the girl included macrocephaly and precocious puberty at age 7 years, and in the man, hypoplastic femoral condyles.
A rare systemic disease characterized by a severe phenotype in all male patients, combining abnormality of connective tissue typical for Ehlers-Danlos syndrome (including joint hypermobility, scoliosis, soft and doughy skin, hyperextensible skin, abnormal scarring, facial peculiarities, and generalized hypotonia, among others) and eventually lethal congestive heart failure due to polyvalvular disease.
Primary immunodeficiency syndrome due to p14 deficiency is characterised by short stature, hypopigmentation, coarse facies and frequent bronchopulmonary Streptococcus pneumoniae infections.
Molecular Genetics Bohn et al. (2007) performed genomewide linkage analysis in a Caucasian Mennonite family with a primary immunodeficiency syndrome and found significant linkage on chromosome 1q21-q23 between D1S498 and D1S1153.
Holoprosencephaly-hypokinesia syndrome is an extremely rare and fatal central nervous system malformation occurring during embryogenesis, presenting prenatally with holoprosencephaly and fetal hypokinesia as major features.
The disorder resembled the Neu-Laxova syndrome (256520) in intrauterine growth retardation, multiple joint contractures, severe microcephaly, and recurrence in families. However, the lack of hyperkeratosis or ichthyosis and the resulting characteristic facial appearance, the lack of distal extremity swelling, and the presence of holoprosencephaly distinguished the disorder from the Neu-Laxova syndrome. The validity of the syndrome and the probable X-linked recessive inheritance suggested by Morse et al. (1987) were indicated by the report of Hockey et al. (1988): single cases in each of 3 sibships connected through females were affected with what appeared to be precisely the same disorder.
Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is a rare, acquired peripheral neuropathy disease characterized by rapidly progressive oropharyngeal (facial palsy, dysarthria) and cervicobrachial weakness, associated with upper limb weakness and hypo/areflexia, in the absence of ophthalmoplegia, ataxia, altered consciousness, and prominent lower limb weakness.
Therefore, other options should always be considered in the differential diagnosis. [9] Types [ edit ] Pai syndrome [ edit ] The Pai Syndrome is a rare subtype of frontonasal dysplasia. ... Generally, patients with these lipomas present with strokes. However, patients with the Pai syndrome don’t. That is why it is suggested that isolated nervous system lipomas have a different embryological origin than the lipomas present in the Pai syndrome. ... Until today there is no known cause for the Pai syndrome. The large variety in phenotypes make the Pai syndrome difficult to diagnose. ... "Syndromes, disorders and maternal risk factors associated with neural tube defects (V)" . ... S2CID 20674158 . ^ Golabi M, Gonalez MC, Edwards MS (1983). "A new syndrome of oculoauriculovertebral dyspasia and midline craniofacial defect: the oculoauriculofrontonasal syndrome: Two new cases in sibs".
There are three main types of frontonasal dysplasia that are distinguished by their genetic causes and symptoms. Other frontonasal dysplasia syndromes have also been described. Frontonasal dysplasia is very rare, with around 100 cases reported in the literature.
"Primary Progressive Multiple Sclerosis Evolving From Radiologically Isolated Syndrome". Annals of Neurology . 79 (2): 288–294. doi : 10.1002/ana.24564 . ... "Incidental MRI anomalies suggestive of multiple sclerosis: the radiologically isolated syndrome". Neurology . 72 (9): 800–805. doi : 10.1212/01.wnl.0000335764.14513.1a . ... Al (2017-10-01). "Radiologically isolated syndrome and multiple sclerosis" . Multiple Sclerosis and Related Disorders . 17 : 234–237. doi : 10.1016/j.msard.2017.08.016 . ... Mark; Donlon, Stacy; Hua, Le H. (2014-03-05). "Radiologically Isolated Syndrome: 5-Year Risk for an Initial Clinical Event" . ... "Prevalence of Radiologically Isolated Syndrome and White Matter Signal Abnormalities in Healthy Relatives of Patients with Multiple Sclerosis" .
It can be congenital or caused by a penetrant injury. [1] Isolated aniridia is a congenital disorder which is not limited to a defect in iris development, but is a panocular condition with macular and optic nerve hypoplasia , cataract , and corneal changes. [2] Vision may be severely compromised and the disorder is frequently associated with a number of ocular complications: nystagmus , amblyopia , buphthalmos , and cataract . [1] Aniridia in some individuals occurs as part of a syndrome, such as WAGR syndrome ( kidney nephroblastoma (Wilms tumour), genitourinary anomalies and intellectual disability), or Gillespie syndrome ( cerebellar ataxia ). ... These patients often also have genitourinary abnormalities and intellectual disability ( WAGR syndrome ). Several different mutations may affect the PAX6 gene. ... Online Mendelian Inheritance in Man (OMIM): 106210 AN Online Mendelian Inheritance in Man (OMIM): 106220 Aniridia and absent patella Online Mendelian Inheritance in Man (OMIM): 106230 Aniridia, microcornea, and spontaneously reabsorbed cataract Online Mendelian Inheritance in Man (OMIM): 206700 Aniridia, cerebellar ataxia, and mental deficiency (Gillespie syndrome) Mutational analysis [ edit ] Molecular ( DNA ) testing for PAX6 gene mutations (by sequencing of the entire coding region and deletion/duplication analysis ) is available for isolated aniridia and the Gillespie syndrome. For the WAGR syndrome, high-resolution cytogenetic analysis and fluorescence in situ hybridization (FISH) can be utilized to identify deletions within chromosome band 11p13, where both the PAX6 and WT1 genes are located. [5] Treatment [ edit ] In May 2018, the U.S. ... The prosthetic iris is held in place by the anatomical structures of the eye or, if needed, by sutures. [6] See also [ edit ] WAGR syndrome Scleral lenses References [ edit ] ^ a b Lang, K (2007).
A number sign (#) is used with this entry because of evidence that aniridia-3 (AN3) is caused by heterozygous mutation in the TRIM44 gene (612298) on chromosome 11p13. One such family has been reported. For a general phenotypic description and a discussion of genetic heterogeneity of aniridia, see AN1 (106210). Clinical Features Zhang et al. (2015) studied a 4-generation Chinese family in which 8 patients had aniridia without systemic abnormalities. The patients ranged in age from 7 to 55 years, and all had complete bilateral defects of the iris. Visual acuity gradually decreased over time due to the irregular lens opacity caused by lack of iris block and direct exposure to the sun.
There were no apparent autistic problems or intellectual disabilities in affected individuals, and systemic evaluation excluded WAGR syndrome (194072), Axenfeld, Rieger and Peters anomalies, iridocorneal endothelial syndromes, and sclerocornea.
Genetic Heterogeneity of Aniridia There is also evidence that aniridia-2 (AN2) is caused by mutation in a PAX6 cis-regulatory element (SIMO) that resides in an intron of the adjacent ELP4 gene (606985), and that aniridia-3 (AN3) is caused by mutation in the TRIM44 gene (612298) on chromosome 11p13. See also Gillespie syndrome (206700), in which aniridia is associated with cerebellar ataxia and mental retardation. ... Ticho et al. (2006) described a 6-year-old Caucasian boy with 'atypical Gillespie syndrome' (see 206700) and a de novo mutation in the PAX6 gene (see MOLECULAR GENETICS), who had partial aniridia, balance disorder, hand tremor, and learning disability. ... Although the ocular features and learning disorder were suggestive of Gillespie syndrome (see 206700), the authors stated that the novelty of the PAX6 mutation and relative subtlety of neurologic findings argued against that conclusion. ... The authors noted that the patient lacked the festooned pupillary edge and tufting considered to be pathognomonic for Gillespie syndrome, and that she had other clinical manifestations that were atypical for Gillespie patients; they stated that the role of additional unknown genetic variants could not be excluded. ... From a review of many reported cases, Moore et al. (1986) concluded that single breaks are associated with isolated aniridia, whereas deletion of 11p13 results in the WAGR syndrome. The association of a disorder with seemingly balanced autosomal reciprocal translocation of several other types has been observed (see, for example, 101200, 115650, 127300, 157900, 175700, 182900, 268800).
Aniridia may occur either as an isolated eye abnormality or as part of the Wilms tumor-aniridia-genital anomalies-retardation (WAGR) syndrome . Isolated aniridia may be caused by mutations in the PAX6 gene and is inherited in an autosomal dominant pattern.
Isolated aniridia is a congenital bilateral ocular malformation characterized by the complete or partial absence of the iris. Epidemiology The annual incidence is estimated at 1/ 64,000- 1/ 96,000. Clinical description Isolated aniridia can occur in association with a range of other ocular anomalies including cataract, glaucoma (usually occurring during adolescence), corneal pannus, optic nerve hypoplasia, absence of macular reflex, ectopia lentis, nystagmus, and photophobia, all of which generally result in poor vision. Etiology Aniridia is due to mutations in the PAX6 gene (11p13) encoding a transcriptional regulator involved in oculogenesis. PAX6 mutations result in alterations in corneal cytokeratin expression, cell adhesion and glycoconjugate expression.
Find sources: "Mast cell activation syndrome" – news · newspapers · books · scholar · JSTOR ( April 2020 ) Not to be confused with Mastocytosis . ... S2CID 43636053 . ^ a b c d Akin C, Valent P, Metcalfe DD (December 2010). "Mast cell activation syndrome: Proposed diagnostic criteria" . ... PMID 25841551 . ^ a b c d White A (17 February 2015). "A Tale of Two Syndromes – POTS and MCAS " " . The Dysautonomia Dispatch . ... "AAAAI Mast Cell Disorders Committee Work Group Report: Mast cell activation syndrome (MCAS) diagnosis and management" . ... Further reading [ edit ] The role of mast cells in functional GI disorders FFT at Gut/BMJ Mast Cell Activation Syndrome – May 2015 Spectrum of mast cell activation disorders – 2014 Mast cell activation syndromes: definition and classification – 2013 Expanding Spectrum of Mast Cell Activation Disorders: Monoclonal and Idiopathic Mast Cell Activation Syndromes – 2013 External links [ edit ] Classification D ICD - 10 : D89.4 ICD - 9-CM : 279.8
Laparoscopic Hernia Surgery Contents 1 Signs and symptoms 2 Diagnosis 3 Treatment 4 Eponym 5 Raveenthiran syndrome 6 References 7 External links Signs and symptoms [ edit ] Individuals typically present with either intermittent pain (coming and going), a lump, or mass all which are classic signs of a bowel obstruction . [2] The patient may have a protuberance when standing in an upright position although discomfort can sometimes be confused by its anatomical region for a peptic ulceration . [3] The bulge may be painful when the patient stretches but then goes away when they are lying down in a resting position. [4] However, a number of patients present with no obvious symptoms but vague tenderness along the area in which the Spigelian fascia is located. [5] Diagnosis [ edit ] Ultrasound Imaging or a CT Scan will provide better imaging for the detection of a hernia than an xray. [6] The diagnosis of a Spigelian hernia is traditionally difficult if only given a history and physical examination. [7] People who are good candidates for elective Spigelian hernia surgery, not only but also, after receiving an initial diagnostic consultation by a licensed medical professional, will be advised to see a physician to schedule surgery. ... In 1764, almost a century later, the Flemish anatomist, Josef Klinkosch was acknowledged for recognizing and describing a hernia located in the Spigelian fascia , and coined the term Spigelian hernia. [13] Raveenthiran syndrome [ edit ] Dr. Raveenthiran described a new syndrome in which Spigelian hernia and cryptorchidism (undescended testis) occur together. [14] Some common complications of this distinct syndrome cryptorchidism are testicular torsion , and its link to testicular cancer. [15] References [ edit ] ^ Skandalakis, PN; Zoras, O (2006-12-01). ... "Congenital Spigelian hernia with cryptorchidism: probably a new syndrome". Hernia . 9 (4): 378–80. doi : 10.1007/s10029-005-0316-z . ... Laparoscopic Repair of Spigelian Hernia Medtube v t e Diseases of the digestive system Upper GI tract Esophagus Esophagitis Candidal Eosinophilic Herpetiform Rupture Boerhaave syndrome Mallory–Weiss syndrome UES Zenker's diverticulum LES Barrett's esophagus Esophageal motility disorder Nutcracker esophagus Achalasia Diffuse esophageal spasm Gastroesophageal reflux disease (GERD) Laryngopharyngeal reflux (LPR) Esophageal stricture Megaesophagus Esophageal intramural pseudodiverticulosis Stomach Gastritis Atrophic Ménétrier's disease Gastroenteritis Peptic (gastric) ulcer Cushing ulcer Dieulafoy's lesion Dyspepsia Pyloric stenosis Achlorhydria Gastroparesis Gastroptosis Portal hypertensive gastropathy Gastric antral vascular ectasia Gastric dumping syndrome Gastric volvulus Buried bumper syndrome Gastrinoma Zollinger–Ellison syndrome Lower GI tract Enteropathy Small intestine ( Duodenum / Jejunum / Ileum ) Enteritis Duodenitis Jejunitis Ileitis Peptic (duodenal) ulcer Curling's ulcer Malabsorption : Coeliac Tropical sprue Blind loop syndrome Small bowel bacterial overgrowth syndrome Whipple's Short bowel syndrome Steatorrhea Milroy disease Bile acid malabsorption Large intestine ( Appendix / Colon ) Appendicitis Colitis Pseudomembranous Ulcerative Ischemic Microscopic Collagenous Lymphocytic Functional colonic disease IBS Intestinal pseudoobstruction / Ogilvie syndrome Megacolon / Toxic megacolon Diverticulitis / Diverticulosis / SCAD Large and/or small Enterocolitis Necrotizing Gastroenterocolitis IBD Crohn's disease Vascular : Abdominal angina Mesenteric ischemia Angiodysplasia Bowel obstruction : Ileus Intussusception Volvulus Fecal impaction Constipation Diarrhea Infectious Intestinal adhesions Rectum Proctitis Radiation proctitis Proctalgia fugax Rectal prolapse Anismus Anal canal Anal fissure / Anal fistula Anal abscess Hemorrhoid Anal dysplasia Pruritus ani GI bleeding Blood in stool Upper Hematemesis Melena Lower Hematochezia Accessory Liver Hepatitis Viral hepatitis Autoimmune hepatitis Alcoholic hepatitis Cirrhosis PBC Fatty liver NASH Vascular Budd–Chiari syndrome Hepatic veno-occlusive disease Portal hypertension Nutmeg liver Alcoholic liver disease Liver failure Hepatic encephalopathy Acute liver failure Liver abscess Pyogenic Amoebic Hepatorenal syndrome Peliosis hepatis Metabolic disorders Wilson's disease Hemochromatosis Gallbladder Cholecystitis Gallstone / Cholelithiasis Cholesterolosis Adenomyomatosis Postcholecystectomy syndrome Porcelain gallbladder Bile duct / Other biliary tree Cholangitis Primary sclerosing cholangitis Secondary sclerosing cholangitis Ascending Cholestasis / Mirizzi's syndrome Biliary fistula Haemobilia Common bile duct Choledocholithiasis Biliary dyskinesia Sphincter of Oddi dysfunction Pancreatic Pancreatitis Acute Chronic Hereditary Pancreatic abscess Pancreatic pseudocyst Exocrine pancreatic insufficiency Pancreatic fistula Other Hernia Diaphragmatic Congenital Hiatus Inguinal Indirect Direct Umbilical Femoral Obturator Spigelian Lumbar Petit's Grynfeltt-Lesshaft Undefined location Incisional Internal hernia Richter's Peritoneal Peritonitis Spontaneous bacterial peritonitis Hemoperitoneum Pneumoperitoneum Classification D ICD - 10 : K43.9 ICD - 9-CM : 553.29 Wikimedia Commons has media related to Spigelian hernia .