Rare manifestations of Potter Sequence: A Case Report

Gautam, Uttara; Kafley, Rishikesh; Chikanbanjar, Vijaya Kumar; Shakya, Alyssa; Basnet, Rydam; Manandhar, Sunil Raja

doi:10.31729/jnma.4683

Cited by 4 publications

(6 citation statements)

References 6 publications

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“…Muscle defects or absence of the muscular layer of the gastric wall, iatrogenesis in the management of tracheoesophageal fistulas, hypoxia/ischemia, early sepsis, duodenal/jejunal obstruction, use of ibuprofen-paracetamol, esophageal atresia, administration of orogastric catheter, among others, are some of the causes reported in the literature [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] . Gastric perforation as a complication of Potter's syndrome has not been reported among the case series and case reports published so far [3] , [4] , [5] , [17] , [18] , [19] .…”

Section: Discussionmentioning

confidence: 97%

First case report of spontaneous perinatal gastric perforation in premature neonate with potter sequence and syndrome

Muñoz-Murillo

Hernández-López

et al. 2021

International Journal of Surgery Case Reports

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Introduction and importance The Potter sequence is defined as a series of congenital defects related to severe oligohydramnios, associated with polycystic kidney disease, bilateral renal agenesis, pulmonary hypoplasia, obstructive uropathy and premature rupture of membrane, which compromises the life of the neonate sometime after birth. Within the evidence published so far, which is very little, no perforation of the gastrointestinal tract has been reported as a complication of this condition. Case presentation Male neonate born preterm with prenatal diagnosis of pulmonary hypoplasia, polycystic renal dysplasia and severe oligohydramnios (Potter sequence), presented acute respiratory distress syndrome 10 min after birth, requiring mechanical ventilation and admission to the intensive care unit. During her stay in intensive care, he developed abdominal distension and presence of biliary content in the nasogastric tube. An abdominal X-ray was performed and showed signs of pneumoperitoneum, evidencing gastric perforation on exploratory laparotomy. Clinical discussion Gastric perforation in neonates is a condition that causes high health costs, morbidity, high risk of mortality and disability, regardless of the cause. The management of gastric perforation in Potter syndrome, as well as any other complication, represents a challenge due to the prognosis of these patients. Renal failure and acute respiratory distress syndrome are disorders that compromise the function of various structures and organs such as the heart and brain. Conclusion Gastric perforation is a possible complication of the Potter sequence or syndrome. In addition, there is no literature describing the benefits or disadvantages of specific surgical techniques in the resolution of perforation.

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Section: Discussionmentioning

confidence: 97%

First case report of spontaneous perinatal gastric perforation in premature neonate with potter sequence and syndrome

Muñoz-Murillo

Hernández-López

et al. 2021

International Journal of Surgery Case Reports

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“…Прогноз при ДПА зависит от наличия или отсутствия гипоплазии легких. Даже изолированная двусторонняя агенезия в большинстве случаев несовместима с жизнью [1,13]. Больные с двусторонней изолированной ДПА нуждаются в пожизненной заместительной терапии -проведении диализа, а в лучшем случае при достижении определенного возраста -трансплантации почки.…”

Section: Discussionunclassified

“…Кроме того, синдром Поттера диагностируется в 0,2-0,4% всех аутопсий мертворожденных и умерших в раннем неонатальном периоде. При этом у мальчиков заболевание встречается в 2-3 раза чаще [1][2][3].…”

Section: Introductionunclassified

“…Патогенез заболевания запускается тяжелыми врожденными аномалиями почек, ведущими к возникновению олигогидрамниона, который влечет за собой недоразвитие легочной ткани. Давление на стенку матки из-за маловодия приводит к необычному внешнему виду лица, неправильному положению и дефектам развития конечностей [1].…”

Section: Introductionunclassified

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A case report of Potter’s syndrome in a newborn

Shidakov¹,

Shavtikova²,

Dzhibabova³

2021

Medicinskij sovet

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Introduction. Potter’s sequence is a very rare and severe syndromic complex that includes congenital kidney defects leading to oligohydramnios, lung hypoplasia and structural skeletal disorders. Clinical case presentation: observation of a boy born from the 5th pregnancy to the mother at 37 weeks. Antenatally, the fetus was found to have bilateral renal and urinary bladder agenesis, malformations of the limbs, but the parents refused to terminate the pregnancy. After birth, the child was diagnosed with Potter’s syndrome with severe hypoplasia of the lungs, which required mechanical ventilation. In 2 hours after birth, the child developed a tension pneumothorax, which was arrested by performing drainage of the pleural cavity. During the day, the patient received complex treatment in the intensive care unit, despite which, by the 2nd day of life, the death of the child occurred. Currently, there are no guidelines for the treatment of Potter syndrome with proven positive long-term outcomes.Discussion. Children with Potter syndrome do not have the same set of symptoms, but they develop a chain of events leading to a common ultimate result - decreased amount of amniotic fluid. Abnormalities leading to oligohydramnios may include severe hypoplasia, dysplasia, polycystic, obstructive uropathy or renal agenesis. In most cases, the disease occurs sporadically, but there are also forms with transmission of the disorder through successive generations: autosomal dominant or recessive inheritance of polycystic disease, hereditary renal dysplasia caused by mutations in RET, UPK3A genes and other chromosomal abnormalities.Conclusion. Potter syndrome is a set of severe syndromes manifested by abnormalities in the development of kidneys, oligohydramnios, leading to lung hypoplasia, skeletal disorders and other congenital anomalies. The severity of congenital defects included in the set depends on the time periods when oligohydramnios occurred. Despite the availability of experimental therapies, the disease is now considered to be fatal.

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“…Erschwerend ist in unserem Fall die Einhaltung der orthopädischen Therapie bei einem kleinen, vulnerablen Frühgeborenen. Aufgrund postnataler Komplikationen mit intermittierend lebensbedrohlicher Verschlechterung musste die orthopädische Therapie mehrfach unterbrochen werden 1 2 3 4 5 .…”

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